Pub Date : 2020-10-12DOI: 10.26420/annhematoloncol.2020.1314
M. Ma’koseh, S. Sa’deh, K. Halahleh, H. Abu-Jazar, L. Dahabreh, E. KhattabE, M. AbuShanap, A. AlShyoukh, R. Al-Far, H. Hashem, R. Najjar, W. Da’na, A. Tbakhi
In Multiple Myeloma (MM), response to High-Dose Chemotherapy (HDC) and Autologous Stem Cell Transplant (ASCT) has important prognostic and therapeutic implications. Best timing for response evaluation after ASCT is not well studied. Our study evaluated the correlation between response on day 30 and day 100 after ASCT with Progression Free Survival (PFS) and Overall Survival (OS) in 119 MM patients. Median follow-up was 39.8 months. Complete Response (CR) was achieved in 53.8% and 55.5% of patients on D 30 and D 100, respectively. On D30, there was no significant difference in PFS or OS in CR vs. no CR group (35.4 vs. 22.1 months, p: 0.058) and (92.6 months vs. not reached p: 0.96) respectively nor in responders (R) vs. Non-Responders (NR) group (97.8 vs. 47.1 months p: 0.08) and (30.2 vs. 18.9 months, p: 0.09) respectively. While on D100, PFS was significantly better in CR vs. no CR group (33.8 vs. 18.1 months, p: 0.0047) as well as in R vs. NR (30.6 vs. 16.9 months p: 0.015). However, OS was not better in either (92.6 vs. 52.1 months p: 0.46) and (92.6 months vs. not reached p: 0, 88) respectively. In conclusion, after HDC and ASCT for MM, we recommend doing response evaluation on D100 rather than D30 as it better correlates with PFS. Further studies are required to confirm this finding in the era of consolidation and maintenance treatment.
{"title":"Comparison of Two Post-Transplant Predictive Indexes: Day 100 is a Better Time-Point for Response Evaluation after Autologous Stem Cell Transplantation in Multiple Myeloma: Ass Retrospective Study","authors":"M. Ma’koseh, S. Sa’deh, K. Halahleh, H. Abu-Jazar, L. Dahabreh, E. KhattabE, M. AbuShanap, A. AlShyoukh, R. Al-Far, H. Hashem, R. Najjar, W. Da’na, A. Tbakhi","doi":"10.26420/annhematoloncol.2020.1314","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2020.1314","url":null,"abstract":"In Multiple Myeloma (MM), response to High-Dose Chemotherapy (HDC) and Autologous Stem Cell Transplant (ASCT) has important prognostic and therapeutic implications. Best timing for response evaluation after ASCT is not well studied. Our study evaluated the correlation between response on day 30 and day 100 after ASCT with Progression Free Survival (PFS) and Overall Survival (OS) in 119 MM patients. Median follow-up was 39.8 months. Complete Response (CR) was achieved in 53.8% and 55.5% of patients on D 30 and D 100, respectively. On D30, there was no significant difference in PFS or OS in CR vs. no CR group (35.4 vs. 22.1 months, p: 0.058) and (92.6 months vs. not reached p: 0.96) respectively nor in responders (R) vs. Non-Responders (NR) group (97.8 vs. 47.1 months p: 0.08) and (30.2 vs. 18.9 months, p: 0.09) respectively. While on D100, PFS was significantly better in CR vs. no CR group (33.8 vs. 18.1 months, p: 0.0047) as well as in R vs. NR (30.6 vs. 16.9 months p: 0.015). However, OS was not better in either (92.6 vs. 52.1 months p: 0.46) and (92.6 months vs. not reached p: 0, 88) respectively. In conclusion, after HDC and ASCT for MM, we recommend doing response evaluation on D100 rather than D30 as it better correlates with PFS. Further studies are required to confirm this finding in the era of consolidation and maintenance treatment.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41314488","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 : 2019-12-06DOI: 10.26420/annhematoloncol.2019.1276
P. Lemež, H. Dignum, M. Ganczakowski, L. Chiecchio, R. Ayto, K. Baker, S. Singh, T. Cranfield, G. Matthias, C. James, R. Corser
