Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.015
Kemal Fıdan , Ali Ünal , Neslihan Mandacı Şanlı , Ebubekir Sıddık İpekçi
Case report: Introduction
Medulloblastoma is the most common malignant primary embryonal brain tumor in children and occurs in the cerebellum. Approximately 70% of patients are diagnosed before the age of 20. The disease is rare after the 4th decade of life. It originates from the brainstem and metastasizes to other brain tissue, ventricles and medulla spinalis via CSF. Metastasis to bone, bone marrow, lung or lymph nodes outside the CNS is a very rare condition.
Surgery, chemotherapy and radiotherapy are used in the treatment of medulloblastoma. In some patients (patients in the high-risk group, relapsed/refractory patients), autologous stem cell transplantation(ASCT) is performed following high-dose chemotherapy to increase survival rates.
Here, we will present a case of medulloblastoma in which we performed autologous stem cell transplantation in our center.
A 30-year-old male patient applied to the neurology clinic in May 2020 with complaints of headache, dizziness, nausea, vomiting and fainting. In the brain imaging, a 6 × 4 cm mass lesion was observed in the posterior fossa, located in the ventricle and causing compression symptoms (Cystic Astrocytoma? Medulloblastoma?). The patient underwent ventriculoperitoneal shunt and subtotal mass excision at the neurosurgery clinic. The biopsy pathology result was reported as medulloblastoma (classical type, p53 mutation positive). Chemotherapy was recommended by the oncology clinic, but the patient did not accept the treatment. In August 2020, the patient was given cranial RT and was subsequently followed without medication.
in June 2023 due to complaints of pain and weakness in both lower extremities, there was an intradural mass lesion (25 × 19 mm) obliterating the spinal cord at the T11-T12 level and extending to the extraspinal area, and a diffuse mass lesion within the spinal cord at the T10 level with a craniocaudal length of 17 mm. Mass excision as a result of pathology; It was reported as classical medulloblastoma (non-WNT/non-SHH group (grade 4)).
After the patient was given 2 courses of mini-ICE chemotherapy, a nearly complete response in the imaging. The patient was mobilized with G-CSF. In our center, the patient was performed autologous stem cell transplantation (6.55 × 106 /kg cells) with temozolamide (2 × 200mg/m2 on days -6,-5,-4), etoposide (100 mg/m2 on days -7,-6,-5,-4,-3,-2), thiotepa (300 mg/m2, on days -4,-3,-2) protocol in November 2023.The patient, who had neutrophil and platelet engraftment on the 10th day after transplantation, was discharged with outpatient clinic control.
Discussion and conclusion
Although the prognosis has improved in children with medulloblastoma, an estimated 20-30% will relapse following initial treatment (1). Re
{"title":"Autologous stem cell transplantation experience in an adult recurrent medulloblastoma patient: Case report","authors":"Kemal Fıdan , Ali Ünal , Neslihan Mandacı Şanlı , Ebubekir Sıddık İpekçi","doi":"10.1016/j.htct.2024.04.015","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.015","url":null,"abstract":"<div><h3>Case report: Introduction</h3><p>Medulloblastoma is the most common malignant primary embryonal brain tumor in children and occurs in the cerebellum. Approximately 70% of patients are diagnosed before the age of 20. The disease is rare after the 4th decade of life. It originates from the brainstem and metastasizes to other brain tissue, ventricles and medulla spinalis via CSF. Metastasis to bone, bone marrow, lung or lymph nodes outside the CNS is a very rare condition.</p><p>Surgery, chemotherapy and radiotherapy are used in the treatment of medulloblastoma. In some patients (patients in the high-risk group, relapsed/refractory patients), autologous stem cell transplantation(ASCT) is performed following high-dose chemotherapy to increase survival rates.</p><p>Here, we will present a case of medulloblastoma in which we performed autologous stem cell transplantation in our center.</p><p><strong><em>Key words:</em></strong> Medulloblastoma, autologous stem cell transplantation</p></div><div><h3>Case report</h3><p>A 30-year-old male patient applied to the neurology clinic in May 2020 with complaints of headache, dizziness, nausea, vomiting and fainting. In the brain imaging, a 6 × 4 cm mass lesion was observed in the posterior fossa, located in the ventricle and causing compression symptoms (Cystic Astrocytoma? Medulloblastoma?). The patient underwent ventriculoperitoneal shunt and subtotal mass excision at the neurosurgery clinic. The biopsy pathology result was reported as medulloblastoma (classical type, p53 mutation positive). Chemotherapy was recommended by the oncology clinic, but the patient did not accept the treatment. In August 2020, the patient was given cranial RT and was subsequently followed without medication.