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BOSUTINIB TREATMENT OF CHRONIC MYELOID LEUKEMIA IN LOMBARDY. Acta Haematol. (IF 1.7) Pub Date : 2024-09-18 Alessandra Iurlo,Cristina Bucelli,Tamara Intermesoli,Chiara Elena,Mariella D'Adda,Elena Agostani,Cristina Fiamenghi,Margherita Maffioli,Nicola Orofino,Francesca Lunghi,Angelo Gardellini,Maria Cristina Carraro,Alessandro Inzoli,Federica Gigli,Roberto Palazzolo,Vanda Bertolli,Daniele Cattaneo,Ester Maria Pungolino,Carlo Gambacorti-Passerini
Introduction Up to 30% of CML patients will require a therapeutic change during follow-up, due to intolerance and/or resistance to first-line TKI approach. In this context, bosutinib (BOS) has not only demonstrated its effica-cy, but also presents a favorable safety profile, without comorbid conditions representing an absolute contraindication to its use. Methods To gain further into BOS treatment
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Beneficial effect of integrated nutritional interventions in patients with hematological diseases undergoing hematopoietic stem cell transplant. Acta Haematol. (IF 1.7) Pub Date : 2024-09-12 Mimi Geng,Zihui Sun
INTRODUCTION The nutritional status of patients undergoing hematopoietic stem cell transplantation (HSCT) is critically important. This study was aimed to assess the impact of comprehensive nutritional interventions on the well-being of individuals with hematological diseases who underwent HSCT. METHODS A total of 175 patients with hematological diseases who underwent HSCT were included, with 94 in
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Outcomes of Fedratinib in routine Treatment of ruxolitinib-resistant or refractory patients with Primary and post-polycythemia vera or essential thrombocythemia Myelofibrosis: A nationalwide retrospective study. Acta Haematol. (IF 1.7) Pub Date : 2024-09-12 Adrian Duek,Alexandra Tzinman,Kira Maziuk,Assaf Levy,Martin Ellis,Galia Stemer,Adi Shacham Abulafia,Amos Cohen,Noa Lavi,Aaron Ronson,Andrey Braester,Shirley Shapira,Jonathan Canaani,Yulia Volchek,Ronit Leiba,Merab Leiba
INTRODUCTION In recent years, fedratinib, a selective JAK2 inhibitor, has emerged as a potential therapeutic option for patients who have failed or are intolerant to ruxolitinib. Despite the promising results observed in clinical studies, real-world evidence from the United States and Europe suggests that the efficacy of fedratinib may be less conclusive. We report the characteristics, treatment patterns
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Real-world impact of routine addition of anti-thymocyte globulin to standard GVHD prophylaxis in myeloablative unrelated donor transplants: important gains in graft vs host disease prevention though no difference in overall survival. Acta Haematol. (IF 1.7) Pub Date : 2024-08-28 Ni Bai,Wasithep Limvorapitak,Robert Henderson,Yasser Abou Mourad,Shanee Chung,Donna Forrest,Kevin Hay,Florian Kuchenbauer,Stephen Nantel,Sujaatha Narayanan,Thomas Nevill,Maryse Power,Judith Rodrigo,Claudie Roy,David Sanford,Kevin Song,Ryan Stubbins,Heather Sutherland,Cynthia Toze,Jennifer White
INTRODUCTION Anti-thymocyte globulin (ATG) has been demonstrated to reduce the incidence of graft-versus-host disease (GVHD); however, it remains controversial whether these gains are offset by an increase in relapse. METHODS We conducted a retrospective historical control study consisting patients (n=210) who underwent myeloablative allogeneic hematopoietic stem-cell transplantation (HSCT) from 2014
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Prognostic value of the pretransplant fibrosis-4 (FIB-4) index on nonrelapse and overall mortality following unrelated single-unit cord blood transplantation in adults. Acta Haematol. (IF 1.7) Pub Date : 2024-08-28 Takaaki Konuma,Maki Monna-Oiwa,Seiko Kato,Masamichi Isobe,Satoshi Takahashi,Yasuhito Nannya
