Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106167
Birol Güvenç
<div><h3>Objective</h3><div>Introduction: AL amyloidosis results from deposition of misfolded immunoglobulin light chains in various organs, with cardiac involvement occurring in approximately 60-70% of cases. Cardiac amyloidosis typically presents with heart failure symptoms and distinctive echocardiographic features including increased wall thickness, "sparkling" myocardium appearance, and restrictive physiology. While commonly associated with multiple myeloma, oligosecretory variants can pose diagnostic challenges due to minimal or absent monoclonal protein secretion in serum.</div></div><div><h3>Case Report</h3><div>A 43-year-old female presented with progressive palpitations, dyspnea, fatigue, and peripheral edema. Initial evaluation by cardiology revealed significant cardiac abnormalities prompting comprehensive investigation.</div><div>Echocardiography demonstrated characteristic findings highly suggestive of cardiac amyloidosis: concentric left ventricular hypertrophy with "sparkling" myocardium appearance, restrictive diastolic pattern, biatrial enlargement, moderate tricuspid regurgitation, and mild pulmonary hypertension. The constellation of findings was inconsistent with hypertensive heart disease, raising suspicion for infiltrative cardiomyopathy.</div><div>Given the typical echocardiographic appearance, the patient was referred to hematology for amyloidosis evaluation. Laboratory assessment revealed elevated inflammatory markers (CRP: 59-74 mg/L) but notably, serum protein electrophoresis showed no distinct M-band. However, serum immunofixation was positive only for lambda light chains with negative IgA, IgG, and IgM, suggesting an oligosecretory plasma cell disorder.</div><div>Bone marrow biopsy revealed 40% plasma cell infiltration with immunophenotype showing CD38(+), CD56(+), and CD19(-) with lambda light chain restriction. Critically, Congo red staining was positive, confirming amyloid deposition and establishing the diagnosis of AL amyloidosis. Cytogenetic analysis by FISH was negative for high-risk abnormalities including p53 deletion, RB1 deletion, t(11;14), and t(4;14).</div><div>Additional imaging revealed multisystem involvement: chest CT showed ground-glass opacities in lower lobes with reactive mediastinal lymphadenopathy, while abdominal ultrasound demonstrated grade 1 hepatosteatosis, minimal splenomegaly, and mild ascites, consistent with systemic amyloid deposition.</div><div>The patient's medical history was notable for appendiceal mucinous neoplasm in 2022, raising questions about potential relationships between these conditions.</div><div>Clinical presentation included progressive heart failure symptoms with peripheral edema, confirming cardiac involvement as the primary manifestation.</div></div><div><h3>Discussion</h3><div>This case illustrates several important clinical aspects of AL amyloidosis. The presentation in a 43-year-old patient is relatively uncommon, as AL amyloidosis typically affects older adults
{"title":"AL Amyloidosis Presenting with Cardiac Involvement in a 43-Year-Old Woman with Oligosecretory Multiple Myeloma","authors":"Birol Güvenç","doi":"10.1016/j.htct.2025.106167","DOIUrl":"10.1016/j.htct.2025.106167","url":null,"abstract":"<div><h3>Objective</h3><div>Introduction: AL amyloidosis results from deposition of misfolded immunoglobulin light chains in various organs, with cardiac involvement occurring in approximately 60-70% of cases. Cardiac amyloidosis typically presents with heart failure symptoms and distinctive echocardiographic features including increased wall thickness, \"sparkling\" myocardium appearance, and restrictive physiology. While commonly associated with multiple myeloma, oligosecretory variants can pose diagnostic challenges due to minimal or absent monoclonal protein secretion in serum.</div></div><div><h3>Case Report</h3><div>A 43-year-old female presented with progressive palpitations, dyspnea, fatigue, and peripheral edema. Initial evaluation by cardiology revealed significant cardiac abnormalities prompting comprehensive investigation.</div><div>Echocardiography demonstrated characteristic findings highly suggestive of cardiac amyloidosis: concentric left ventricular hypertrophy with \"sparkling\" myocardium appearance, restrictive diastolic pattern, biatrial enlargement, moderate tricuspid regurgitation, and mild pulmonary hypertension. The constellation of findings was inconsistent with hypertensive heart disease, raising suspicion for infiltrative cardiomyopathy.</div><div>Given the typical echocardiographic appearance, the patient was referred to hematology for amyloidosis evaluation. Laboratory assessment revealed elevated inflammatory markers (CRP: 59-74 mg/L) but notably, serum protein electrophoresis showed no distinct M-band. However, serum immunofixation was positive only for lambda light chains with negative IgA, IgG, and IgM, suggesting an oligosecretory plasma cell disorder.</div><div>Bone marrow biopsy revealed 40% plasma cell infiltration with immunophenotype showing CD38(+), CD56(+), and CD19(-) with lambda light chain restriction. Critically, Congo red staining was positive, confirming amyloid deposition and establishing the diagnosis of AL amyloidosis. Cytogenetic analysis by FISH was negative for high-risk abnormalities including p53 deletion, RB1 deletion, t(11;14), and t(4;14).</div><div>Additional imaging revealed multisystem involvement: chest CT showed ground-glass opacities in lower lobes with reactive mediastinal lymphadenopathy, while abdominal ultrasound demonstrated grade 1 hepatosteatosis, minimal splenomegaly, and mild ascites, consistent with systemic amyloid deposition.</div><div>The patient's medical history was notable for appendiceal mucinous neoplasm in 2022, raising questions about potential relationships between these conditions.</div><div>Clinical presentation included progressive heart failure symptoms with peripheral edema, confirming cardiac involvement as the primary manifestation.</div></div><div><h3>Discussion</h3><div>This case illustrates several important clinical aspects of AL amyloidosis. The presentation in a 43-year-old patient is relatively uncommon, as AL amyloidosis typically affects older adults","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106167"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145796757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106138
Candaş Mumcu, Birol Güvenç