The prognosis of older patients with de novo Acute Myeloid Leukemia (AML) is usually dismal. Palliative therapy with LDAC is one of the treatment options with a median survival of less than one year. Several reported older cases with AML with a survival of 25-51 months on therapy with LDAC lack details of the AML type, clinical characteristics, and treatment. This case report describes a 79-year old man with AML M2, normal karyotype, leukocytosis 33.7 x 10 9 /L, and involving only Granulocyte-Macrophage Line (GM-AML) who survived 84 months on 53 repeated cycles of LDAC, the longest described survival on LDAC. His leukemic cells exhibited Nucleophosmin 1 ( NPM1 ) mutation and Fms-Like Tyrosine-kinase 3 gene ( FLT3 ) Internal Tandem Duplication (ITD) with a high FLT3 -ITD to FLT3 WT allelic ratio, typical immunophenotype, morphology and no dysplastic features. We propose that older patients with de novo GM-AML with these characteristics may benefit from prolonged LDAC therapy. Changes; EMD: Erythroblastic and/or Megakaryocytic Dysplasia; BM: Bone Marrow; FBC: Full Blood Cell count; FLT3- ITD: Fms-like Tyrosine kinase-3 gene ( FLT3 ) Internal Tandem Duplication (ITD); FLT3 WT: FLT3 Wild Type; GM-AML: AML involving only cells of Granulocytic-Macrophage line; CR: Complete Remission; HC: Hydroxycarbamide; LDAC: Low-Dose Cytarabine; NK: normal karyotype; NPM1 : Nucleophosmin 1 gene; PML/RARA : Fusion gene of Promyelocytic Leukemia gene/Retinoic Acid Receptor-Alpha gene; PS: Performance Status; RBC: Red Blood Cells; SICT: Standard-Dose Induction Chemotherapy; WBC: White Blood Cells
老年新发急性髓细胞白血病（AML）患者的预后通常是令人沮丧的。LDAC的姑息治疗是中位生存期不到一年的治疗选择之一。一些报告的老年AML患者在接受LDAC治疗后存活25-51个月，缺乏AML类型、临床特征和治疗的详细信息。本病例报告描述了一名79岁男性，患有AML M2，核型正常，白细胞增多症33.7 x 10 9/L，仅涉及粒细胞巨噬细胞系（GM-AML），他在53个重复周期的LDAC中存活了84个月，是LDAC中所描述的存活时间最长的。他的白血病细胞表现出核磷酸蛋白1（NPM1）突变和Fms样酪氨酸激酶3基因（FLT3）内部串联重复（ITD），具有高的FLT3-ITD与FLT3 WT等位基因比例、典型的免疫表型、形态和无发育异常特征。我们提出，具有这些特征的新发GM-AML老年患者可能受益于长期LDAC治疗。变更；EMD：红细胞和/或巨核细胞发育不良；BM：骨髓；FBC：全血细胞计数；FLT3-ITD：Fms样酪氨酸激酶-3基因（FLT3）内部串联复制（ITD）；FLT3 WT：FLT3野生型；GM-AML:AML仅涉及粒细胞巨噬细胞系的细胞；CR：完全缓解；HC：羟基脲；LDAC：低剂量阿糖胞苷；NK：正常核型；NPM1：核磷酸蛋白1基因；PML/RARA：早幼粒细胞白血病基因/维甲酸受体α基因的融合基因；PS：性能状态；RBC：红细胞；SICT：标准剂量诱导化疗；WBC：白细胞
{"title":"Prolonged Survival of a 79-Year Old Man with Acute Myeloid Leukemia M2, Normal Karyotype, NPM1 and FLT3-ITD Mutations, WBC 33.7 × 109/L, and Involving only Granulocyte-Macrophage Line on 53 Cycles of Low-Dose Cytarabine","authors":"P. Lemež, H. Dignum, M. Ganczakowski, L. Chiecchio, R. Ayto, K. Baker, S. Singh, T. Cranfield, G. Matthias, C. James, R. Corser","doi":"10.26420/annhematoloncol.2019.1276","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2019.1276","url":null,"abstract":"The prognosis of older patients with de novo Acute Myeloid Leukemia (AML) is usually dismal. Palliative therapy with LDAC is one of the treatment options with a median survival of less than one year. Several reported older cases with AML with a survival of 25-51 months on therapy with LDAC lack details of the AML type, clinical characteristics, and treatment. This case report describes a 79-year old man with AML M2, normal karyotype, leukocytosis 33.7 x 10 9 /L, and involving only Granulocyte-Macrophage Line (GM-AML) who survived 84 months on 53 repeated cycles of LDAC, the longest described survival on LDAC. His leukemic cells exhibited Nucleophosmin 1 ( NPM1 ) mutation and Fms-Like Tyrosine-kinase 3 gene ( FLT3 ) Internal Tandem Duplication (ITD) with a high FLT3 -ITD to FLT3 WT allelic ratio, typical immunophenotype, morphology and no dysplastic features. We propose that older patients with de novo GM-AML with these characteristics may benefit from prolonged LDAC therapy. Changes; EMD: Erythroblastic and/or Megakaryocytic Dysplasia; BM: Bone Marrow; FBC: Full Blood Cell count; FLT3- ITD: Fms-like Tyrosine kinase-3 gene ( FLT3 ) Internal Tandem Duplication (ITD); FLT3 WT: FLT3 Wild Type; GM-AML: AML involving only cells of Granulocytic-Macrophage line; CR: Complete Remission; HC: Hydroxycarbamide; LDAC: Low-Dose Cytarabine; NK: normal karyotype; NPM1 : Nucleophosmin 1 gene; PML/RARA : Fusion gene of Promyelocytic Leukemia gene/Retinoic Acid Receptor-Alpha gene; PS: Performance Status; RBC: Red Blood Cells; SICT: Standard-Dose Induction Chemotherapy; WBC: White Blood Cells","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45518842","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 : 2019-11-26DOI: 10.26420/annhematoloncol.2019.1273