INTRODUCTION The fibrosis-4 (FIB-4) index is a non-invasive marker of liver fibrosis. The FIB-4 index predicts poor outcomes in patients with hepatic and non-hepatic diseases. However, the association of the FIB-4 index with mortality and liver-related clinical outcomes following cord blood transplantation (CBT) is unclear. METHODS We retrospectively evaluated the impact of the pretransplant FIB-4
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Outcomes of Patients with Myeloid Malignancies and Cardiovascular Disease Undergoing Allogeneic Stem Cell Transplantation. Acta Haematol. (IF 1.7) Pub Date : 2024-08-27 Gabriela Sanchez-Petitto,Olga Goloubeva,James Childress,Tahreem Iqbal,Jack Masur,Max An,Safwan Muhammad,Justin Lawson,Grace Li,Brian Barr,Ashkan Emadi,Vu H Duong,Nancy M Hardy,Aaron P Rapoport,Maria R Baer,Sandrine Niyongere,Jean Yared
INTRODUCTION/BACKGROUND Reduced-intensity conditioning (RIC) and non-myeloablative (NMA) regimens have enabled patients with cardiovascular disease (CVD) to undergo allogeneic stem cell transplantation (allo-HSCT). However, little is known about long-term outcomes, including cardiovascular (CV) complications. METHODS We retrospectively studied 99 consecutive patients with acute myeloid leukemia (AML)
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Disseminated Mucormycosis and T-Cell-Depleted Allogeneic Stem Cell Transplantation: An Unusual Case Study. Acta Haematol. (IF 1.7) Pub Date : 2024-08-21 Oana Diana Dragoi,Mili Shah,Victoria Potter,Daniele Avenoso,Pramila Krishnamurthy,Alireza Abdolrasouli,Silke Schelenz,Julie Chandra,Varun Mehra
INTRODUCTION Invasive fungal infections are a primary cause of morbidity and mortality in patients with haematological malignancies. CASE PRESENTATION We describe an unusual clinical and radiological presentation of invasive mucormycosis (IM) in a 69-year-old patient with relapsed acute myeloid leukaemia. The patient was diagnosed with disseminated IM with involvement of the central nervous system
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The Latin-American Experience in POEMS Syndrome: A Study of the GELAMM (Grupo de Estudio Latinoamericano de Mieloma Múltiple). Acta Haematol. (IF 1.7) Pub Date : 2024-08-10 Moisés Manuel Gallardo-Pérez,Paola Negrete-Rodríguez,Morie A Gertz,Camila Peña,Eloisa Riva,Virginia Gilli,Gloritza Rodríguez,César Samánez,Joaquín Ferreira,Sergio Portiño,Jacqueline Montaña,Pilar León,Yaima Gutiérrez,Caroline Del-Castanhel,Cristian Seehaus,Maria Eugenia Funes,Rodrigo Meneces-Bustillo,Patricio Duarte,Claudia Shanley,Giannini Elvira,Paola Ochoa,Hernán López-Vidal,Humberto Martinez-Cordero
INTRODUCTION POEMS syndrome is a rare paraneoplastic syndrome caused by an underlying plasma cell disorder. The acronym refers to the following features: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal paraproteinemia, and skin changes. METHODS The study was conducted at 24 hematological centers across 8 Latin-American countries. The study included a total of 46 patients {median age
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A Case of Recurrent Localized Pulmonary Nodular Light Chain Amyloidosis Treated with Daratumumab plus CyBorD. Acta Haematol. (IF 1.7) Pub Date : 2024-07-29 Ted Raddell,Farah Ashraf,Xiaofeng Zhao,Osheen Abramian,Tulin Budak-Alpdogan
INTRODUCTION Nodular pulmonary amyloidosis (NPA) is a localized form of light chain (AL) amyloidosis often found incidentally and typically has an indolent and benign disease course treated with resection or local excision. We present a patient with recurrent localized AL amyloidosis who required further treatment. CASE PRESENTATION A 63-year-old female with monoclonal gammopathy of undetermined significance
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Prognostic Factors for Chronic Thrombocytopenia in Systemic Lupus Erythematosus with Immune Thrombocytopenia. Acta Haematol. (IF 1.7) Pub Date : 2024-07-29 Soo Min Ahn,Eun-Ji Choi,Ji Seon Oh,Yong-Gil Kim,Chang-Keun Lee,Bin Yoo,Seokchan Hong