<div><h3>Introduction</h3><div>Hematologic abnormalities in young adults are frequently attributed to infections or reactive processes, yet concurrent cytopenias and lymphocytosis may herald malignant conditions. Acute lymphoblastic leukemia (ALL) is uncommon in adults but should be considered in the presence of persistent unexplained hematologic abnormalities (Inaba et al., 2021). Here, we present a 29-year-old male patient initially hospitalized with myocarditis, in whom incidental hematologic findings prompted further investigation and ultimately led to the diagnosis of precursor B-cell acute lymphoblastic leukemia (B-ALL), Türkiye.</div></div><div><h3>Methods</h3><div>The patient, with comorbid obesity, hyperlipidemia, prediabetes, and coronary artery disease, was admitted to the coronary intensive care unit due to myocarditis. Laboratory evaluation revealed neutropenia, lymphocytosis, anemia, and severe thrombocytopenia. Hematology consultation was obtained, and systematic infectious and metabolic workup was performed, including TORCH, hepatitis panel, HIV, brucella, and syphilis, all of which were negative. Nutritional deficiencies were excluded. Bone marrow aspiration and biopsy were conducted to clarify the unexplained cytopenias.</div></div><div><h3>Results</h3><div>Peripheral smear showed marked lymphocytosis. Bone marrow evaluation demonstrated precursor B-cell blasts consistent with B-ALL. The patient had a prior history of episodic polycythemia treated with phlebotomy at an external center, but no prior evaluation for myeloproliferative neoplasm was documented. Physical examination was remarkable for obesity and cervical lymphadenopathy. Despite the confirmed diagnosis of B-ALL, the patient declined further therapy and left the clinic against medical advice.</div></div><div><h3>Discussion</h3><div>This case underscores the diagnostic challenge posed by overlapping cardiac and hematologic findings. While myocarditis can present with systemic manifestations that mimic hematologic disorders, persistent cytopenias with lymphocytosis should prompt early hematology evaluation (Terwilliger & Abdul-Hay, 2017). Adult B-ALL often carries a poor prognosis compared to pediatric cases, and early initiation of therapy is critical to improving outcomes (Kantarjian et al., 2017). Moreover, this case highlights the importance of considering hematologic malignancy in young adults with incidental laboratory abnormalities, even in the context of alternative explanations such as infection or cardiac disease. Systematic diagnostic workup, including bone marrow biopsy, remains the gold standard for definitive diagnosis (Hunger & Mullighan, 2015).</div></div><div><h3>Conclusion</h3><div>We report a young adult male followed for myocarditis in whom incidental hematologic abnormalities revealed underlying precursor B-cell ALL. This case emphasizes the necessity of maintaining a broad differential diagnosis in young adults with unexplained cytopenias a
年轻人血液学异常通常归因于感染或反应性过程，但同时发生的细胞减少和淋巴细胞增多可能预示着恶性疾病的发生。急性淋巴细胞白血病（ALL）在成人中并不常见，但应考虑存在持续不明原因的血液异常（Inaba et al., 2021）。在这里，我们报告了一位29岁的男性患者，他最初因心肌炎住院，偶然的血液学发现促使他进一步调查，最终诊断为前体b细胞急性淋巴细胞白血病（B-ALL）， t rkiye。方法患者合并肥胖、高脂血症、前驱糖尿病、冠心病，因心肌炎入住冠状动脉重症监护病房。实验室评估显示中性粒细胞减少、淋巴细胞增多、贫血和严重的血小板减少。进行血液学会诊，并进行系统的感染和代谢检查，包括TORCH、肝炎面板、HIV、布鲁氏菌和梅毒，均为阴性。排除营养缺乏。骨髓穿刺和活检以澄清不明原因的细胞减少。结果外周血涂片显示明显淋巴细胞增多。骨髓评估显示前体b细胞母细胞与B-ALL一致。患者既往有发作性红细胞增多症病史，曾在外部中心接受过静脉切开术治疗，但既往未见骨髓增生性肿瘤的评估记录。体格检查有明显的肥胖和颈部淋巴结病。尽管确诊为B-ALL，但患者拒绝进一步治疗，并不遵医嘱离开了诊所。本病例强调了心脏和血液检查结果重叠所带来的诊断挑战。虽然心肌炎可表现出类似血液学疾病的全身性表现，但持续性细胞减少伴淋巴细胞增多应提示早期血液学评估（Terwilliger & abdulal - hay, 2017）。与儿科病例相比，成人B-ALL的预后往往较差，早期开始治疗对改善预后至关重要（Kantarjian等，2017）。此外，该病例强调了考虑年轻成人偶发实验室异常的血液恶性肿瘤的重要性，即使在感染或心脏病等其他解释的背景下也是如此。系统的诊断检查，包括骨髓活检，仍然是明确诊断的金标准（Hunger & Mullighan, 2015）。结论我们报告了一位年轻的成年男性心肌炎患者，其偶然的血液学异常显示潜在的前体b细胞ALL。这个病例强调了对有不明原因的细胞减少症和淋巴细胞增多症的年轻成人进行广泛鉴别诊断的必要性，以及不延误骨髓评估的必要性。及时识别对于及时开始治疗和改善患者预后至关重要。
{"title":"Incidentally Detected Precursor B-Cell Acute Lymphoblastic Leukemia in a Patient Monitored for Myocarditis: A Case Report","authors":"Candaş Mumcu, Birol Güvenç","doi":"10.1016/j.htct.2025.106138","DOIUrl":"10.1016/j.htct.2025.106138","url":null,"abstract":"<div><h3>Introduction</h3><div>Hematologic abnormalities in young adults are frequently attributed to infections or reactive processes, yet concurrent cytopenias and lymphocytosis may herald malignant conditions. Acute lymphoblastic leukemia (ALL) is uncommon in adults but should be considered in the presence of persistent unexplained hematologic abnormalities (Inaba et al., 2021). Here, we present a 29-year-old male patient initially hospitalized with myocarditis, in whom incidental hematologic findings prompted further investigation and ultimately led to the diagnosis of precursor B-cell acute lymphoblastic leukemia (B-ALL), Türkiye.</div></div><div><h3>Methods</h3><div>The patient, with comorbid obesity, hyperlipidemia, prediabetes, and coronary artery disease, was admitted to the coronary intensive care unit due to myocarditis. Laboratory evaluation revealed neutropenia, lymphocytosis, anemia, and severe thrombocytopenia. Hematology consultation was obtained, and systematic infectious and metabolic workup was performed, including TORCH, hepatitis panel, HIV, brucella, and syphilis, all of which were negative. Nutritional deficiencies were excluded. Bone marrow aspiration and biopsy were conducted to clarify the unexplained cytopenias.</div></div><div><h3>Results</h3><div>Peripheral smear showed marked lymphocytosis. Bone marrow evaluation demonstrated precursor B-cell blasts consistent with B-ALL. The patient had a prior history of episodic polycythemia treated with phlebotomy at an external center, but no prior evaluation for myeloproliferative neoplasm was documented. Physical examination was remarkable for obesity and cervical lymphadenopathy. Despite the confirmed diagnosis of B-ALL, the patient declined further therapy and left the clinic against medical advice.</div></div><div><h3>Discussion</h3><div>This case underscores the diagnostic challenge posed by overlapping cardiac and hematologic findings. While myocarditis can present with systemic manifestations that mimic hematologic disorders, persistent cytopenias with lymphocytosis should prompt early hematology evaluation (Terwilliger & Abdul-Hay, 2017). Adult B-ALL often carries a poor prognosis compared to pediatric cases, and early initiation of therapy is critical to improving outcomes (Kantarjian et al., 2017). Moreover, this case highlights the importance of considering hematologic malignancy in young adults with incidental laboratory abnormalities, even in the context of alternative explanations such as infection or cardiac disease. Systematic diagnostic workup, including bone marrow biopsy, remains the gold standard for definitive diagnosis (Hunger & Mullighan, 2015).</div></div><div><h3>Conclusion</h3><div>We report a young adult male followed for myocarditis in whom incidental hematologic abnormalities revealed underlying precursor B-cell ALL. This case emphasizes the necessity of maintaining a broad differential diagnosis in young adults with unexplained cytopenias a","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106138"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145796972","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106154
Yakup ÜNSAL , Muhammed MURATI , Güler DELİBALTA , Serdar Bedii OMAY
<div><h3>Objective</h3><div>Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative therapeutic modality for patients with acute myeloid leukemia (AML)<sup>1</sup>. Patients undergoing HSCT are highly susceptible to SARS-CoV-2 infection due to profound immunosuppression, delayed immune reconstitution, and graft-versus-host disease (GVHD) prophylaxis<sup>2</sup>,⁴. Covid-19 infection in HSCT recipients, particularly during the early post-transplant period, has been associated with high morbidity and mortality⁵. Studies have demonstrated increased mortality in patients diagnosed with Covid-19 during HSCT, especially in the early phase⁶. Herein, we present a case of Covid-19 pneumonia that developed during allogeneic HSCT with myeloablative conditioning in a patient with high-risk AML.