</p><p>in June 2023 due to complaints of pain and weakness in both lower extremities, there was an intradural mass lesion (25 × 19 mm) obliterating the spinal cord at the T11-T12 level and extending to the extraspinal area, and a diffuse mass lesion within the spinal cord at the T10 level with a craniocaudal length of 17 mm. Mass excision as a result of pathology; It was reported as classical medulloblastoma (non-WNT/non-SHH group (grade 4)).</p><p>After the patient was given 2 courses of mini-ICE chemotherapy, a nearly complete response in the imaging. The patient was mobilized with G-CSF. In our center, the patient was performed autologous stem cell transplantation (6.55 × 10<sup>6</sup> /kg cells) with temozolamide (2 × 200mg/m<sup>2</sup> on days -6,-5,-4), etoposide (100 mg/m<sup>2</sup> on days -7,-6,-5,-4,-3,-2), thiotepa (300 mg/m<sup>2</sup>, on days -4,-3,-2) protocol in November 2023.The patient, who had neutrophil and platelet engraftment on the 10th day after transplantation, was discharged with outpatient clinic control.</p></div><div><h3>Discussion and conclusion</h3><p>Although the prognosis has improved in children with medulloblastoma, an estimated 20-30% will relapse following initial treatment (1). Re","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S253113792400097X/pdfft?md5=24f2eb33639c79652e30054fb86fbeac&pid=1-s2.0-S253113792400097X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878979","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.003
Elmir Guluyev , Madad Abbasov , Azer Kerimov
Objective
Primary myelofibrosis (PMF) is a rare Ph chromosome-negative chronic myeloproliferative neoplasm characterized by the proliferation of atypical clonal megakaryocytes and fibrosis of the bone marrow. The activation of the JAK-STAT pathway plays a central role in the pathogenesis of the disease. The majority of patients with primary myelofibrosis have one of three main genetic mutations, including JAK2 V617F, CALR exon 9, or MPL W515. The clinical features of the disease are highly heterogeneous. Common symptoms and signs include fatigue, constitutional symptoms, itching, abdominal discomfort, bone pain, anemia, leukocytosis, thrombocytopenia, and splenomegaly. A number of clinical studies on the demographic and clinical features of myelofibrosis have been carried out in different countries. Detailed demographic and clinical characteristics of patients with BMF have not been thoroughly studied in Azerbaijan. The aim of our study was to characterize the demographic, clinical, and laboratory parameters of patients with primary myelofibrosis in Azerbaijan. All patients were registered at the Azerbaijan National Center for Hematology and Transfusion.
Methodology
A retrospective analysis was conducted on the demographic, clinical, and laboratory data of 131 patients diagnosed with PMF between January 1, 2011, and December 1, 2023. The diagnosis of all patients was revised according to the WHO 2016 criteria for PMF. The fibrosis of the bone marrow was assessed histologically according to the Thiele grading system. Ultrasound examination was used to assess splenomegaly, with a craniocaudal size of >14 cm being considered as splenomegaly. All data were collected from clinical records. This was a retrospective, observational, single-center study.
Results
A total of one hundred thirty-one (131) patients with primary myelofibrosis were analyzed. Of these, 65 (49.6%) were male. The median age of the patients was 57.5 years (range 19-80), with 9 (6.87%) patients being under 40 years of age. The median hemoglobin level was 10.7 g/dl (range 2.1-19.4), median white blood cell count was 12.86 × 10^12/l (range 0.45-121), median platelet count was 322 × 10^12/l (range 24-1940), and median LDH was 530 U/l (range 181-1586). Splenomegaly was detected in 96 patients, with an average spleen size (19.5 cm)reported. Fifty-one patients had Hgb < 10 g/dl. At the time of diagnosis, the pre-fibrotic stage was identified in the bone marrow examination of sixteen patients (17.8%). Splenomegaly was detected in 96 (91.4%) patients. Of the 66 patients who underwent genetic testing, 44 had a positive Jak2V617F mutation, 2 had a positive CALR mutation, and 1 had a positive MPL mutation.
Conclusion
Thus, this study has investigated the demographic, clinical, and laboratory characteristics of patients with primary myelofibrosis in Azerbaijan.