INTRODUCTION We aimed to identify the clinical characteristics and risk factors for chronic immune thrombocytopenia (ITP) in patients with systemic lupus erythematosus (SLE). METHODS We retrospectively reviewed patients diagnosed with SLE-associated ITP between January 2000 and December 2021. Patient characteristics were analyzed according to the progression of chronic thrombocytopenia. No response
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Long-Term Follow-Up of Eltrombopag Treatment for Patients with Cyclosporin A Refractory/Relapsed Transfusion-Dependent Non-Severe Aplastic Anemia: A Report from a Single Center in China. Acta Haematol. (IF 1.7) Pub Date : 2024-07-17 Qinglin Hu,Yuan Yang,Chen Yang,Miao Chen,Bing Han
INTRODUCTION Aplastic anemia (AA) is characterized by bone marrow failure and cytopenia. Eltrombopag (ELT) is effective and safe for treating refractory/relapsed AA; however, reports on the long-term outcomes of transfusion-dependent non-severe AA (TD-NSAA) are limited. METHODS Patients with TD-NSAA refractory to immunosuppressive therapy (IST) or relapsed after IST, treated with ELT alone, and followed
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Factor VIII Levels and ISTH Disseminated Intravascular Coagulation Scores Do Not Distinguish Disseminated Intravascular Coagulation from the Coagulopathy of Liver Disease. Acta Haematol. (IF 1.7) Pub Date : 2024-07-16 Cecily Allen,Marina Heskel,Ayesha Butt,Christopher Tormey,Alexander B Pine,Alfred I Lee,Samir Gautam
INTRODUCTION Distinguishing disseminated intravascular coagulation (DIC) from the coagulopathy of liver disease represents a common clinical challenge. Here, we evaluated the utility of two diagnostic tools frequently used to differentiate between these conditions: factor VIII (FVIII) levels and the International Society on Thrombosis and Hemostasis (ISTH) DIC score. METHODS To this end, we conducted
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Enhanced Survival of Chronic Myelomonocytic Leukemia-Dysplastic over Proliferative Subtype after Allogeneic Hematopoietic Cell Transplant: A Tertiary Center Experience and Literature Review. Acta Haematol. (IF 1.7) Pub Date : 2024-06-26 Hunter D Niehus,Jean Sabile,Richard T Maziarz,Gabrielle Meyers,Rachel Cook,Arpita P Gandhi,Jennifer N Saultz,Shauna Rakshe,Andy Kaempf,Theodore Braun,Yazan Migdady
INTRODUCTION CMML is a rare neoplasm with overlapping myelodysplastic and myeloproliferative features whose only potential cure is allogeneic hematopoietic cell transplantation (allo-HCT). METHODS This retrospective study examined 27 CMML patients with high-risk clinical features who underwent first allo-HCT at our institution between 2004 and 2022. RESULTS Nineteen patients were diagnosed with the
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Effect of Digital Health Coaching on Self-Efficacy and Patient-Reported Outcomes in Individuals with Acute Myeloid and Chronic Lymphocytic Leukemia: A Pilot Randomized Controlled Trial. Acta Haematol. (IF 1.7) Pub Date : 2024-06-11 Jennifer Marvin-Peek,Valerie Shelton,Kelly Brassil,Bryan Fellman,Austin Barr,Kelly Sharon Chien,Danielle Hammond,Mahesh Swaminathan,Nitin Jain,William Wierda,Alessandra Ferrajoli,Courtney DiNardo
INTRODUCTION Promotion of self-efficacy can enhance engagement with health care and treatment adherence in patients with cancer. We report the outcomes of a pilot trial of a digital health coach intervention in patients with leukemia with the aim of improving self-efficacy. METHODS Adult patients with newly diagnosed acute myeloid leukemia (AML) and chronic lymphocytic leukemia (CLL) were randomized
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Safety of Extended Pirtobrutinib Exposure in Relapsed and/or Refractory B-Cell Malignancies. Acta Haematol. (IF 1.7) Pub Date : 2024-06-05 Lindsey E Roeker,Catherine C Coombs,Nirav N Shah,Wojciech Jurczak,Jennifer A Woyach,Chan Y Cheah,Krish Patel,Kami Maddocks,Yucai Wang,Pier Luigi Zinzani,Talha Munir,Youngil Koh,Meghan C Thompson,Catherine E Muehlenbein,Chunxiao Wang,Richard Sizelove,Sarang Abhyankar,Safarulla Hasanabba,Donald E Tsai,Toby A Eyre,Michael Wang