M. Napolitano, M. Sardo, L. LoCoco, S. Raso, M. Mansueto, S. Mancuso, S. Siragusa
Acquired Hemophilia (AH) poses several challenges to clinicians due to potential delays in diagnosis, based on a high index of suspicion, and a high risk of limb and life-threatening bleeding. We here report a case of AH with extremely high inhibitor titer (up to 1200 BU) in a patient who also developed venous thrombosis requiring anticoagulant treatment after prolonged immobilization for femur fracture. Multiple lines of immunosuppressive treatment were needed to achieve inhibitor eradication, probably due to the extremely high inhibitor titer, bleeding management also required several lines of treatment with bypassing agents. Bleeding treatment was here monitored by global hemostatic assays. Management of AH in a reference center allowed to achieve complete remission even in a very intricate condition. was transferred to our unit for transfusion-dependent anemia and persistently prolonged Activated Partial Thromboplastin Time (APTT). The patient was first admitted to a psychiatric unit for a reduction in his mood and attempted suicide; a fracture of the left femur secondary to an accidental fall at home was diagnosed. In consideration of the time since the fall, the patient was managed with conservative therapy with complete immobilization and prophylaxis of venous thromboembolism with Low Molecular Weight Heparin (LMWH). During rehabilitative therapy, painful chest wall and right lower limb muscle hematomas occurred associated with progressive anemia requiring transfusion. The patient was then transferred to an internal medical unit after a CT scan detected active bleeding in the thoracic muscle associated with prolonged APTT for which LMWH was discontinued and fresh frozen plasma administered. He was admitted to our unit 12 days after the first detection of prolonged APTT. The patient presented poor general clinical conditions with a high thrombotic risk due to immobilization for the fracture. A clinical exam revealed a large hematoma of the lower and upper limbs and back. His blood count showed severe anemia. Isolated prolonged APTT (106 seconds) was confirmed. AH was suspected, clotting FVIII activity
{"title":"Acquired Hemophilia A Associated with Venous Thrombosis and Very High Inhibitor Titer: A Challenging Scenario","authors":"M. Napolitano, M. Sardo, L. LoCoco, S. Raso, M. Mansueto, S. Mancuso, S. Siragusa","doi":"10.26420/annhematoloncol.2019.1273","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2019.1273","url":null,"abstract":"Acquired Hemophilia (AH) poses several challenges to clinicians due to potential delays in diagnosis, based on a high index of suspicion, and a high risk of limb and life-threatening bleeding. We here report a case of AH with extremely high inhibitor titer (up to 1200 BU) in a patient who also developed venous thrombosis requiring anticoagulant treatment after prolonged immobilization for femur fracture. Multiple lines of immunosuppressive treatment were needed to achieve inhibitor eradication, probably due to the extremely high inhibitor titer, bleeding management also required several lines of treatment with bypassing agents. Bleeding treatment was here monitored by global hemostatic assays. Management of AH in a reference center allowed to achieve complete remission even in a very intricate condition. was transferred to our unit for transfusion-dependent anemia and persistently prolonged Activated Partial Thromboplastin Time (APTT). The patient was first admitted to a psychiatric unit for a reduction in his mood and attempted suicide; a fracture of the left femur secondary to an accidental fall at home was diagnosed. In consideration of the time since the fall, the patient was managed with conservative therapy