{"title":"RETROSPECTIVE ANALYSIS OF PRIMARY MYELOFIBROSIS PATIENTS IN AZERBAIJAN","authors":"Elmir Guluyev , Madad Abbasov , Azer Kerimov","doi":"10.1016/j.htct.2024.04.003","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.003","url":null,"abstract":"<div><h3>Objective</h3><p>Primary myelofibrosis (PMF) is a rare Ph chromosome-negative chronic myeloproliferative neoplasm characterized by the proliferation of atypical clonal megakaryocytes and fibrosis of the bone marrow. The activation of the JAK-STAT pathway plays a central role in the pathogenesis of the disease. The majority of patients with primary myelofibrosis have one of three main genetic mutations, including JAK2 V617F, CALR exon 9, or MPL W515. The clinical features of the disease are highly heterogeneous. Common symptoms and signs include fatigue, constitutional symptoms, itching, abdominal discomfort, bone pain, anemia, leukocytosis, thrombocytopenia, and splenomegaly. A number of clinical studies on the demographic and clinical features of myelofibrosis have been carried out in different countries. Detailed demographic and clinical characteristics of patients with BMF have not been thoroughly studied in Azerbaijan. The aim of our study was to characterize the demographic, clinical, and laboratory parameters of patients with primary myelofibrosis in Azerbaijan. All patients were registered at the Azerbaijan National Center for Hematology and Transfusion.</p></div><div><h3>Methodology</h3><p>A retrospective analysis was conducted on the demographic, clinical, and laboratory data of 131 patients diagnosed with PMF between January 1, 2011, and December 1, 2023. The diagnosis of all patients was revised according to the WHO 2016 criteria for PMF. The fibrosis of the bone marrow was assessed histologically according to the Thiele grading system. Ultrasound examination was used to assess splenomegaly, with a craniocaudal size of >14 cm being considered as splenomegaly. All data were collected from clinical records. This was a retrospective, observational, single-center study.</p></div><div><h3>Results</h3><p>A total of one hundred thirty-one (131) patients with primary myelofibrosis were analyzed. Of these, 65 (49.6%) were male. The median age of the patients was 57.5 years (range 19-80), with 9 (6.87%) patients being under 40 years of age. The median hemoglobin level was 10.7 g/dl (range 2.1-19.4), median white blood cell count was 12.86 × 10^12/l (range 0.45-121), median platelet count was 322 × 10^12/l (range 24-1940), and median LDH was 530 U/l (range 181-1586). Splenomegaly was detected in 96 patients, with an average spleen size (19.5 cm)reported. Fifty-one patients had Hgb < 10 g/dl. At the time of diagnosis, the pre-fibrotic stage was identified in the bone marrow examination of sixteen patients (17.8%). Splenomegaly was detected in 96 (91.4%) patients. Of the 66 patients who underwent genetic testing, 44 had a positive Jak2V617F mutation, 2 had a positive CALR mutation, and 1 had a positive MPL mutation.</p></div><div><h3>Conclusion</h3><p>Thus, this study has investigated the demographic, clinical, and laboratory characteristics of patients with primary myelofibrosis in Azerbaijan.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924000853/pdfft?md5=92532ac229269be46d80ad380e36012a&pid=1-s2.0-S2531137924000853-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C-type lectin receptors (CLRs) expressed by DC are considered attractive targets for effective targeting of antigen to antigen-presenting cells, since the participation of CLRs can additionally stimulate antigen presentation and, accordingly, subsequent activation of T cells. To study the ability of DC to enhance antigen capture and presentation using a library of fluorescein-labeled polyacrylamide glycoconjugates.
Methodology
DC was obtained by culturing human peripheral blood monocytes in a complete RPMI-1640 nutrient medium containing GM-CSF, IL-4 and TNFa. Immunophenotypes were analyzed using flow cytometric analysis. In our study, synthetic FSL (Function-Spacer-Lipid) constructs will be used: polyacrylamide glycoconjugate (Adi-sp)3-βDD-PAA-Fluo, conjugate N-acetyllactosamine, glycolipid (Adi-sp)3-βDD ((Adi-sp)3-βDD-DOPE). Next, the binding of these cells to glycoprobes was investigated.
Results
A new class of glycoconjugates specific for binding to C-type lectin receptors has been synthesized. The key cytokines for the cultivation of DC are GM-CSF (final concentration 80 ng/ml), IL-4 (final concentration 10 ng/ml), as well as differentiation inducers: TNF-α, PGE2. Mapping of human blood cells using a library of fluorescein-labeled polyacrylamide glycoconjugates showed that the studied glycoprobes bind to more than 15% of the human leukocyte population.
Conclusion
In our proposed research project, a new approach will be used to study the strategy of enhancing the capture and presentation of antigen by dendritic cells by targeting C-type lectin receptors.
目的 DC表达的C型凝集素受体（CLRs）被认为是将抗原有效靶向抗原呈递细胞的有吸引力的靶点，因为CLRs的参与可额外刺激抗原呈递，并相应地刺激T细胞的后续活化。为了研究 DC 利用荧光素标记的聚丙烯酰胺糖轭化合物库增强抗原捕获和递呈的能力，我们在含有 GM-CSF、IL-4 和 TNFa 的完全 RPMI-1640 营养培养基中培养人外周血单核细胞，从而获得方法学 DC。采用流式细胞分析法对免疫表型进行分析。在我们的研究中，将使用合成的 FSL（Function-Spacer-Lipid）构建物：聚丙烯酰胺糖共轭物（(Adi-sp)3-βDD-PAA-Fluo）、N-乙酰半乳糖胺共轭物、糖脂（(Adi-sp)3-βDD）（(Adi-sp)3-βDD-DOPE）。结果合成了一类能与 C 型凝集素受体特异性结合的新型糖结合物。培养 DC 的关键细胞因子是 GM-CSF（最终浓度为 80 ng/ml）、IL-4（最终浓度为 10 ng/ml）以及分化诱导剂：TNF-α、PGE2。使用荧光素标记的聚丙烯酰胺糖轭化合物库绘制的人类血细胞图显示，所研究的糖探针能与 15%以上的人类白细胞结合。