</div></div><div><h3>Case report</h3><div>Our patient was a 33-year-old female diagnosed with AML in March 2025. Following diagnosis, she received induction therapy with the 3+7 regimen (Idarubicin + Cytarabine). As remission was not achieved, she was administered FLAG-Mito as reinduction therapy. Despite two cycles of induction, no response was obtained, and the patient was considered refractory AML; thus, allogeneic transplantation was planned. During the pre-transplant period, she received two cycles of azacitidine + venetoclax. On July 21, 2025, myeloablative conditioning (Fludarabine + Treosulfan) was initiated. During conditioning, her caregiver developed upper respiratory tract infection symptoms and tested positive for Covid-19. Since the patient was asymptomatic, the transplantation procedure was continued. On July 28, 2025, she underwent allogeneic transplantation from her HLA-matched sibling donor. GVHD prophylaxis consisted of CsA + MTX, and voriconazole was given for antifungal prophylaxis. On day +3 post-transplant, the patient developed fever (38°C) and was treated as febrile neutropenia with broad-spectrum antibiotics (Meropenem). SARS-CoV-2 PCR testing was positive. Initially, she presented with mild symptoms, but one week after positivity, chest imaging revealed diffuse pulmonary infiltrates consistent with Covid-19 pneumonia (Figure-1). Despite broad-spectrum antibiotics, she required high-dose corticosteroids and a single dose of tocilizumab (400 mg) for cytokine release syndrome. Oxygen support was initiated. Ten days after pneumonia diagnosis, respiratory distress worsened, and she was admitted to the intensive care unit. On day +18, CPAP was initiated. Neutrophil engraftment was achieved on day +19. However, despite non-invasive respiratory support, progressive respiratory failure necessitated intubation. Shortly after intubation, the patient developed cardiac arrest and, despite CPR, she passed away.</div></div><div><h3>DISCUSSION</h3><div>Patients undergoing allogeneic HSCT develop profound immunosuppression, predisposing them to opportunistic infections with high mortality. COVİD-19, which caused a global pandemic in 2020, has
{"title":"A CASE OF COVID-19 PNEUMONIA DEVELOPING DURING ALLOGENEIC STEM CELL TRANSPLANTATION IN A PATIENT WITH ACUTE MYELOID LEUKEMIA","authors":"Yakup ÜNSAL , Muhammed MURATI , Güler DELİBALTA , Serdar Bedii OMAY","doi":"10.1016/j.htct.2025.106154","DOIUrl":"10.1016/j.htct.2025.106154","url":null,"abstract":"<div><h3>Objective</h3><div>Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative therapeutic modality for patients with acute myeloid leukemia (AML)<sup>1</sup>. Patients undergoing HSCT are highly susceptible to SARS-CoV-2 infection due to profound immunosuppression, delayed immune reconstitution, and graft-versus-host disease (GVHD) prophylaxis<sup>2</sup>,⁴. Covid-19 infection in HSCT recipients, particularly during the early post-transplant period, has been associated with high morbidity and mortality⁵. Studies have demonstrated increased mortality in patients diagnosed with Covid-19 during HSCT, especially in the early phase⁶. Herein, we present a case of Covid-19 pneumonia that developed during allogeneic HSCT with myeloablative conditioning in a patient with high-risk AML.</div></div><div><h3>Case report</h3><div>Our patient was a 33-year-old female diagnosed with AML in March 2025. Following diagnosis, she received induction therapy with the 3+7 regimen (Idarubicin + Cytarabine). As remission was not achieved, she was administered FLAG-Mito as reinduction therapy. Despite two cycles of induction, no response was obtained, and the patient was considered refractory AML; thus, allogeneic transplantation was planned. During the pre-transplant period, she received two cycles of azacitidine + venetoclax. On July 21, 2025, myeloablative conditioning (Fludarabine + Treosulfan) was initiated. During conditioning, her caregiver developed upper respiratory tract infection symptoms and tested positive for Covid-19. Since the patient was asymptomatic, the transplantation procedure was continued. On July 28, 2025, she underwent allogeneic transplantation from her HLA-matched sibling donor. GVHD prophylaxis consisted of CsA + MTX, and voriconazole was given for antifungal prophylaxis. On day +3 post-transplant, the patient developed fever (38°C) and was treated as febrile neutropenia with broad-spectrum antibiotics (Meropenem). SARS-CoV-2 PCR testing was positive. Initially, she presented with mild symptoms, but one week after positivity, chest imaging revealed diffuse pulmonary infiltrates consistent with Covid-19 pneumonia (Figure-1). Despite broad-spectrum antibiotics, she required high-dose corticosteroids and a single dose of tocilizumab (400 mg) for cytokine release syndrome. Oxygen support was initiated. Ten days after pneumonia diagnosis, respiratory distress worsened, and she was admitted to the intensive care unit. On day +18, CPAP was initiated. Neutrophil engraftment was achieved on day +19. However, despite non-invasive respiratory support, progressive respiratory failure necessitated intubation. Shortly after intubation, the patient developed cardiac arrest and, despite CPR, she passed away.</div></div><div><h3>DISCUSSION</h3><div>Patients undergoing allogeneic HSCT develop profound immunosuppression, predisposing them to opportunistic infections with high mortality. COVİD-19, which caused a global pandemic in 2020, has ","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106154"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145796979","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106148
Bengü MACİT, Arzu AKYAY, Yurday ÖNCÜL
<div><div>Wiskott-Aldrich Syndrome (WAS) is an X-linked disorder characterized by severe thrombocytopenia, eczema, humoral and cellular immunodeficiency, and an increased susceptibility to lymphoid malignancies. The milder form is X-linked thrombocytopenia (XLT), characterized by persistent thrombocytopenia with minimal or no signs of eczema or immunodeficiency.</div><div>An eight-year-old male patient was admitted to the hospital at six months of age with complaints of coughing and wheezing. Bone marrow aspiration was performed after thrombocytopenia was detected in a complete blood count, and the bone marrow was interpreted as technically hypocellular. Immun thrombocytopenia was suspected, and follow-up was recommended. Since he had no bleeding, he did not return to our clinic. When the patient was scheduled circumcision at the age of seven, his platelet count was 30,000/mm3, so he was referred to our clinic from anesthesia.</div><div>In his medical history, he had severe eczema as a baby, which later improved, and he has no history of bleeding.</div><div>In his family history, his uncles also had low platelet counts, and three of his uncles underwent splenectomy for this reason, after which their platelet counts returned to normal.</div><div>On physical examination, the patient had no signs of dermatitis, petechiae, purpura, or ecchymosis. The liver and spleen were palpable at 2 cm.</div><div>Laboratory tests: Hgb: 12.9 g/dl, Htc: 36.7%, white blood cells: 12,360/mm3, platelets: 30,000/mm3, MPV: 9.1 fl (normal), and platelet size appeared normal in the peripheral smear.Sedimentation was normal, and immunoglobulin values were normal for age.</div><div>Based on these findings, a preliminary diagnosis of XLT was considered, and WAS genetics were sent. In the WAS gene, a c.223G>A (p.V75M) hemizygous mutation was detected. The patient was diagnosed with XLT based on clinical findings and this mutation in the WAS gene. Eltrombopag treatment was initiated but was ineffective, so a splenectomy was performed.Subsequently, the platelet count reached 323,000/mm3. No decrease was observed during follow-up.</div><div>This situation can cause confusion with immune thrombocytopenia (ITP) and delay the diagnosis of XLT. Since XLT patients present with a milder clinical picture than classic WAS, treatment selection should be based on the patient's clinical presentation. Studies have shown that IVIG or antibiotic prophylaxis has no effect on the frequency or severity of infections in these patients. Since our patient did not have a history of frequent or severe infections, we did not initiate these treatments. Studies have shown that the incidence of bleeding decreases significantly after splenectomy, but the incidence of infection increases. Our patient's platelet counts returned to normal after splenectomy. To reduce the frequency of infections, we administered encapsulated bacterial vaccines and started penicillin prophylaxis for our patient. Due to the p