with complete immobilization and prophylaxis of venous thromboembolism with Low Molecular Weight Heparin (LMWH). During rehabilitative therapy, painful chest wall and right lower limb muscle hematomas occurred associated with progressive anemia requiring transfusion. The patient was then transferred to an internal medical unit after a CT scan detected active bleeding in the thoracic muscle associated with prolonged APTT for which LMWH was discontinued and fresh frozen plasma administered. He was admitted to our unit 12 days after the first detection of prolonged APTT. The patient presented poor general clinical conditions with a high thrombotic risk due to immobilization for the fracture. A clinical exam revealed a large hematoma of the lower and upper limbs and back. His blood count showed severe anemia. Isolated prolonged APTT (106 seconds) was confirmed. AH was suspected, clotting FVIII activity","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-11-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43981351","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 : 2019-11-19DOI: 10.26420/annhematoloncol.2019.1270
Lage Lapc
Histiocytic Sarcoma (HS) is an uncommon malignancy derived from macrophage/dendritic lineage cells and accounts for less than 1% of hematologic tumors. Due to its rarity, little is known about its clinical-biological behavior and therapeutic recommendations have not been standardized so far. In addition to lymph node enlargement most cases present extranodal involvement, especially in the gastrointestinal tract, skin and soft tissues. Bone involvement is usually described in association with systemic disease and in advanced stages. Here we describe a case of patient with HS with primary bone involvement and multiple osteolytic lesions and bone fractures in the absence of another organs involvement. A male patient presented with an aggressive disease with rapid progression and his disease was unresponsive to standard therapy. Although rare, this kind of HS presentation should fall within the scope of differential diagnosis of other neoplasms with widespread bone involvement such as multiple myeloma and bone metastasis from solid tumors as the prostate, breast and lungs. Hemoglobin Concentration; RDW: Red Cell Distribution Width; WBC: White Blood Count; TIBC: Total Iron-Binding Capacity; ESR: Erythroid Sedimentation Rate; CRP: C-Reactive Protein; Ca: Calcium; HIV: Human Immunodeficiency Virus; MRI: Magnetic Resonance Imaging; CT: Computerized Tomography; H&E: Haematoxyline & Eosin staining; TdT: Terminal Deoxynucleotidyl Transferase; 18-FDG-PETCT: 18-Fluorodeoxyglucose-Computerized Tomography With Positron Emission; Max: Maximum; SUV: Standardized UpTake Value; CHOEP: Cyclophosphamide, doxorubicin, Oncovin, Etoposide and Prednisone; G-CSF: Granulocyte Colony Stimulating Factor; OS: Overall Survival; ICE: Ifosfamide, Carboplatin and Etoposide
{"title":"Primary Histiocytic Sarcoma of Bone with Disseminated Involvement - Initial Presentation Mimicking Multiple Myeloma: The Atypical Face of a Rare Neoplasm","authors":"Lage Lapc","doi":"10.26420/annhematoloncol.2019.1270","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2019.1270","url":null,"abstract":"Histiocytic Sarcoma (HS) is an uncommon malignancy derived from macrophage/dendritic lineage cells and accounts for less than 1% of hematologic tumors. Due to its rarity, little is known about its clinical-biological behavior and therapeutic recommendations have not been standardized so far. In addition to lymph node enlargement most cases present extranodal involvement, especially in the gastrointestinal tract, skin and soft tissues. Bone involvement is usually described in association with systemic disease and in advanced stages. Here we describe a case of patient with HS with primary bone involvement and multiple osteolytic lesions and bone fractures in the absence of another organs involvement. A male patient presented with an aggressive disease with rapid