{"title":"A STRATEGY FOR DIRECT DELIVERY OF ANTIGENIC CONSTRUCTS TO DENDRITIC CELL RECEPTORS","authors":"Anzhelika Melnikova, Tatiana Mushkarina, Lyudmila Grivtsova","doi":"10.1016/j.htct.2024.04.011","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.011","url":null,"abstract":"<div><h3>Objective</h3><p>C-type lectin receptors (CLRs) expressed by DC are considered attractive targets for effective targeting of antigen to antigen-presenting cells, since the participation of CLRs can additionally stimulate antigen presentation and, accordingly, subsequent activation of T cells. To study the ability of DC to enhance antigen capture and presentation using a library of fluorescein-labeled polyacrylamide glycoconjugates.</p></div><div><h3>Methodology</h3><p>DC was obtained by culturing human peripheral blood monocytes in a complete RPMI-1640 nutrient medium containing GM-CSF, IL-4 and TNFa. Immunophenotypes were analyzed using flow cytometric analysis. In our study, synthetic FSL (Function-Spacer-Lipid) constructs will be used: polyacrylamide glycoconjugate (Adi-sp)3-βDD-PAA-Fluo, conjugate N-acetyllactosamine, glycolipid (Adi-sp)3-βDD ((Adi-sp)3-βDD-DOPE). Next, the binding of these cells to glycoprobes was investigated.</p></div><div><h3>Results</h3><p>A new class of glycoconjugates specific for binding to C-type lectin receptors has been synthesized. The key cytokines for the cultivation of DC are GM-CSF (final concentration 80 ng/ml), IL-4 (final concentration 10 ng/ml), as well as differentiation inducers: TNF-α, PGE2. Mapping of human blood cells using a library of fluorescein-labeled polyacrylamide glycoconjugates showed that the studied glycoprobes bind to more than 15% of the human leukocyte population.</p></div><div><h3>Conclusion</h3><p>In our proposed research project, a new approach will be used to study the strategy of enhancing the capture and presentation of antigen by dendritic cells by targeting C-type lectin receptors.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924000932/pdfft?md5=3280085fb110ea4bd524202cfc5dff64&pid=1-s2.0-S2531137924000932-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878978","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.044
Ali ÖZDEMİR , Funda ERKASAR
Objective
β-thalassemia major (β-TM) is an autosomal recessive disorder caused by mutations in the β-globin gene of hemoglobin. The disease is characterized by splenomegaly due to ineffective erythropoiesis, iron accumulation signs in tissues as a result of increased iron absorption, bone expansion due to increased erythropoietic activity, and decreased tissue oxygenation. One of the effected organ can be the lungs due to excessive iron deposition in these patients. The current study aimed to investigate the effect of serum ferritin level, which is known as a marker of iron accumulation in tissues, on pulmonary function tests (PFT) in patients with β-TM.
Methodology
Patients aged ≥6 years who were regularly followed in the pediatric hematology section of Mersin City Research and Training Hospital with a diagnosis of β-TM were included. All patients received regular blood transfusion and iron chelation therapy. Study participants underwent PFT prior to blood transfusion in the pediatric pulmonology section.
Results
A total of 43 patients with β-TM were studied. Included patients were divided into two groups according to the mean annual ferritin level; low ferritin group if below 2000 ml/ng and high ferritin group if above 2000 ml/ng. The low ferritin group was consisted of 19 patients and the high ferritin group was consisted of 24 patients. The characteristics of these two groups are shown in Table 1. There were no statistical significance in age, gender, body mass index, age at diagnosis, mean annual hemoglobin, splenectomy, cardiac involvement and oxygen saturation among both groups, but the number of annual transfusion was significantly higher in the high ferritin group than lower ferritin group.
When PFT parameters of both groups were compared, FVC (forced vital capacity) was statistically lower in the high ferritin group compared to the low ferritin group. Other parameters included FEV1 (forced expiratory volume in 1 second), FEV1/FVC ratio, PEF (peak expiratory flow) and FEF25-75 (forced expiratory flow between 25% and 75% of vital capacity) were similar among groups. (Table 2)
Conclusion
Patients with β-TM may accumulate iron in the interstitial area of the lungs which can lead fibrosis and impaired lung function over time. There are several studies investigated lung dysfunction and its etiology in these patients. Although the results of the studies are varied, the majority of them reported a restrictive pattern of respiratory dysfunction in thalassemia patients. Additionally, some studies showed the presence of mild or moderate obstruction in small airways and decrease in diffusion capacity with the increase of alveolar-capillary membrane thickness at advanced ages.In the present study, we found that patients with β-TM who had high ferritin level showed restrictive type lung function compared to those with low
{"title":"THE EFFECT OF FERRITIN LEVEL ON RESPIRATORY FUNCTIONS IN PATIENTS WITH Β-THALASSEMIA MAJOR","authors":"Ali ÖZDEMİR , Funda ERKASAR","doi":"10.1016/j.htct.2024.04.044","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.044","url":null,"abstract":"<div><h3>Objective</h3><p>β-thalassemia major (β-TM) is an autosomal recessive disorder caused by mutations in the β-globin gene of hemoglobin. The disease is characterized by splenomegaly due to ineffective erythropoiesis, iron accumulation signs in tissues as a result of increased iron absorption, bone expansion due to increased erythropoietic activity, and decreased tissue oxygenation. One of the effected organ can be the lungs due to excessive iron deposition in these patients. The current study aimed to investigate the effect of serum ferritin level, which is known as a marker of iron accumulation in tissues, on pulmonary function tests (PFT) in patients with β-TM.</p></div><div><h3>Methodology</h3><p>Patients aged ≥6 years who were regularly followed in the pediatric hematology section of Mersin City Research and Training Hospital with a diagnosis of β-TM were included. All patients received regular blood transfusion and iron chelation therapy. Study participants underwent PFT prior to blood transfusion in the pediatric pulmonology section.