{"title":"A CASE OF X-LINKED THROMBOCYTOPENIA CONFUSED WITH IMMUNE THROMBOCYTOPENIA","authors":"Bengü MACİT, Arzu AKYAY, Yurday ÖNCÜL","doi":"10.1016/j.htct.2025.106148","DOIUrl":"10.1016/j.htct.2025.106148","url":null,"abstract":"<div><div>Wiskott-Aldrich Syndrome (WAS) is an X-linked disorder characterized by severe thrombocytopenia, eczema, humoral and cellular immunodeficiency, and an increased susceptibility to lymphoid malignancies. The milder form is X-linked thrombocytopenia (XLT), characterized by persistent thrombocytopenia with minimal or no signs of eczema or immunodeficiency.</div><div>An eight-year-old male patient was admitted to the hospital at six months of age with complaints of coughing and wheezing. Bone marrow aspiration was performed after thrombocytopenia was detected in a complete blood count, and the bone marrow was interpreted as technically hypocellular. Immun thrombocytopenia was suspected, and follow-up was recommended. Since he had no bleeding, he did not return to our clinic. When the patient was scheduled circumcision at the age of seven, his platelet count was 30,000/mm3, so he was referred to our clinic from anesthesia.</div><div>In his medical history, he had severe eczema as a baby, which later improved, and he has no history of bleeding.</div><div>In his family history, his uncles also had low platelet counts, and three of his uncles underwent splenectomy for this reason, after which their platelet counts returned to normal.</div><div>On physical examination, the patient had no signs of dermatitis, petechiae, purpura, or ecchymosis. The liver and spleen were palpable at 2 cm.</div><div>Laboratory tests: Hgb: 12.9 g/dl, Htc: 36.7%, white blood cells: 12,360/mm3, platelets: 30,000/mm3, MPV: 9.1 fl (normal), and platelet size appeared normal in the peripheral smear.Sedimentation was normal, and immunoglobulin values were normal for age.</div><div>Based on these findings, a preliminary diagnosis of XLT was considered, and WAS genetics were sent. In the WAS gene, a c.223G>A (p.V75M) hemizygous mutation was detected. The patient was diagnosed with XLT based on clinical findings and this mutation in the WAS gene. Eltrombopag treatment was initiated but was ineffective, so a splenectomy was performed.Subsequently, the platelet count reached 323,000/mm3. No decrease was observed during follow-up.</div><div>This situation can cause confusion with immune thrombocytopenia (ITP) and delay the diagnosis of XLT. Since XLT patients present with a milder clinical picture than classic WAS, treatment selection should be based on the patient's clinical presentation. Studies have shown that IVIG or antibiotic prophylaxis has no effect on the frequency or severity of infections in these patients. Since our patient did not have a history of frequent or severe infections, we did not initiate these treatments. Studies have shown that the incidence of bleeding decreases significantly after splenectomy, but the incidence of infection increases. Our patient's platelet counts returned to normal after splenectomy. To reduce the frequency of infections, we administered encapsulated bacterial vaccines and started penicillin prophylaxis for our patient. Due to the p","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106148"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145797060","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106164
Birol Güvenç
<div><h3>Introduction</h3><div>Follicular lymphoma (FL) grade 1–2 typically behaves indolently and is often managed with watchful waiting when tumor burden is low. However, moderately high FDG uptake on PET-CT may suggest biological heterogeneity or incipient transformation despite low-grade histology, creating a management dilemma. We report a patient with biopsy-proven FL grade 1–2 and unexpectedly “hot” PET signals, illustrating decision points between immediate therapy and surveillance.</div></div><div><h3>Methods</h3><div>Single-patient case report. We reviewed clinical data, laboratory tests, excisional lymph-node histology with immunohistochemistry (IHC), bone marrow (BM) evaluation, and whole-body PET-CT at diagnosis. Management decisions were based on symptoms, tumor burden, and longitudinal imaging.</div></div><div><h3>Results</h3><div>A 53-year-old woman presented with a painless, mobile left axillary mass detected 2 months earlier. She denied fever, drenching night sweats, or weight loss. Physical exam revealed a ∼3 cm left axillary node; no hepatosplenomegaly or other palpable lymphadenopathy.</div><div>PET-CT demonstrated a <strong>30 × 22 mm</strong> left axillary node with <strong>SUVmax 9.12</strong>, additional mediastinal paraaortic/aortopulmonary nodes (<strong>SUVmax 5.89</strong>), and tiny bilateral apical lung nodules with low uptake. The liver contained a <strong>15 × 12 mm</strong> hypodense lesion with faint FDG avidity and mild hepatomegaly; spleen and adrenals were normal; bone involvement was absent.</div><div>Excisional node biopsy showed <strong>classical FL, grade 1–2</strong>. IHC: <strong>CD20+, CD23+, CD5–, Cyclin D1–; Ki-67 ≈10%</strong>. BM aspirate/biopsy exhibited normal hematopoiesis with <strong>no lymphoma infiltration</strong> (reticulin 0/4; amyloid negative). Baseline blood counts and biochemistry were within reference limits except for a mildly elevated LDH.</div><div>Composite staging favored <strong>advanced-stage (IIIA–IIIB) FL</strong> owing to mediastinal involvement and hepatomegaly, yet <strong>clinical tumor burden was low</strong>: solitary bulky node absent, no B symptoms, preserved counts, and no organ compromise.</div><div>Given the discrepancy—<strong>indolent histology with relatively high axillary SUV</strong>—management options were discussed. Because transformation was not proven (low Ki-67, no high-grade features on biopsy, and no PET focus >10 with structural suspicion elsewhere), we selected <strong>watchful waiting</strong> with close clinical and PET/CT surveillance, reserving therapy for symptomatic progression, GELF high-tumor-burden criteria, rising SUVs or node growth, or any histologic evidence of transformation (repeat biopsy triggered by interval changes). Single-agent rituximab or R-based chemoimmunotherapy would be considered if progression occurs.</div></div><div><h3>Discussion</h3><div>This case highlights a <strong>clinic–radiologic mismatch</strong>: low-grade FL