progression and his disease was unresponsive to standard therapy. Although rare, this kind of HS presentation should fall within the scope of differential diagnosis of other neoplasms with widespread bone involvement such as multiple myeloma and bone metastasis from solid tumors as the prostate, breast and lungs. Hemoglobin Concentration; RDW: Red Cell Distribution Width; WBC: White Blood Count; TIBC: Total Iron-Binding Capacity; ESR: Erythroid Sedimentation Rate; CRP: C-Reactive Protein; Ca: Calcium; HIV: Human Immunodeficiency Virus; MRI: Magnetic Resonance Imaging; CT: Computerized Tomography; H&E: Haematoxyline & Eosin staining; TdT: Terminal Deoxynucleotidyl Transferase; 18-FDG-PETCT: 18-Fluorodeoxyglucose-Computerized Tomography With Positron Emission; Max: Maximum; SUV: Standardized UpTake Value; CHOEP: Cyclophosphamide, doxorubicin, Oncovin, Etoposide and Prednisone; G-CSF: Granulocyte Colony Stimulating Factor; OS: Overall Survival; ICE: Ifosfamide, Carboplatin and Etoposide","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48332167","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 : 2019-03-08DOI: 10.26420/ANNHEMATOLONCOL.2019.1242
A. Araújo
{"title":"Pulmonary Sclerosing Pneumocytoma and Adenocarcinoma Presenting as Two Distinct Contralateral Neoplasms in the Same Patient","authors":"A. Araújo","doi":"10.26420/ANNHEMATOLONCOL.2019.1242","DOIUrl":"https://doi.org/10.26420/ANNHEMATOLONCOL.2019.1242","url":null,"abstract":"","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-03-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48380969","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 : 2019-03-04DOI: 10.26420/ANNHEMATOLONCOL.2019.1240
A. Płotka
Oncological patients are at an increased risk of an opportunistic infection caused by Listeria monocytogenes . Listeriosis remains infrequent in oncological patients. This article describes a case report of a 15-year old boy diagnosed with T-ALL, who developed L.monocytogenes infection during an intensive cancer treatment. The patient’s treatment proceeded with numerous complications. Impaired coagulation led to genetic examination and factor V Leiden and heterozygotic C667T and A1298C mutations of MTHFR detection. Corticosteroid therapy resulted in insulin resistance and hyperbilirubinemia with normal hepatic enzymes level. Gilbert’s syndrome was confirmed. CT evaluating remission revealed asymptomatic invasive pulmonary aspergillosis. After finishing Protocol II the rise in CRP was observed with decreased number of WBC. Patient was feverish and complained of nonspecific abdominal pain and dysuria. USG detected fluid in right iliac fossa. A few hours later boy’s neurological state dramatically worsened. CT revealed acute hydrocephalus, which needed immediate drainage. Listeria monocytogenes was isolated from CSF. Despite intensive antibiotic therapy, infection caused boy’s death. On clinical examination massive submandibular and cervical lymphadenopathy, petechiae all over the body, especially severe in pelvic girdle, splenomegaly (spleen reaching lower quadrant) and hepatomegaly (3cm above subcostal arch), tachycardia (150/min), on auscultation diminished vesicular murmur paraspinal on both sides were noticed. Blood tests revealed leukocytosis (104 ths./µl), III grade thrombocytopenia (22ths/µl) and hyperuricemia (27mg/dl), high level of creatinine (1,18mg/dl) and urea (75mg/dl). Boy presented with, both laboratorary and clinically, renal insufficiency in 4 th stage (GFR-22ml/min). Intravenous rehydration, rasburicase and diuretics were administered resulting in gradual decrease and normalisation of uric acid. RTG and CT examinations revealed widened mediastinum (implying tumour), pleural and pericardial effusion. Due to clinical presentation and laboratory findings, the initial diagnosis of T-cell acute lymphoblastic leukemia was made and confirmed by myelogram and immunological examination of bone marrow. Treatment according to ALL IC BFM 2009 for intermediate-risk ALL was immediately administered. During induction phase clinical and biochemical signs of renal insufficiency occurred. Patient required hemodialysis with calcium supplementation (Ca-0,81 mmol/L) and hyperphosphatemia management at the same time (P-12,42mmol/L). Boy developed symptoms of tetany, which disappeared after normalisation of calcium