</p></div><div><h3>Results</h3><p>A total of 43 patients with β-TM were studied. Included patients were divided into two groups according to the mean annual ferritin level; low ferritin group if below 2000 ml/ng and high ferritin group if above 2000 ml/ng. The low ferritin group was consisted of 19 patients and the high ferritin group was consisted of 24 patients. The characteristics of these two groups are shown in Table 1. There were no statistical significance in age, gender, body mass index, age at diagnosis, mean annual hemoglobin, splenectomy, cardiac involvement and oxygen saturation among both groups, but the number of annual transfusion was significantly higher in the high ferritin group than lower ferritin group.</p><p>When PFT parameters of both groups were compared, FVC (forced vital capacity) was statistically lower in the high ferritin group compared to the low ferritin group. Other parameters included FEV<sub>1</sub> (forced expiratory volume in 1 second), FEV<sub>1</sub>/FVC ratio, PEF (peak expiratory flow) and FEF<sub>25-75</sub> (forced expiratory flow between 25% and 75% of vital capacity) were similar among groups. (Table 2)</p></div><div><h3>Conclusion</h3><p>Patients with β-TM may accumulate iron in the interstitial area of the lungs which can lead fibrosis and impaired lung function over time. There are several studies investigated lung dysfunction and its etiology in these patients. Although the results of the studies are varied, the majority of them reported a restrictive pattern of respiratory dysfunction in thalassemia patients. Additionally, some studies showed the presence of mild or moderate obstruction in small airways and decrease in diffusion capacity with the increase of alveolar-capillary membrane thickness at advanced ages.In the present study, we found that patients with β-TM who had high ferritin level showed restrictive type lung function compared to those with low ","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001263/pdfft?md5=a6ef8e7af121d170cbbc8f0152a6fb26&pid=1-s2.0-S2531137924001263-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878544","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
We aimed to investigate the indications for Carbohydrate antigen 19-9 (CA 19-9), carbohydrate antigen 125 (CA-125), carbohydrate antigen 15-3 (CA15-3) and carcinoembryogenic antigen (CEA) tumor biomarkers, less commonly used in children, and their association with patients diagnosed with childhood cancers.
Methodology
The study aimed to include patients aged 0-18 who had CA 19-9, CA-125, CA 15-3 and CEA tumor biomarker assessments at Adana City Training and Research Hospital (ACTRH) between 01.11.2022 and 01.11.2023. CA 19-9, CA-125, CA 15-3 and CEA values were recorded from routinely collected serum/blood samples of the patients. The relationship between tumor biomarkers and patients diagnosed with childhood cancers was evaluated.
Results
The study included 211 patients. Out of 211 patients, 145 (68.7%) were female, and 66 (31.3%) were male. Malignancy was detected in 35 patients (16.6%). There was no statistically significant relationship observed between CA 15-3, CA 19-9, and CEA positivity and the detection of malignancy. The respective p-values were found to be (p=0.711, p= 0.533, p=0.573). A statistically significant relationship was observed between CA-125 positivity and the detection of malignancy (p=0.002).
Conclusion
Tumor markers alone are not sufficient for making a definitive diagnosis or determining treatment decisions. However further comprehensive studies are needed for detection of association conventional tumor markers and childhood cancers.
{"title":"EVALUATION OF THE ASSOCIATION OF TUMOR BIOMARKERS WITH CHILDHOOD CANCERS","authors":"Şule ÇALIŞKAN KAMIŞ , Metin ÇİL , Begül YAĞCI KÜPELİ","doi":"10.1016/j.htct.2024.04.043","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.043","url":null,"abstract":"<div><h3>Objective</h3><p>We aimed to investigate the indications for Carbohydrate antigen 19-9 (CA 19-9), carbohydrate antigen 125 (CA-125), carbohydrate antigen 15-3 (CA15-3) and carcinoembryogenic antigen (CEA) tumor biomarkers, less commonly used in children, and their association with patients diagnosed with childhood cancers.</p></div><div><h3>Methodology</h3><p>The study aimed to include patients aged 0-18 who had CA 19-9, CA-125, CA 15-3 and CEA tumor biomarker assessments at Adana City Training and Research Hospital (ACTRH) between 01.11.2022 and 01.11.2023. CA 19-9, CA-125, CA 15-3 and CEA values were recorded from routinely collected serum/blood samples of the patients. The relationship between tumor biomarkers and patients diagnosed with childhood cancers was evaluated.</p></div><div><h3>Results</h3><p>The study included 211 patients. Out of 211 patients, 145 (68.7%) were female, and 66 (31.3%) were male. Malignancy was detected in 35 patients (16.6%). There was no statistically significant relationship observed between CA 15-3, CA 19-9, and CEA positivity and the detection of malignancy. The respective p-values were found to be (p=0.711, p= 0.533, p=0.573). A statistically significant relationship was observed between CA-125 positivity and the detection of malignancy (p=0.002).</p></div><div><h3>Conclusion</h3><p>Tumor markers alone are not sufficient for making a definitive diagnosis or determining treatment decisions. However further comprehensive studies are needed for detection of association conventional tumor markers and childhood cancers.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001251/pdfft?md5=78cb5f38388d222e37665dba01ff65ef&pid=1-s2.0-S2531137924001251-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878543","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.022
Berksoy Sahin, Birol Guvenc
Here we presented a 44 yr old male patient with an abdominal pain who had a distal gastric adenocarcinoma in his endoscopic biopsy. The pathology reported a chromogranine negative, CK20-positive, PD-L1 5% positive adenocarcinoma with MLH1 (-)and PMS-2(-) MSI status. PET/CT showed enlarged gastric wall (SUVmax 23.99) and enlarged perigastric lymphadenopathy (SUVmax 22.03) and no distant metastasis.