{"title":"Indolent Follicular Lymphoma with “Hot” PET: A Clinic–Radiologic Mismatch That Challenges Early Treatment vs Watchful Waiting","authors":"Birol Güvenç","doi":"10.1016/j.htct.2025.106164","DOIUrl":"10.1016/j.htct.2025.106164","url":null,"abstract":"<div><h3>Introduction</h3><div>Follicular lymphoma (FL) grade 1–2 typically behaves indolently and is often managed with watchful waiting when tumor burden is low. However, moderately high FDG uptake on PET-CT may suggest biological heterogeneity or incipient transformation despite low-grade histology, creating a management dilemma. We report a patient with biopsy-proven FL grade 1–2 and unexpectedly “hot” PET signals, illustrating decision points between immediate therapy and surveillance.</div></div><div><h3>Methods</h3><div>Single-patient case report. We reviewed clinical data, laboratory tests, excisional lymph-node histology with immunohistochemistry (IHC), bone marrow (BM) evaluation, and whole-body PET-CT at diagnosis. Management decisions were based on symptoms, tumor burden, and longitudinal imaging.</div></div><div><h3>Results</h3><div>A 53-year-old woman presented with a painless, mobile left axillary mass detected 2 months earlier. She denied fever, drenching night sweats, or weight loss. Physical exam revealed a ∼3 cm left axillary node; no hepatosplenomegaly or other palpable lymphadenopathy.</div><div>PET-CT demonstrated a <strong>30 × 22 mm</strong> left axillary node with <strong>SUVmax 9.12</strong>, additional mediastinal paraaortic/aortopulmonary nodes (<strong>SUVmax 5.89</strong>), and tiny bilateral apical lung nodules with low uptake. The liver contained a <strong>15 × 12 mm</strong> hypodense lesion with faint FDG avidity and mild hepatomegaly; spleen and adrenals were normal; bone involvement was absent.</div><div>Excisional node biopsy showed <strong>classical FL, grade 1–2</strong>. IHC: <strong>CD20+, CD23+, CD5–, Cyclin D1–; Ki-67 ≈10%</strong>. BM aspirate/biopsy exhibited normal hematopoiesis with <strong>no lymphoma infiltration</strong> (reticulin 0/4; amyloid negative). Baseline blood counts and biochemistry were within reference limits except for a mildly elevated LDH.</div><div>Composite staging favored <strong>advanced-stage (IIIA–IIIB) FL</strong> owing to mediastinal involvement and hepatomegaly, yet <strong>clinical tumor burden was low</strong>: solitary bulky node absent, no B symptoms, preserved counts, and no organ compromise.</div><div>Given the discrepancy—<strong>indolent histology with relatively high axillary SUV</strong>—management options were discussed. Because transformation was not proven (low Ki-67, no high-grade features on biopsy, and no PET focus >10 with structural suspicion elsewhere), we selected <strong>watchful waiting</strong> with close clinical and PET/CT surveillance, reserving therapy for symptomatic progression, GELF high-tumor-burden criteria, rising SUVs or node growth, or any histologic evidence of transformation (repeat biopsy triggered by interval changes). Single-agent rituximab or R-based chemoimmunotherapy would be considered if progression occurs.</div></div><div><h3>Discussion</h3><div>This case highlights a <strong>clinic–radiologic mismatch</strong>: low-grade FL","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106164"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145796515","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106130
Neslihan MANDACI ŞANLI
<div><h3>Objective</h3><div>Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is vital in the treatment of high-risk hematologic cancers. Due to the immune system reconstitution process in the post-transplant period, infections are a leading cause of mortality and morbidity. Therefore, we aimed to investigate the efficacy of granulocyte transfusion (GT) therapy in patients who developed febrile neutropenia during allo-HSCT</div></div><div><h3>Methodology</h3><div>This retrospective study included 22 patients who underwent allo-HSCT at the Erciyes University Bone Marrow Transplantation Unit between January 2016 and January 2024 and developed febrile neutropenia. Patient characteristics were recorded. GT was administered to patients with an absolute neutrophil count (ANC) <0.5 × 10<sup>3</sup>/µL for at least three days, evidence of bacterial and/or fungal infection, and no response to appropriate antimicrobials for at least 48 hours.</div></div><div><h3>Results</h3><div>The median age was 42 years (min-max, 19-66 years). The majority of patients were diagnosed with acute myeloid leukemia (AML) (50%)(11/22). The median CRP value was 168.5 mg/dl (min-max, 31.1-360 mg/dl). In 40.9 % of patients who received GT, their primary disease was in complete remission, while in 59.1 %, their primary disease was relapse. The infection etiologies included pneumonia (n=5), sepsis (n=2), pneumonia and sepsis (n=11), pneumonia + sepsis + catheter-associated infection (n=4), catheter-associated infection + mucositis (n=1), and abscess (n=1). Each patient received a median of 3 GTs (min-max, 1-6). The median transfused granulocyte dose per transfusion was 3.5 × 10<sup>10</sup> (min-max, 0.8-9.4 × 10<sup>10</sup>). The median dose transfused, calculated based on the recipient's body weight, was 5.1 × 10<sup>8</sup>/kg (min-max, 0.8-17 × 10<sup>8</sup>/kg). On average, the median number of granulocytes transfused per patient was 5.3 × 10<sup>8</sup>/kg (min-max, 1.9-11.3 × 10<sup>8</sup>/kg). The median time from HSCT to the first GT was 192 days (min-max, 50-795 days). The median duration of fever before GT was three days (min-max, 2-6 days), and the time until the fever defervescence was 2 days (min-max, 1-5 days). The median duration of neutropenia before GT is 25 days (min-max, 8-30 days).</div><div>After GTX treatment, A favorable response was observed in 16 of 24 infection episodes (66.7%) regarding the resolution of infections. In 4 of the 8 infection episodes where the infection did not resolve, the patient also had a relapse of the disease. In 5 of 12 infection episodes that required intensive care, the need for intensive care was eliminated after GT. A statistically significant difference was found between the time of GT initiation and the ANC, TLC, and PLT counts on the fourth-day post-GT (p =0.001, p=0.001, p=0.003, separately for ANC, TLC, and PLT). The median follow-up in our cohort of patients is 600 days. The 30-day and 100-day OS were 6
{"title":"EFFECTİVE TREATMENT OF LONG-TERM NEUTROPENİA AND SEPSİS WİTH GRANULOCYTE TRANSFUSİON İN PATİENTS UNDERGOİNG ALLOGENEİC HEMATOPOİETİC STEM CELL TRANSPLANTATİON","authors":"Neslihan MANDACI ŞANLI","doi":"10.1016/j.htct.2025.106130","DOIUrl":"10.1016/j.htct.2025.106130","url":null,"abstract":"<div><h3>Objective</h3><div>Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is vital in the treatment of high-risk hematologic cancers. Due to the immune system reconstitution process in the post-transplant period, infections are a leading cause of mortality and morbidity. Therefore, we aimed to investigate the efficacy of granulocyte transfusion (GT) therapy in patients who developed febrile neutropenia during allo-HSCT</div></div><div><h3>Methodology</h3><div>This retrospective study included 22 patients who underwent allo-HSCT at the Erciyes University Bone Marrow Transplantation Unit between January 2016 and January 2024 and developed febrile neutropenia. Patient characteristics were recorded. GT was administered to patients with an absolute neutrophil count (ANC) <0.5 × 10<sup>3</sup>/µL for at least three days, evidence of bacterial and/or fungal infection, and no response to appropriate antimicrobials for at least 48 hours.