肿瘤患者有增加的由单核细胞增生李斯特菌引起的机会性感染的风险。李斯特菌病在肿瘤患者中仍不常见。这篇文章描述了一个15岁的男孩诊断为T-ALL的病例报告，他在强化癌症治疗期间发展为单核细胞增生杆菌感染。病人的治疗过程中出现了许多并发症。凝血功能受损导致基因检查和因子V Leiden和杂合子C667T和A1298C突变检测MTHFR。皮质类固醇治疗导致胰岛素抵抗和肝酶水平正常的高胆红素血症。吉尔伯特综合症被确诊。CT评估缓解显示无症状侵袭性肺曲霉病。完成方案II后，CRP升高，白细胞减少。患者发热，主诉非特异性腹痛和排尿困难。USG检测到右侧髂窝有液体。几个小时后，男孩的神经状态急剧恶化。CT显示急性脑积水，需要立即引流。从脑脊液中分离到单核细胞增生李斯特菌。尽管进行了密集的抗生素治疗，感染还是导致了男孩的死亡。临床检查见大量下颌骨及颈部淋巴结肿大，全身瘀点，尤以骨盆带严重，脾肿大(脾及下腹)及肝肿大(肋下弓以上3cm)，心动过速(150/min)，听诊见双侧椎管旁水疱性杂音减弱。血液检查显示白细胞增多(104毫克/微升)，III级血小板减少(22毫克/微升)和高尿酸血症(27毫克/分升)，高水平肌酐(1.18毫克/分升)和尿素(75毫克/分升)。男孩在实验室和临床均表现为第4期肾功能不全(GFR-22ml/min)。静脉补液、使用毛囊酶和利尿剂使尿酸逐渐降低并恢复正常。RTG和CT检查显示纵隔增宽(暗示肿瘤)，胸膜和心包积液。根据临床表现和实验室结果，初步诊断为t细胞急性淋巴细胞白血病，并通过骨髓造影和免疫学检查证实。根据ALL IC BFM 2009立即对中度风险ALL进行治疗。诱导期出现肾功能不全的临床生化征象。患者需要血液透析，同时补钙(ca -0,81 mmol/L)和高磷血症管理(p -12,42mmol/L)。男孩出现了手足搐搦的症状，在钙恢复正常后症状消失了
{"title":"Adverse Course of Listeria monocytogenes Infection in Pediatric Patient with T-Cell Acute Lymphoblastic Leukemia","authors":"A. Płotka","doi":"10.26420/ANNHEMATOLONCOL.2019.1240","DOIUrl":"https://doi.org/10.26420/ANNHEMATOLONCOL.2019.1240","url":null,"abstract":"Oncological patients are at an increased risk of an opportunistic infection caused by Listeria monocytogenes . Listeriosis remains infrequent in oncological patients. This article describes a case report of a 15-year old boy diagnosed with T-ALL, who developed L.monocytogenes infection during an intensive cancer treatment. The patient’s treatment proceeded with numerous complications. Impaired coagulation led to genetic examination and factor V Leiden and heterozygotic C667T and A1298C mutations of MTHFR detection. Corticosteroid therapy resulted in insulin resistance and hyperbilirubinemia with normal hepatic enzymes level. Gilbert’s syndrome was confirmed. CT evaluating remission revealed asymptomatic invasive pulmonary aspergillosis. After finishing Protocol II the rise in CRP was observed with decreased number of WBC. Patient was feverish and complained of nonspecific abdominal pain and dysuria. USG detected fluid in right iliac fossa. A few hours later boy’s neurological state dramatically worsened. CT revealed acute hydrocephalus, which needed immediate drainage. Listeria monocytogenes was isolated from CSF. Despite intensive antibiotic therapy, infection caused boy’s death. On clinical examination massive submandibular and cervical lymphadenopathy, petechiae all over the body, especially severe in pelvic girdle, splenomegaly (spleen reaching lower quadrant) and hepatomegaly (3cm above subcostal arch), tachycardia (150/min), on auscultation diminished vesicular murmur paraspinal on both sides were noticed. Blood tests revealed leukocytosis (104 ths./µl), III grade thrombocytopenia (22ths/µl) and hyperuricemia (27mg/dl), high level of creatinine (1,18mg/dl) and urea (75mg/dl). Boy presented with, both laboratorary and clinically, renal insufficiency in 4 th stage (GFR-22ml/min). Intravenous rehydration, rasburicase and diuretics were administered resulting in gradual decrease and normalisation of uric acid. RTG and CT examinations revealed widened mediastinum (implying tumour), pleural and pericardial effusion. Due to clinical presentation and laboratory findings, the initial diagnosis of T-cell acute lymphoblastic leukemia was made and confirmed by myelogram and immunological examination of bone marrow. Treatment according to ALL IC BFM 2009 for intermediate-risk ALL was immediately administered. During induction phase clinical and biochemical signs of renal insufficiency occurred. Patient required hemodialysis with calcium supplementation (Ca-0,81 mmol/L) and hyperphosphatemia management at the same time (P-12,42mmol/L). Boy developed symptoms of tetany, which disappeared after normalisation of calcium","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46879239","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 : 2019-02-19DOI: 10.26420/annhematoloncol.2019.1239