The patient received 4 courses of Nivolumab plus FLOT-4 chemoimmunothrapy in neoadjuvant setting. He experienced Grade 2 myelotoxicity and 2 packages of red blood were transfused. Following 4 courses of chemoimmunotherapy a total gasterectomy was performed and the pathology reported no evidence of tumor in the stomach and also perigastric lymph-nodes revealing a pathological complete response.
There has been no standart treatment for MSI-high gastric cancer, yet. Very few phase I-II studies wth limited number of patients suggest an immunotherapy-based treatment.
Here we report a combination regimen of original FLOT-4 chemotherapy with an PD-L1 Ab (nivolumab) that resulted a pCR in the neoadjuvant setting. Four courses of the same chemotherapy was planned in the adjuvant setting.
{"title":"Neoadjuvant chemoimmunotherapy for a patient with micro-stallete instabile gastric cancer resulted a pathological complete response","authors":"Berksoy Sahin, Birol Guvenc","doi":"10.1016/j.htct.2024.04.022","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.022","url":null,"abstract":"<div><p>Here we presented a 44 yr old male patient with an abdominal pain who had a distal gastric adenocarcinoma in his endoscopic biopsy. The pathology reported a chromogranine negative, CK20-positive, PD-L1 5% positive adenocarcinoma with MLH1 (-)and PMS-2(-) MSI status. PET/CT showed enlarged gastric wall (SUVmax 23.99) and enlarged perigastric lymphadenopathy (SUVmax 22.03) and no distant metastasis.</p><p>The patient received 4 courses of Nivolumab plus FLOT-4 chemoimmunothrapy in neoadjuvant setting. He experienced Grade 2 myelotoxicity and 2 packages of red blood were transfused. Following 4 courses of chemoimmunotherapy a total gasterectomy was performed and the pathology reported no evidence of tumor in the stomach and also perigastric lymph-nodes revealing a pathological complete response.</p><p>There has been no standart treatment for MSI-high gastric cancer, yet. Very few phase I-II studies wth limited number of patients suggest an immunotherapy-based treatment.</p><p>Here we report a combination regimen of original FLOT-4 chemotherapy with an PD-L1 Ab (nivolumab) that resulted a pCR in the neoadjuvant setting. Four courses of the same chemotherapy was planned in the adjuvant setting.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001044/pdfft?md5=801f17c9342bc60060a9afea781a88cf&pid=1-s2.0-S2531137924001044-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878986","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.047
Meryem SENER , Hilal Nur YALDIZ , Candas MUMCU , Bengisu Ece DUMAN , Berra Nur ISCI , Emre BAL , Irem KABALCI KADIOGLU , Birol GUVENC
This case report details the diagnosis, treatment, and management of a rare case of severe malaria in a 57-year-old male with a significant travel history, having returned from Sudan where he worked as a textile master for three years. Despite initial improvement after standard malaria treatment 1.5 years prior in Sudan, the patient presented with high fever, chills, shivering, weakness, and loss of appetite in October 2023. Laboratory findings indicated an infection, and an abdominal ultrasound revealed hepatic steatosis and splenomegaly. A peripheral smear confirmed the presence of Plasmodium vivax. Given the severity of the patient's condition, characterized by hypotension and the risk of complications due to his background of diabetes, hypertension, and cardiovascular disease, he was treated with a combination of standard antimalarial therapy (artemether, lumefantrine, and primaquine) and erythrocyte exchange apheresis. This multidisciplinary approach led to significant improvement in his health. This case underscores the importance of considering travel history in the differential diagnosis and highlights the efficacy of combining erythrocyte exchange apheresis with standard antimalarial therapy in managing severe cases of malaria, which is particularly rare in non-endemic regions.
