Haematologica最新文献_第4页

Genetic and clinical characteristics of patients with Shwachman Diamond syndrome with special consideration of treatment with granulocyte-colony stimulating factor.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-27 DOI: 10.3324/haematol.2024.286119

Sabine Mellor-Heineke, Julia Skokowa, Natali Gerschmann, Ekaterina Deordieva, Ivan Tesakov, Sally Kinsey, Maja Klaudel-Dreszler, Piero Farruggia, Martina Sukova, Mikael Sundin, Yasmine El Chazli, Tania N Masmas, Leen Willems, Georg Ebetsberger-Dachs, Hans C Erichsen, Orna Steinberg-Shemer, Anna Shcherbina, Karl Welte, Cornelia Zeidler

引用次数: 0

MAPK-kinase mutations and aortic lesions are associated with distribution of circulating monocytes in histiocytosis.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-27 DOI: 10.3324/haematol.2024.286716

Jerome Razanamahery, Helene Greigert, Matthias Papo, Jean-Francois Emile, Julien Guy, Francesco Pegoraro, Julien Haroche, Sylvain Audia, Maxime Samson, Bernard Bonnotte

引用次数: 0

Cytogenetic abnormalities in polycythemia vera: phenotypic correlates and prognostic relevance in 669 informative cases.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-27 DOI: 10.3324/haematol.2025.287569

Moazah Iftikhar, Masooma Rana, Yamna Jadoon, Maymona Abdelmagid, Kaaren Reichard, Cinthya Zepeda Mendoza, Animesh Pardanani, Ayalew Tefferi, Naseema Gangat

引用次数: 0

Pseudothrombocytopenia and other conditions associated with spuriously low platelet counts.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-27 DOI: 10.3324/haematol.2024.286234

Marco Cattaneo

{"title":"Pseudothrombocytopenia and other conditions associated with spuriously low platelet counts.","authors":"Marco Cattaneo","doi":"10.3324/haematol.2024.286234","DOIUrl":"https://doi.org/10.3324/haematol.2024.286234","url":null,"abstract":"Accurate measurements of the platelet count are necessary to diagnose thrombocytosis or thrombocytopenia correctly, gauge the severity of the clinical risk and identify the most appropriate therapeutic intervention. Despite increased diagnostic accuracy with the electronic counters, it is still unsatisfactory in rare situations. Conditions causing spurious thrombocytopenia include the following. 1) Pre-analytical errors, such as difficult venipunctures, over-/under-filling of blood collection tubes, insufficient mixing of blood with the anticoagulant (EDTA), which may cause fibrin formation; 2) EDTA-induced, temperature- and time-dependent, antibody-mediated in vitro platelet agglutination, with consequent reduction in the number of single platelets in the sample; the condition, referred to as Pseudothrombocytopenia, is benign and does not need follow-up or medical interventions; the use of alternative in vitro anticoagulants does not prevent agglutinates formation in all samples; accurate platelet counts could be obtained by testing EDTAsamples immediately after blood collection. 3) EDTA-induced in vitro platelet adherence to leukocytes (Platelet Satellitism), caused by bridging IgG antibodies binding to GPIIb-IIIa on platelets and the Fc receptor-III on leukocytes; occasionally, leukocytes may phagocytose platelets and/or form platelets/leukocytes clumps. 4) Presence of large/giant platelets (commonly from patients with congenital or acquired thrombocytopenia) that are not recognized as such by electronic counters, which distinguish platelets from other cells based on their smaller size. 5) Blood from patients with type 2B von Willebrand Disease, which may display large/giant platelets and platelet agglutinates. All the above conditions are easily identifiable by microscopic examination of anticoagulated peripheral blood smears, which is an indispensable diagnostic procedure in hematology.","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":""},"PeriodicalIF":8.2,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143718149","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

New insights into the generation and function of megakaryocytes in health and disease.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-27 DOI: 10.3324/haematol.2024.287236

Changhong Du, Jun Chen, Junping Wang

{"title":"New insights into the generation and function of megakaryocytes in health and disease.","authors":"Changhong Du, Jun Chen, Junping Wang","doi":"10.3324/haematol.2024.287236","DOIUrl":"https://doi.org/10.3324/haematol.2024.287236","url":null,"abstract":"Megakaryocytes (MKs) are canonically viewed as specialized hematopoietic cells merely producing platelets and generated through a series of hematopoietic stem and progenitor cells in the bone marrow (BM). Despite essential physiological functions, the generation and function of MKs remain incompletely understood. Recent studies, almost in mice, have begun to redefine the cellular hierarchy of megakaryopoiesis, and shed new light on the alternative routes and mechanisms of megakaryopoiesis. Moreover, the conception of MKs as a homogenous cell population with the sole purpose of platelet production is being challenged. Conversely, MKs are shown to be a heterogenous cell population with distinct differentiation routes and versatile functions. Especially, MKs show abilities reminiscent of immune cells, and are increasingly considered as the root dictating the hemostatic and immune functions of platelets in various physiopathological conditions. Furthermore, although been well known as a component of the BM niche maintaining hematopoietic stem cells (HSCs) during homeostasis, the newly-identified properties of MKs further make them a key supporter for stressed or diseased HSCs during myeloablative injury, aging and hematopoietic malignancy. Therefore, the generation and function of MKs are more diverse than we previously thought. Here, we review the recent literatures that expand our views of MK differentiation and heterogeneity, as well as of MK functions with special focuses on their immune functions and supporting roles for stressed or diseased HSCs.","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":""},"PeriodicalIF":8.2,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143718804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Teclistamab therapy for refractory type 1 cryoglobulinemia