</div></div><div><h3>Results</h3><div>The median age was 42 years (min-max, 19-66 years). The majority of patients were diagnosed with acute myeloid leukemia (AML) (50%)(11/22). The median CRP value was 168.5 mg/dl (min-max, 31.1-360 mg/dl). In 40.9 % of patients who received GT, their primary disease was in complete remission, while in 59.1 %, their primary disease was relapse. The infection etiologies included pneumonia (n=5), sepsis (n=2), pneumonia and sepsis (n=11), pneumonia + sepsis + catheter-associated infection (n=4), catheter-associated infection + mucositis (n=1), and abscess (n=1). Each patient received a median of 3 GTs (min-max, 1-6). The median transfused granulocyte dose per transfusion was 3.5 × 10<sup>10</sup> (min-max, 0.8-9.4 × 10<sup>10</sup>). The median dose transfused, calculated based on the recipient's body weight, was 5.1 × 10<sup>8</sup>/kg (min-max, 0.8-17 × 10<sup>8</sup>/kg). On average, the median number of granulocytes transfused per patient was 5.3 × 10<sup>8</sup>/kg (min-max, 1.9-11.3 × 10<sup>8</sup>/kg). The median time from HSCT to the first GT was 192 days (min-max, 50-795 days). The median duration of fever before GT was three days (min-max, 2-6 days), and the time until the fever defervescence was 2 days (min-max, 1-5 days). The median duration of neutropenia before GT is 25 days (min-max, 8-30 days).</div><div>After GTX treatment, A favorable response was observed in 16 of 24 infection episodes (66.7%) regarding the resolution of infections. In 4 of the 8 infection episodes where the infection did not resolve, the patient also had a relapse of the disease. In 5 of 12 infection episodes that required intensive care, the need for intensive care was eliminated after GT. A statistically significant difference was found between the time of GT initiation and the ANC, TLC, and PLT counts on the fourth-day post-GT (p =0.001, p=0.001, p=0.003, separately for ANC, TLC, and PLT). The median follow-up in our cohort of patients is 600 days. The 30-day and 100-day OS were 6","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106130"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145796547","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106205
Damla Ortaboz
<div><div>Mantle cell lymphoma (MCL) is a rare and aggressive subtype of non-Hodgkin lymphoma (NHL) characterized by the overexpression of cyclin D1 due to the chromosomal translocation t(11;14)(q13;q32). Despite advances in therapeutic approaches, MCL remains a significant clinical challenge, particularly in relapsed and refractory (R/R) cases. Relapse occurs when the disease reappears after an initial response to therapy, while refractory MCL refers to cases where the disease fails to respond adequately to standard treatment regimens. Both conditions are associated with poor prognosis and limited treatment options, reflecting the need for novel therapeutic strategies.</div><div>Relapsed MCL is characterized by clonal evolution and the emergence of more aggressive phenotypes, including resistance to previously administered therapies. Refractory cases, on the other hand, exhibit intrinsic or acquired resistance mechanisms, such as mutations in the B-cell receptor (BCR) signaling pathway, TP53 abnormalities, and alterations in DNA damage response genes.</div><div>Recent therapeutic advances have improved outcomes for R/R MCL patients. Targeted therapies, including Bruton’s tyrosine kinase (BTK) inhibitors such as ibrutinib, acalabrutinib, and zanubrutinib, have demonstrated significant efficacy by disrupting BCR signaling. Ibrutinib, the first BTK inhibitor approved for R/R MCL, has shown durable responses in clinical trials, although resistance to BTK inhibitors is a growing concern. Lenalidomide, an immunomodulatory agent, and venetoclax, a BCL-2 inhibitor, have also shown promise in heavily pretreated patients. Furthermore, chimeric antigen receptor (CAR) T-cell therapy targeting CD19, such as brexucabtagene autoleucel, represents a groundbreaking approach for patients with chemorefractory disease. While these therapies offer hope, their application is often limited by adverse events, accessibility, and high costs.</div><div>Biological heterogeneity within MCL further complicates the management of R/R cases. The proliferation index (Ki-67), TP53 mutation status, and the presence of blastoid or pleomorphic variants are critical prognostic factors influencing treatment decisions. Additionally, the integration of next-generation sequencing (NGS) and molecular profiling enables the identification of actionable mutations and pathways, paving the way for personalized medicine.</div><div>Despite these advancements, challenges remain in optimizing the sequencing of therapies, managing toxicities, and overcoming resistance. Clinical trials continue to explore novel agents, including bispecific antibodies, proteasome inhibitors, and checkpoint inhibitors, as well as combination strategies to enhance efficacy and minimize resistance. Moreover, the role of minimal residual disease (MRD) monitoring in guiding treatment remains an area of active investigation.</div><div>In conclusion, relapsed and refractory MCL represents a complex clinical entity with sign
{"title":"RELAPS/REFRACTORY MANTLE CELL LYMPHOMA TREATMENT","authors":"Damla Ortaboz","doi":"10.1016/j.htct.2025.106205","DOIUrl":"10.1016/j.htct.2025.106205","url":null,"abstract":"<div><div>Mantle cell lymphoma (MCL) is a rare and aggressive subtype of non-Hodgkin lymphoma (NHL) characterized by the overexpression of cyclin D1 due to the chromosomal translocation t(11;14)(q13;q32). Despite advances in therapeutic approaches, MCL remains a significant clinical challenge, particularly in relapsed and refractory (R/R) cases. Relapse occurs when the disease reappears after an initial response to therapy, while refractory MCL refers to cases where the disease fails to respond adequately to standard treatment regimens. Both conditions are associated with poor prognosis and limited treatment options, reflecting the need for novel therapeutic strategies.</div><div>Relapsed MCL is characterized by clonal evolution and the emergence of more aggressive phenotypes, including resistance to previously administered therapies. Refractory cases, on the other hand, exhibit intrinsic or acquired resistance mechanisms, such as mutations in the B-cell receptor (BCR) signaling pathway, TP53 abnormalities, and alterations in DNA damage response genes.</div><div>Recent therapeutic advances have improved outcomes for R/R MCL patients. Targeted therapies, including Bruton’s tyrosine kinase (BTK) inhibitors such as ibrutinib, acalabrutinib, and zanubrutinib, have demonstrated significant efficacy by disrupting BCR signaling. Ibrutinib, the first BTK inhibitor approved for R/R MCL, has shown durable responses in clinical trials, although resistance to BTK inhibitors is a growing concern. Lenalidomide, an immunomodulatory agent, and venetoclax, a BCL-2 inhibitor, have also shown promise in heavily pretreated patients. Furthermore, chimeric antigen receptor (CAR) T-cell therapy targeting CD19, such as brexucabtagene autoleucel, represents a groundbreaking approach for patients with chemorefractory disease. While these therapies offer hope, their application is often limited by adverse events, accessibility, and high costs.</div><div>Biological heterogeneity within MCL further complicates the management of R/R cases. The proliferation index (Ki-67), TP53 mutation status, and the presence of blastoid or pleomorphic variants are critical prognostic factors influencing treatment decisions. Additionally, the integration of next-generation sequencing (NGS) and molecular profiling enables the identification of actionable mutations and pathways, paving the way for personalized medicine.</div><div>Despite these advancements, challenges remain in optimizing the sequencing of therapies, managing toxicities, and overcoming resistance. Clinical trials continue to explore novel agents, including bispecific antibodies, proteasome inhibitors, and checkpoint inhibitors, as well as combination strategies to enhance efficacy and minimize resistance. Moreover, the role of minimal residual disease (MRD) monitoring in guiding treatment remains an area of active investigation.</div><div>In conclusion, relapsed and refractory MCL represents a complex clinical entity with sign","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106205"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145796984","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106144