{"title":"From Diagnosis to Recovery: Addressing Rare Malaria with Travel History Using Standard and Apheresis Therapies","authors":"Meryem SENER , Hilal Nur YALDIZ , Candas MUMCU , Bengisu Ece DUMAN , Berra Nur ISCI , Emre BAL , Irem KABALCI KADIOGLU , Birol GUVENC","doi":"10.1016/j.htct.2024.04.047","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.047","url":null,"abstract":"<div><p>This case report details the diagnosis, treatment, and management of a rare case of severe malaria in a 57-year-old male with a significant travel history, having returned from Sudan where he worked as a textile master for three years. Despite initial improvement after standard malaria treatment 1.5 years prior in Sudan, the patient presented with high fever, chills, shivering, weakness, and loss of appetite in October 2023. Laboratory findings indicated an infection, and an abdominal ultrasound revealed hepatic steatosis and splenomegaly. A peripheral smear confirmed the presence of Plasmodium vivax. Given the severity of the patient's condition, characterized by hypotension and the risk of complications due to his background of diabetes, hypertension, and cardiovascular disease, he was treated with a combination of standard antimalarial therapy (artemether, lumefantrine, and primaquine) and erythrocyte exchange apheresis. This multidisciplinary approach led to significant improvement in his health. This case underscores the importance of considering travel history in the differential diagnosis and highlights the efficacy of combining erythrocyte exchange apheresis with standard antimalarial therapy in managing severe cases of malaria, which is particularly rare in non-endemic regions.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001299/pdfft?md5=51c0cc080e61ac6970fab3feaf20789e&pid=1-s2.0-S2531137924001299-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140879030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The use of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET-СT) to determine the initial stage and assess the response to treatment for aggressive lymphomas is considered standard. Evaluation of bone marrow infiltration in PET-CT with 18F-FDG usually makes it possible to distinguish normal regenerating bone marrow after chemotherapy by the characteristic nature of absorption.
Case report
A 54-year-old patient diagnosed with diffuse large B-cell lymphoma (DLBCL) with lesions of the lymph nodes and bone marrow of the focal form with osteodestruction of the lytic type. Therapy at the A.F.Tsyba MRRC – 6 cycles of R-CHOP, completed in December 2022.
Results
The PET-CT - 2 cycles is completely normalized. The February 2023, PET-CT showed an increase in the level of metabolism in one of the foci of osteodestruction in the pelvic bones. The biopsy, March 2023, absence of signs of DLBCL. PET-CT, June 2023, the increase of contrast accumulation in previously identified foci. Trepan biopsy in July 2023 – a picture of hematopoiesis foci in the bone marrow, a statement of remission. PET-CT scan in December 2023 confirming the remission of the disease.
Conclusion
False-positive PET-CT results in the era of rituximab began to be detected with greater frequency, therefore, their assessment and correct interpretation, as well as additional clarification using other available techniques, are necessary in modern clinical practice to choose tactics for further therapy.
{"title":"A FALSE POSITIVE PET-CT RESULT AFTER TREATMENT OF A PATIENT WITH DIFFUSE B-CELL LARGE CELL LYMPHOMA. A CLINICAL CASE.","authors":"Artem Vovchenko , Anastasia Galitsyna , Andrey Danilenko , Natalya Falaleeva , Alena Terekhova , Daniil Manaenkov , Sergei Ivanov , Andrey Kaprin","doi":"10.1016/j.htct.2024.04.027","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.027","url":null,"abstract":"<div><h3>Objective</h3><p>The use of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET-СT) to determine the initial stage and assess the response to treatment for aggressive lymphomas is considered standard. Evaluation of bone marrow infiltration in PET-CT with 18F-FDG usually makes it possible to distinguish normal regenerating bone marrow after chemotherapy by the characteristic nature of absorption.</p></div><div><h3>Case report</h3><p>A 54-year-old patient diagnosed with diffuse large B-cell lymphoma (DLBCL) with lesions of the lymph nodes and bone marrow of the focal form with osteodestruction of the lytic type. Therapy at the A.F.Tsyba MRRC – 6 cycles of R-CHOP, completed in December 2022.</p></div><div><h3>Results</h3><p>The PET-CT - 2 cycles is completely normalized. The February 2023, PET-CT showed an increase in the level of metabolism in one of the foci of osteodestruction in the pelvic bones. The biopsy, March 2023, absence of signs of DLBCL. PET-CT, June 2023, the increase of contrast accumulation in previously identified foci. Trepan biopsy in July 2023 – a picture of hematopoiesis foci in the bone marrow, a statement of remission. PET-CT scan in December 2023 confirming the remission of the disease.</p></div><div><h3>Conclusion</h3><p>False-positive PET-CT results in the era of rituximab began to be detected with greater frequency, therefore, their assessment and correct interpretation, as well as additional clarification using other available techniques, are necessary in modern clinical practice to choose tactics for further therapy.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001093/pdfft?md5=091ba0047dbcb6051005775b9ed5b727&pid=1-s2.0-S2531137924001093-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878836","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
This case report delves into the intricacies of managing a patient diagnosed with both essential thrombocythemia and Addison's disease, illustrating the challenges and importance of an integrated approach to complex, coexisting conditions. A 47-year-old woman presented with enduring symptoms of fatigue, skin darkening, and appetite loss, which progressively led to substantial weight loss. Initially treated for essential thrombocythemia, a common yet serious myeloproliferative disorder, her condition did not fully improve with standard therapy, including hydroxyurea. Further evaluation was prompted by her deteriorating clinical status, characterized by severe hypotension and exacerbated systemic symptoms, leading to the diagnosis of primary adrenal insufficiency or Addison's disease. The confirmation of Addison's disease, alongside essential thrombocythemia, necessitated a tailored therapeutic strategy that addressed both endocrine and hematological aspects. With the initiation of appropriate therapy targeting Addison's disease, alongside ongoing management of essential thrombocythemia, the patient experienced a significant alleviation of symptoms and stabilization of her condition. This case underscores the necessity for vigilance and comprehensive evaluation in patients with non-specific systemic symptoms, highlighting the potential for concurrent, serious medical diagnoses.