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-20 DOI: 10.3324/haematol.2025.287313

Justin Battaglini, Joel Henderson, Vaishali Sanchorawala, Raphael Szalat

引用次数: 0

Isatuximab plus bortezomib, lenalidomide, and dexamethasone for transplant-ineligible newly diagnosed multiple myeloma patients: a frailty subgroup analysis of the IMROZ trial.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-20 DOI: 10.3324/haematol.2024.287200

Salomon Manier, Meletios-Athanasios Dimopoulos, Xavier P Leleu, Philippe Moreau, Michele Cavo, Hartmut Goldschmidt, Robert Z Orlowski, Muriel Tron, Christina Tekle, Marie-France Brégeault, Andrea T Shafer, Meral Beksac, Thierry Facon

{"title":"Isatuximab plus bortezomib, lenalidomide, and dexamethasone for transplant-ineligible newly diagnosed multiple myeloma patients: a frailty subgroup analysis of the IMROZ trial.","authors":"Salomon Manier, Meletios-Athanasios Dimopoulos, Xavier P Leleu, Philippe Moreau, Michele Cavo, Hartmut Goldschmidt, Robert Z Orlowski, Muriel Tron, Christina Tekle, Marie-France Brégeault, Andrea T Shafer, Meral Beksac, Thierry Facon","doi":"10.3324/haematol.2024.287200","DOIUrl":"https://doi.org/10.3324/haematol.2024.287200","url":null,"abstract":"Patients with multiple myeloma meeting frailty criteria have worse outcomes than those identified as non-frail. Here, we present a post-hoc subgroup analysis of IMROZ, a global, phase III, open-label study investigating isatuximab (Isa) with bortezomib, lenalidomide, and dexamethasone (VRd) followed by Isa-Rd (n=265) versus VRd followed by Rd (n=181) in newly diagnosed transplant-ineligible multiple myeloma (Ti NDMM) patients using the simplified International Myeloma Working Group (sIMWG) frailty score. Although patients aged >80 were excluded, there was no exclusion for patients meeting frailty criteria. All patients received standard VRd/Rd dosing; Isa-VRd patients received intravenous Isa (cycle 1, 10 mg/kg QW; cycles 2-17, Q2W; subsequent cycles, Q4W). Patients with a frailty score of 0/1 were considered non-frail; scores ≥2 were frail. Using this scoring, 26.7% of patients were frail (26.0% Isa-VRd; 27.6% VRd), and 72.0% non-frail (72.8% Isa-VRd; 70.7% VRd). After a median follow-up of 59.7 months, Isa-VRd significantly improved progression-free survival versus VRd in frail patients (hazard ratio [HR] 0.518; 95% confidence interval [CI] 0.294-0.912, P=0.0227) and non-frail patients (HR 0.615; 95% CI 0.419-0.903, P=0.0131). Significantly more frail patients receiving Isa-VRd than VRd achieved minimal residual disease negativity and complete response (odds ratio 3.459 [95% CI 1.495-8.006], P=0.0030, 10-5 by next-generation sequencing). Rates of treatment-emergent adverse events leading to definitive discontinuation were similar between both arms regardless of frailty status. This post-hoc subgroup analysis of the IMROZ trial demonstrated that Isa-VRd is an effective option with a manageable safety profile for frail patients with Ti NDMM.","PeriodicalId":12964,"journal":{"name":"Haematologica","volume":" ","pages":""},"PeriodicalIF":8.2,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143663419","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

NPM1 mutation subtype switch in acute myeloid leukemia.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-20 DOI: 10.3324/haematol.2024.286916

Benjamin Podvin, Romane Joudinaud, Christophe Roumier, Augustin Boudry, Laure Farnault, Mélinda Dantec, Céline Berthon, Florent Dumezy, Laure Goursaud, Maxime Bucci, Claude Preudhomme, Nicolas Duployez

引用次数: 0

Predictors of response to pacritinib therapy and survival among 60 Mayo Clinic patients with myelofibrosis treated outside of clinical trials.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-20 DOI: 10.3324/haematol.2025.287576

Maymona Abdelmagid, Jeanne M Palmer, Aref Al-Kali, Mehrdad Hefazi, James Foran, Kebede H Begna, Cecilia Y Arana Yi, Animesh Pardanani, Naseema Gangat, Ayalew Tefferi

引用次数: 0

Lower incidence of chronic graft-versus-host disease after ruxolitinib plus extracorporeal photopheresis versus ruxolitinib alone in steroid-refractory acute graft-versus-host disease following allogeneic stem cell transplantation.

IF 8.2 1区医学

Haematologica Pub Date : 2025-03-20 DOI: 10.3324/haematol.2024.286824

Iryna Lastovytska, Silke Heidenreich, Evgeny Klyuchnikov, Christian Niederwieser, Nico Gagelmann, Johanna Richter, Radwan Massoud, Kristin Rathje, Tetiana Perekhrestenko, Gaby Zeck, Catherina Lück, Dietlinde Janson, Christine Wolschke, Francis Ayuk, Nicolaus Kröger

引用次数: 0