eJHaem最新文献 - Book学术

Aleukemic congenital leukemia cutis preceding monocytic leukemia with favorable outcome: A case report 先于单核细胞白血病的先天性Aleukemic切缘白血病，预后良好：病例报告

eJHaem Pub Date : 2024-02-13 DOI: 10.1002/jha2.861

Caroline Lo Presti, Gautier Szymanski, Christine Lefebvre, Marie‐Christine Jacob, Pascal Mossuz, Hugo Gil

引用次数: 0

Haemoglobin Ottawa, sickle cell trait and vaso‐occlusive crises 血红蛋白渥太华、镰状细胞性状和血管闭塞性危象

eJHaem Pub Date : 2024-02-12 DOI: 10.1002/jha2.857

Cécile Bobillier, Isabelle Derclaye, Augustin Ferrant, Diane Maisin

{"title":"Haemoglobin Ottawa, sickle cell trait and vaso‐occlusive crises","authors":"Cécile Bobillier, Isabelle Derclaye, Augustin Ferrant, Diane Maisin","doi":"10.1002/jha2.857","DOIUrl":"https://doi.org/10.1002/jha2.857","url":null,"abstract":"A girl with a sickle cell trait had severe VOCs (vaso‐occlusive crises), her father also had a sickle cell trait but mild VOCs, and her mother had no symptoms. Electrophoresis on agarose gel under alkaline conditions showed haemoglobin AS (HbAS) in the girl and in her father, with an S band increased more than expected (46.2% and 41.2% respectively), and a band migrating at C (16.8% and 8.9% respectively) in both. There was a band at S (19.6 %) in her mother. The C band was attributed to a hybrid tetramer with haemoglobin S (HbS) and a Hb variant. A homozygous c.46G>C mutation (Hb Ottawa, the Hb variant) was detected by Sanger sequencing in the girl. Heterozygosity for Hb Ottawa by Sanger sequencing was shown in both the father and the mother. The father, with HbAS and heterozygous for Hb Ottawa, had mild VOCs. Heterozygosity only for Hb Ottawa did not produce any abnormality in the mother. A sister and two brothers of the index patient presented a Hb variant, probably Hb Ottawa, migrating to the S zone (all 20%) at electrophoresis, without HbS. These last three were asymptomatic. We conclude that Hb Ottawa, an α‐globin variant, contributes along with haemoglobin S (HbS) to VOC symptoms.","PeriodicalId":504828,"journal":{"name":"eJHaem","volume":"77 24","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139784615","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}

引用次数: 0

Lisocabtagene maraleucel for treatment of relapsed and refractory primary mediastinal large B‐cell lymphoma in an adolescent patient Lisocabtagene maraleucel 用于治疗一名青少年患者的复发和难治性原发性纵隔大 B 细胞淋巴瘤

eJHaem Pub Date : 2024-02-12 DOI: 10.1002/jha2.859

Dasom Lee, Anmol Goyal, William L Wang, Snegha Ananth, Eric Lau, Michael S Binkley, S. Bharadwaj, Saurabh Dahiya

引用次数: 0

Polycythemia caused by erythropoietin‐producing hemangiosarcoma 生成红细胞生成素的血管肉瘤引起的多血细胞症

eJHaem Pub Date : 2024-02-12 DOI: 10.1002/jha2.860

Osamu Imataki, Makiko Uemura

引用次数: 0

Autoimmune thrombotic thrombocytopenic purpura associated with disseminated sarcoidosis: A case report 与播散性肉样瘤病相关的自身免疫性血栓性血小板减少性紫癜：病例报告

eJHaem Pub Date : 2024-02-11 DOI: 10.1002/jha2.848

Maxime Billotte, Peter Eszto, Benoît Thomas, Pierre Groizard, Pierre Perez, Julien Campagne

引用次数: 0

Changes in bone marrow fibrosis during momelotinib or ruxolitinib therapy do not correlate with efficacy outcomes in patients with myelofibrosis 莫美洛替尼或鲁索利替尼治疗期间骨髓纤维化的变化与骨髓纤维化患者的疗效结果无关

eJHaem Pub Date : 2024-02-05 DOI: 10.1002/jha2.854

Stephen T. Oh, S. Verstovsek, Vikas Gupta, U. Platzbecker, Timothy Devos, J. Kiladjian, D. McLornan, Andrew Perkins, M. Fox, M. McMullin, Adam J. Mead, M. Egyed, J. Mayer, Tomasz Sacha, J. Kawashima, Mei Huang, Bryan Strouse, R. Mesa