Berra Nur İşçi, Birol Güvenç
<div><h3>Introduction</h3><div>Follicular lymphoma (FL) is a germinal-center B-cell neoplasm that typically expresses CD10/BCL6 and lacks CD5. CD5-positive FL is uncommon and may mimic mantle cell lymphoma (MCL), creating critical diagnostic and therapeutic implications. We report an older male with a history of resected cutaneous squamous cell carcinoma (SCC) who presented with a new inguinal lymphadenopathy ultimately diagnosed as FL grade 3A, despite an atypical CD5-positive flow phenotype.</div></div><div><h3>Methods</h3><div>This single-patient case report summarizes clinical data, ^18F-FDG PET/CT findings, flow cytometry, histopathology, and management. PET/CT was performed for staging. Lymph node excision provided tissue for histology and immunohistochemistry (IHC). Peripheral blood flow cytometry used a chronic lymphocytic leukemia (CLL) panel. Bone-marrow aspirate/biopsy were attempted for staging.</div></div><div><h3>Results</h3><div>A 70-year-old man with previously excised cutaneous SCC (disease-free) was evaluated for new left inguinal lymphadenopathy. PET/CT demonstrated a metabolically active left inguinal node (∼17 × 15 mm, SUVmax 12.18) with no other pathologic uptake in the neck, chest, liver, spleen, or adrenals. A posteromedial femoral hypodense nodule (∼20 × 15 mm) and a subcutaneous scapular lesion (∼26 × 20 mm) showed no increased FDG uptake.</div><div>Excisional biopsy of the inguinal node revealed non-Hodgkin lymphoma, classic follicular lymphoma, grade 3A (WHO 2016). IHC showed CD20+, BCL6+, BCL2+, CD10+, CD21 positivity in follicular dendritic cells, CD3–, and Ki-67 ∼25%.</div><div>Peripheral blood flow cytometry demonstrated B-cell markers with CD5 high (∼71%), CD23 low/negative (∼16%), CD10 low (∼4%), CD43 (∼78%), and mild kappa predominance; findings raised concern for MCL. However, nodal histomorphology with CD10/BCL6 positivity supported FL. Bone-marrow aspirate was suboptimal (particle-poor), and iron score could not be assessed; marrow staging biopsy was planned.</div><div>Given grade 3A FL and PET-positive nodal disease, the multidisciplinary tumor board recommended R-CHOP chemoimmunotherapy. Additional work-up (cyclin D1/SOX11 IHC and/or t(11;14) FISH) was advised to definitively exclude MCL due to CD5 expression.</div></div><div><h3>Discussion</h3><div>This case highlights two challenges: (1) Dual malignancy in the same patient (prior SCC, now FL) and (2) immunophenotypic discordance between flow cytometry and histology. CD5-positive FL is rare and easily misclassified as MCL; mislabeling could alter therapy (e.g., bendamustine-rituximab vs R-CHOP and consideration of BTK inhibitors in MCL). When flow suggests MCL but node histology/IHC favors FL, tissue-based cyclin D1/SOX11 and t(11;14) are decisive. Suboptimal marrow underscores the need for core biopsy to complete staging. The absence of systemic FDG-avid disease supports localized nodal involvement at presentation.</div></div><div><h3>Conclusion</h3><div>
{"title":"CD5-positive Grade 3A Follicular Lymphoma Following Resected Cutaneous Squamous Cell Carcinoma: A Case Report","authors":"Berra Nur İşçi, Birol Güvenç","doi":"10.1016/j.htct.2025.106144","DOIUrl":"10.1016/j.htct.2025.106144","url":null,"abstract":"<div><h3>Introduction</h3><div>Follicular lymphoma (FL) is a germinal-center B-cell neoplasm that typically expresses CD10/BCL6 and lacks CD5. CD5-positive FL is uncommon and may mimic mantle cell lymphoma (MCL), creating critical diagnostic and therapeutic implications. We report an older male with a history of resected cutaneous squamous cell carcinoma (SCC) who presented with a new inguinal lymphadenopathy ultimately diagnosed as FL grade 3A, despite an atypical CD5-positive flow phenotype.</div></div><div><h3>Methods</h3><div>This single-patient case report summarizes clinical data, ^18F-FDG PET/CT findings, flow cytometry, histopathology, and management. PET/CT was performed for staging. Lymph node excision provided tissue for histology and immunohistochemistry (IHC). Peripheral blood flow cytometry used a chronic lymphocytic leukemia (CLL) panel. Bone-marrow aspirate/biopsy were attempted for staging.</div></div><div><h3>Results</h3><div>A 70-year-old man with previously excised cutaneous SCC (disease-free) was evaluated for new left inguinal lymphadenopathy. PET/CT demonstrated a metabolically active left inguinal node (∼17 × 15 mm, SUVmax 12.18) with no other pathologic uptake in the neck, chest, liver, spleen, or adrenals. A posteromedial femoral hypodense nodule (∼20 × 15 mm) and a subcutaneous scapular lesion (∼26 × 20 mm) showed no increased FDG uptake.</div><div>Excisional biopsy of the inguinal node revealed non-Hodgkin lymphoma, classic follicular lymphoma, grade 3A (WHO 2016). IHC showed CD20+, BCL6+, BCL2+, CD10+, CD21 positivity in follicular dendritic cells, CD3–, and Ki-67 ∼25%.</div><div>Peripheral blood flow cytometry demonstrated B-cell markers with CD5 high (∼71%), CD23 low/negative (∼16%), CD10 low (∼4%), CD43 (∼78%), and mild kappa predominance; findings raised concern for MCL. However, nodal histomorphology with CD10/BCL6 positivity supported FL. Bone-marrow aspirate was suboptimal (particle-poor), and iron score could not be assessed; marrow staging biopsy was planned.</div><div>Given grade 3A FL and PET-positive nodal disease, the multidisciplinary tumor board recommended R-CHOP chemoimmunotherapy. Additional work-up (cyclin D1/SOX11 IHC and/or t(11;14) FISH) was advised to definitively exclude MCL due to CD5 expression.</div></div><div><h3>Discussion</h3><div>This case highlights two challenges: (1) Dual malignancy in the same patient (prior SCC, now FL) and (2) immunophenotypic discordance between flow cytometry and histology. CD5-positive FL is rare and easily misclassified as MCL; mislabeling could alter therapy (e.g., bendamustine-rituximab vs R-CHOP and consideration of BTK inhibitors in MCL). When flow suggests MCL but node histology/IHC favors FL, tissue-based cyclin D1/SOX11 and t(11;14) are decisive. Suboptimal marrow underscores the need for core biopsy to complete staging. The absence of systemic FDG-avid disease supports localized nodal involvement at presentation.</div></div><div><h3>Conclusion</h3><div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106144"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145796975","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106153
Tuba SARICI , Süleyman ARSLAN
Introduction
Mucormycosis is an opportunistic fungal infection with rapid progression and high mortality, typically occurring in patients with hematologic malignancies, diabetes mellitus, organ transplantation, or prolonged immunosuppression. Clinically, the most common forms are rhino-cerebral, pulmonary, cutaneous, and gastrointestinal involvement. Rhino-cerebral mucormycosis often originates in the paranasal sinuses and may extend to the orbit and brain. Oral mucormycosis is less common and usually presents with maxillary bone necrosis and palatal perforation. Early diagnosis and appropriate antifungal therapy are critical for improving prognosis. In this report, we present a case of newly diagnosed acute myeloid leukemia (AML) who developed rhino-orbito-cerebral mucormycosis involving the maxilla following chemotherapy.