{"title":"Essential Thrombocythemia Complicated by Addison's Disease: A Case of Overlapping Endocrine and Hematological Disorders","authors":"Meryem SENER , Kaan NISANOGLU , Candas MUMCU , Bengisu Ece DUMAN , Berra Nur ISCI , Emre BAL , Irem KABALCI KADIOGLU , Birol GUVENC","doi":"10.1016/j.htct.2024.04.018","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.018","url":null,"abstract":"<div><p>This case report delves into the intricacies of managing a patient diagnosed with both essential thrombocythemia and Addison's disease, illustrating the challenges and importance of an integrated approach to complex, coexisting conditions. A 47-year-old woman presented with enduring symptoms of fatigue, skin darkening, and appetite loss, which progressively led to substantial weight loss. Initially treated for essential thrombocythemia, a common yet serious myeloproliferative disorder, her condition did not fully improve with standard therapy, including hydroxyurea. Further evaluation was prompted by her deteriorating clinical status, characterized by severe hypotension and exacerbated systemic symptoms, leading to the diagnosis of primary adrenal insufficiency or Addison's disease. The confirmation of Addison's disease, alongside essential thrombocythemia, necessitated a tailored therapeutic strategy that addressed both endocrine and hematological aspects. With the initiation of appropriate therapy targeting Addison's disease, alongside ongoing management of essential thrombocythemia, the patient experienced a significant alleviation of symptoms and stabilization of her condition. This case underscores the necessity for vigilance and comprehensive evaluation in patients with non-specific systemic symptoms, highlighting the potential for concurrent, serious medical diagnoses.</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001007/pdfft?md5=a0bc760a8477717ab9ba28149b7be0b6&pid=1-s2.0-S2531137924001007-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-01DOI: 10.1016/j.htct.2024.04.034
Bahar ÖZMÜŞ , Bilal ÖZMÜŞ
Objective
CLINICAL DIAGNOSIS, APPROACH AND MANAGEMENT OF PLASMA CELL DISEASES OF ATYPICAL AGE AND ATYPICAL LOCATION
Case report: OUR FIRST CASE: A 66-YEAR-OLD FEMALE PATIENT APPLIED WITH ABDOMINAL PAIN. HGB: 6,6 AND ENDOSCOPY IS DONE. 8 CMDIFFUSE THICKENING WAS DETECTED IN THE STOMACH. A BIOPSY IS TAKEN. THE RESULT IS STOMACH PLASMOCYTOMA. KT STARTED.
SECOND CASE
A 32-YEAR-OLD FEMALE PATIENT ADMITS WITH WEIGHT LOSS, DYSPNEA AND LEUKOCYTOSIS. IT IS PLASMA CELL LEUKEMIA. THE KIT IS BEING MADE.LATEST CASE: A PATIENT WHO PRESENT WITH DIPLEGIA IN THE MEDULLA SPINALIST HAS A PLASMOCYTOMA IN THE MEDULLA SPINALIST. HE TREATMENT
{"title":"A COMPILATION OF ATYPICAL PLASMA CELL DISCRASIA CASES","authors":"Bahar ÖZMÜŞ , Bilal ÖZMÜŞ","doi":"10.1016/j.htct.2024.04.034","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.034","url":null,"abstract":"<div><h3>Objective</h3><p>CLINICAL DIAGNOSIS, APPROACH AND MANAGEMENT OF PLASMA CELL DISEASES OF ATYPICAL AGE AND ATYPICAL LOCATION</p><p>Case report: OUR FIRST CASE: A 66-YEAR-OLD FEMALE PATIENT APPLIED WITH ABDOMINAL PAIN. HGB: 6,6 AND ENDOSCOPY IS DONE. 8 CMDIFFUSE THICKENING WAS DETECTED IN THE STOMACH. A BIOPSY IS TAKEN. THE RESULT IS STOMACH PLASMOCYTOMA. KT STARTED.</p></div><div><h3>SECOND CASE</h3><p>A 32-YEAR-OLD FEMALE PATIENT ADMITS WITH WEIGHT LOSS, DYSPNEA AND LEUKOCYTOSIS. IT IS PLASMA CELL LEUKEMIA. THE KIT IS BEING MADE.LATEST CASE: A PATIENT WHO PRESENT WITH DIPLEGIA IN THE MEDULLA SPINALIST HAS A PLASMOCYTOMA IN THE MEDULLA SPINALIST. HE TREATMENT</p><p>Methodology</p></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":null,"pages":null},"PeriodicalIF":2.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2531137924001160/pdfft?md5=04b9d01a2971d06c9e10950ed40d0ecd&pid=1-s2.0-S2531137924001160-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140878988","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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