{"title":"Changes in bone marrow fibrosis during momelotinib or ruxolitinib therapy do not correlate with efficacy outcomes in patients with myelofibrosis","authors":"Stephen T. Oh, S. Verstovsek, Vikas Gupta, U. Platzbecker, Timothy Devos, J. Kiladjian, D. McLornan, Andrew Perkins, M. Fox, M. McMullin, Adam J. Mead, M. Egyed, J. Mayer, Tomasz Sacha, J. Kawashima, Mei Huang, Bryan Strouse, R. Mesa","doi":"10.1002/jha2.854","DOIUrl":"https://doi.org/10.1002/jha2.854","url":null,"abstract":"Bone marrow fibrosis (BMF) is a pathological feature of myelofibrosis, with higher grades associated with poor prognosis. Limited data exist on the association between outcomes and BMF changes. We present BMF data from Janus kinase (JAK) inhibitor–naive patients from SIMPLIFY‐1 (NCT01969838), a double‐blind, randomized, phase 3 study of momelotinib vs ruxolitinib. Baseline and week 24 bone marrow biopsies were graded from 0 to 3 as per World Health Organization criteria. Other assessments included Total Symptom Score, spleen volume, transfusion independence status, and hemoglobin levels. Paired samples were available from 144 and 160 patients randomized to momelotinib and ruxolitinib. With momelotinib and ruxolitinib, transfusion independence was achieved by 87% and 44% of patients with BMF improvement of ≥1 grade and 76% and 56% of those with stable/worsening BMF; there was no association between BMF changes and transfusion independence for either arm (momelotinib, p = .350; ruxolitinib, p = .096). Regardless of BMF changes, hemoglobin levels also generally increased on momelotinib but decreased on ruxolitinib. In addition, no associations between BMF changes and spleen (momelotinib, p = .126; ruxolitinib, p = .407)/symptom (momelotinib, p = .617; ruxolitinib, p = .833) outcomes were noted, and no improvement in overall survival was observed with ≥1‐grade BMF improvement (momelotinib, p = .395; ruxolitinib, p = .407). These data suggest that the anemia benefit of momelotinib is not linked to BMF changes, and question the use of BMF assessment as a surrogate marker for clinical benefit with JAK inhibitors.","PeriodicalId":504828,"journal":{"name":"eJHaem","volume":"29 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139866055","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}

引用次数: 0

Allogeneic stem cell transplant recipients surviving at least 2 years without relapse: outcome and risk factors 异体干细胞移植受者存活至少两年且未复发：结果和风险因素

eJHaem Pub Date : 2024-02-05 DOI: 10.1002/jha2.842

B. L. Grønvold, M. Ali, T. Myklebust, Andrea Lenartova, Mats Remberger, I. Abrahamsen, G. Tjønnfjord, A. Myhre, Y. Fløisand, T. Gedde-Dahl

{"title":"Allogeneic stem cell transplant recipients surviving at least 2 years without relapse: outcome and risk factors","authors":"B. L. Grønvold, M. Ali, T. Myklebust, Andrea Lenartova, Mats Remberger, I. Abrahamsen, G. Tjønnfjord, A. Myhre, Y. Fløisand, T. Gedde-Dahl","doi":"10.1002/jha2.842","DOIUrl":"https://doi.org/10.1002/jha2.842","url":null,"abstract":"Outcomes of 2‐year survivours undergoing allo‐haematopoietic stem cell transplantation at Oslo University Hospital were retrospectively assessed with the objectives of identification of risk factors for late death as possible means for precautionary measures and interventions to improve long‐term survival.421 patients with haematological malignancy, transplanted between 2005 and 2019, alive and free of disease after 2 years were included with data reported from The OUS‐HSCT registry. Median follow‐up was 6.2 years (2.016.1), and 232 patients (55%) were observed for minimum 5 years.The probability of being alive 5 and 10 years after HSCT was 86% and 76%. Primary risk factors for late death included initial diagnosis of age ≥ 60 years, chronic lymphocytic leukaemia (CLL), previous blood stream‐ or invasive fungal infection (BSI, IFI), and chronic graft‐versus‐host disease (cGVHD). Transplant‐related mortality (TRM) and relapse at 5 years were 9.0% and 7.7%, respectively. Two factors were associated with the latter: cytomegalovirus (CMV) seronegative donor and CLL. Compared with the age‐ and gender‐matched Norwegian general population, life expectancy was lower for each disease, except for CML.The prospect for the long‐term survival is good for 2‐year survivors of the allogeneic hematopoietic stem cell transplantation. However, life expectancy remains inferior to the age‐ and gender‐matched general population. Optimising prophylaxis and treatment for chronic GVHD, BSI and IFI are needed along with the improved adherence to guidelines for early detection of secondary malignancies. Measures to improve immune reconstitution, possibly the microbiota, and the use of CMV seropositive donors regardless of recipient sero‐status may be warranted and should be addressed in further studies.","PeriodicalId":504828,"journal":{"name":"eJHaem","volume":"242 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139862436","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}

引用次数: 0