Case Report
A 52-year-old male patient was admitted to the hematology outpatient clinic with complaints of epistaxis and fatigue. Laboratory evaluation revealed pancytopenia, and peripheral smear, bone marrow aspiration, and flow cytometry confirmed the diagnosis of acute myeloid leukemia (AML). The patient received induction chemotherapy with daunorubicin (60 mg/m2 for 3 days) and cytarabine (100 mg/m2 for 7 days).
In the second week of treatment, the patient developed pain in the left maxillary region and was referred to the Faculty of Dentistry, Inönü University. Oral and radiological examination (Figure 1)revealed a fixed dental bridge with good marginal adaptation.The prosthetic device was removed, and no pathology was observed in the teeth or surrounding mucosa, Türkiye.
{"title":"Oral and Maxillary Mucormycosis in a Patient with Acute Myeloid Leukemia: A Rare Case Report","authors":"Tuba SARICI , Süleyman ARSLAN","doi":"10.1016/j.htct.2025.106153","DOIUrl":"10.1016/j.htct.2025.106153","url":null,"abstract":"<div><h3>Introduction</h3><div>Mucormycosis is an opportunistic fungal infection with rapid progression and high mortality, typically occurring in patients with hematologic malignancies, diabetes mellitus, organ transplantation, or prolonged immunosuppression. Clinically, the most common forms are rhino-cerebral, pulmonary, cutaneous, and gastrointestinal involvement. Rhino-cerebral mucormycosis often originates in the paranasal sinuses and may extend to the orbit and brain. Oral mucormycosis is less common and usually presents with maxillary bone necrosis and palatal perforation. Early diagnosis and appropriate antifungal therapy are critical for improving prognosis. In this report, we present a case of newly diagnosed acute myeloid leukemia (AML) who developed rhino-orbito-cerebral mucormycosis involving the maxilla following chemotherapy.</div></div><div><h3>Case Report</h3><div>A 52-year-old male patient was admitted to the hematology outpatient clinic with complaints of epistaxis and fatigue. Laboratory evaluation revealed pancytopenia, and peripheral smear, bone marrow aspiration, and flow cytometry confirmed the diagnosis of acute myeloid leukemia (AML). The patient received induction chemotherapy with daunorubicin (60 mg/m<sup>2</sup> for 3 days) and cytarabine (100 mg/m<sup>2</sup> for 7 days).</div><div>In the second week of treatment, the patient developed pain in the left maxillary region and was referred to the Faculty of Dentistry, Inönü University. Oral and radiological examination (Figure 1)revealed a fixed dental bridge with good marginal adaptation.The prosthetic device was removed, and no pathology was observed in the teeth or surrounding mucosa, Türkiye.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 106153"},"PeriodicalIF":1.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145797061","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.htct.2025.106137
Berrak Çağla Şenol, Birol Güvenç
Introduction
Acute myeloid leukemia (AML) is a heterogeneous hematologic malignancy with diverse morphologic and immunophenotypic subtypes. The RAM (rapidly maturing) phenotype is a rare and poorly characterized variant, initially described in pediatric acute leukemia, but has also been identified in older adults (Wells et al., 2018). It is typically defined by CD45^dim, CD34–, CD117+, CD33++, and aberrant CD7 expression, with aggressive clinical behavior and poor prognosis (Nguyen et al., 2021). Here, we present a case of elderly-onset AML with RAM-like immunophenotypic features.
Methods
A 81-year-old female presented with pancytopenia and recurrent subdural hemorrhages. Flow cytometry revealed CD45^dim, CD34–, CD117+, CD33++, and aberrant CD7+ blasts, consistent with RAM-like AML. Cytogenetic analysis showed no recurrent AML-defining translocations by FISH. Comprehensive molecular testing, including FLT3, NPM1, and CEBPA, was negative. Clinical frailty assessment demonstrated a high CIRS score, limiting intensive treatment options.
Results
Bone marrow examination confirmed AML with RAM-like immunophenotype. Given the patient’s age, comorbidities, and recurrent intracranial hemorrhages, intensive induction chemotherapy was contraindicated. Supportive care and hypomethylating agent-based therapy were considered but deferred due to poor functional status and ongoing hemorrhagic risk. The patient remained under best supportive care, including transfusions and infection prophylaxis. Prognosis was explained to the family as extremely poor, consistent with published literature (Al-Kershi et al., 2023).
Discussion
RAM-like AML represents a high-risk immunophenotypic subset, characterized by treatment resistance and inferior outcomes (Wells et al., 2018). Most reported cases occur in children; however, adult and elderly cases are being increasingly recognized (Nguyen et al., 2021). This case highlights the diagnostic challenge and limited therapeutic options in elderly patients, particularly when performance status and comorbidities preclude intensive therapy. Early recognition through flow cytometry is essential for risk stratification and counseling.
Conclusion
We report an elderly female with AML exhibiting RAM-like phenotype, an aggressive and rare immunophenotypic variant. Awareness of this entity is important for hematologists, as it informs prognosis and guides therapeutic decision-making, even when curative approaches are not feasible.
急性髓性白血病（AML）是一种具有多种形态和免疫表型亚型的异质性血液恶性肿瘤。RAM（快速成熟）表型是一种罕见且特征不明确的变异，最初在儿科急性白血病中被描述，但也在老年人中被发现（Wells等人，2018）。它通常由CD45^dim、CD34 -、CD117+、CD33++和CD7异常表达定义，具有侵袭性临床行为和不良预后（Nguyen et al., 2021）。在这里，我们报告了一例具有ram样免疫表型特征的老年发病AML。方法一例81岁女性患者，以全血细胞减少伴复发性硬膜下出血为主。流式细胞术显示CD45^dim， CD34 -, CD117+， CD33+和CD7+异常，与ram样AML一致。细胞遗传学分析显示FISH未发现复发性aml定义易位。综合分子检测FLT3、NPM1、CEBPA均为阴性。临床虚弱评估显示高CIRS评分，限制了强化治疗的选择。结果骨髓检查证实急性髓性白血病具有ram样免疫表型。考虑到患者的年龄、合并症和复发性颅内出血，强化诱导化疗是禁忌。支持治疗和以低甲基化药物为基础的治疗被考虑，但由于功能状态不佳和持续的出血风险而推迟。患者仍在接受最佳支持治疗，包括输血和感染预防。向家属解释预后极差，与已发表的文献一致（al - kershi et al., 2023）。ram样AML是一种高风险的免疫表型亚群，其特点是治疗耐药和预后较差（Wells等，2018）。大多数报告的病例发生在儿童中；然而，越来越多的成年人和老年人病例得到认可（Nguyen et al., 2021）。该病例强调了老年患者的诊断挑战和有限的治疗选择，特别是当表现状况和合并症妨碍强化治疗时。通过流式细胞术进行早期识别对于风险分层和咨询至关重要。结论我们报告一例老年女性急性髓性白血病表现为ram样表型，这是一种侵袭性和罕见的免疫表型变异。意识到这种实体对血液学家很重要，因为它可以告知预后并指导治疗决策，即使在治疗方法不可行的情况下。
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