Kamal Al-Rabi
The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second most common cause of primary amenorrhea, it is characterized by congenital hypoplasia of the uterus and the upper part of the vagina. The incidence of MRKH syndrome has been estimated as 1 in 4500 women [1]. Most of the studies suggest that MRKH syndrome has been considered as a genetic disease, and genes such as the HOXA7, HOXA9-13, HOXD9-13, and WNT4 have been considered as possible offenders [2]. Malignancies of ovaries, uterus and renal were reported in association with this syndrome [3-5]. Here we are reporting the first case of MRKHS associated with AML in literature.
{"title":"AML in Mayer-Rokitansky-Küster-Hauser Syndrome","authors":"Kamal Al-Rabi","doi":"10.26420/annhematoloncol.2019.1239","DOIUrl":"https://doi.org/10.26420/annhematoloncol.2019.1239","url":null,"abstract":"The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second most common cause of primary amenorrhea, it is characterized by congenital hypoplasia of the uterus and the upper part of the vagina. The incidence of MRKH syndrome has been estimated as 1 in 4500 women [1]. Most of the studies suggest that MRKH syndrome has been considered as a genetic disease, and genes such as the HOXA7, HOXA9-13, HOXD9-13, and WNT4 have been considered as possible offenders [2]. Malignancies of ovaries, uterus and renal were reported in association with this syndrome [3-5]. Here we are reporting the first case of MRKHS associated with AML in literature.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46552849","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 : 2019-02-18DOI: 10.26420/ANNHEMATOLONCOL.2019.1237
Yeh Tc
reaction from metaphysis or diaphysis in the proximal phalanx of the right middle finger (Figure 2). Fine needle aspiration cytology was performed suggestive of an inflamed granulation tissue with atypical cells displaying irregular nuclei and ample cytoplasm. Immunohistochemical study of the atypical cells showed positive staining of CD1a, CD5, CD68 (KP1), Langerin, S-100 protein, and negative staining of CD20. The findings confirmed the diagnosis of Langerhans cell histiocytosis. The patient did not receive any treatment, but routine follow-up. One year later, the patient was asymptomatic and in good health.
{"title":"Langerhans Cell Histiocytosis in a Five-Month-Old Infant","authors":"Yeh Tc","doi":"10.26420/ANNHEMATOLONCOL.2019.1237","DOIUrl":"https://doi.org/10.26420/ANNHEMATOLONCOL.2019.1237","url":null,"abstract":"reaction from metaphysis or diaphysis in the proximal phalanx of the right middle finger (Figure 2). Fine needle aspiration cytology was performed suggestive of an inflamed granulation tissue with atypical cells displaying irregular nuclei and ample cytoplasm. Immunohistochemical study of the atypical cells showed positive staining of CD1a, CD5, CD68 (KP1), Langerin, S-100 protein, and negative staining of CD20. The findings confirmed the diagnosis of Langerhans cell histiocytosis. The patient did not receive any treatment, but routine follow-up. One year later, the patient was asymptomatic and in good health.","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49568204","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 : 2019-02-18DOI: 10.26420/ANNHEMATOLONCOL.2019.1238
S. F. Teunissen
{"title":"Presence of Cryoglobulins in Blood Smear","authors":"S. F. Teunissen","doi":"10.26420/ANNHEMATOLONCOL.2019.1238","DOIUrl":"https://doi.org/10.26420/ANNHEMATOLONCOL.2019.1238","url":null,"abstract":"","PeriodicalId":72219,"journal":{"name":"Annals of hematology & oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41472394","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}
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