INTRODUCTION Pirtobrutinib, a highly selective, noncovalent (reversible) Bruton tyrosine kinase inhibitor, has demonstrated promising efficacy in B-cell malignancies and is associated with low rates of discontinuation and dose reduction. Pirtobrutinib is administered until disease progression or toxicity, necessitating an understanding of the safety profile in patients with extended treatment. METHODS
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Pharmacokinetic-Guided Hydroxyurea to Reduce Transfusions in Ugandan Children with Sickle Cell Anemia: Study Design of the Alternative Dosing And Prevention of Transfusions Trial. Acta Haematol. (IF 1.7) Pub Date : 2024-06-05 Alexandra Power-Hays,Ruth Namazzi,Charles Kato,Kathryn E McElhinney,Andrea L Conroy,Heather Hume,Chandy John,Sara M O'Hara,Susan E Stuber,Adam Lane,Teresa S Latham,Robert O Opoka,Russell E Ware
INTRODUCTION People with sickle cell anemia (SCA) may require frequent blood transfusions to treat acute and chronic complications. Hydroxyurea is a life-saving treatment for SCA that could also decrease the need for blood transfusions. Inadequate medication access and challenges in dose optimization limit the widespread use of hydroxyurea in Africa. If feasible, pharmacokinetic (PK) dosing might improve
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Biologic and Clinical Characteristics of Isochromosome der(17)(q10)t(15;17) in Acute Promyelocytic Leukemia. Acta Haematol. (IF 1.7) Pub Date : 2024-05-31 Yuchen Liu,Yi Ning,Gabriel Ghiaur,Ashkan Emadi
INTRODUCTION Acute promyelocytic leukemia (APL) is genetically characterized by the fusion of promyelocytic leukemia (PML) gene with retinoic acid receptor alpha (RARα) resulting from a t(15;17)(q24;q21) chromosomal translocation. An infrequent but recurrent finding in APL is the formation of an isochromosome of the derivative chromosome 17; ider(17)(q10)t(15;17) or ider(17q). This rearrangement in
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TES and SLC40A1 as Potential Biomarkers for Predicting Survival in T-Cell Acute Lymphoblastic Leukemia. Acta Haematol. (IF 1.7) Pub Date : 2024-05-28 Xiangyou Zeng,Kaifan Liu,Ruohao Xu,Lenghe Zhang,Peilong Lai,Xin Du,Jianyu Weng
INTRODUCTION Identifying patients with high-risk T-cell acute lymphoblastic leukemia (T-ALL) is crucial for personalized therapy; however, the lack of robust biomarkers hinders prognosis assessment. To address this issue, our study aimed to screen and validate genes whose expression may serve as predictive indicators of outcomes in T-ALL patients while also investigating the underlying molecular mechanisms
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Eosinophilic Pleocytosis in the Cerebrospinal Fluid following CAR-T Cell Therapy for Central Nervous System Lymphoma: A Case for Warning? Acta Haematol. (IF 1.7) Pub Date : 2024-05-24 Mayasa Abu Ata,Israel Henig,Dana Yehudai-Ofir,Inna Tzoran,Shimrit Ringelstein-Harlev,Tsofia Inbar,Ilana Slouzkey,Michal Karmona Fintuch,Anat Stern,Olesya Stanevsky,Michal Weiler-Sagie,Yaniv Zohar,Ido Livneh,Goni Merhav,Ayelet Eran,Tsila Zuckerman,Ofrat Beyar Katz
INTRODUCTION Chimeric antigen receptor T (CAR-T) cell therapy, emerging as an efficient treatment option for patients with secondary central nervous system (CNS) lymphoma, is frequently complicated with immune effector cell-associated neurotoxicity syndrome (ICANS). CASE PRESENTATION We report a case of a 64-year-old woman with transformed follicular lymphoma, developing high-grade ICANS with eosinophilic
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Novel compound heterogeneous mutations in CYB5R3 gene leading to methemoglobinemia (type I) in a Chinese boy. Acta Haematol. (IF 1.7) Pub Date : 2024-05-23 Yeyi Yang,Yezhen Yang,Ye Meng,Lihua Huang,Zuocheng Yang
INTRODUCTION Congenital methemoglobinemia (RCM) caused by CYB5R3 deficiency due to the mutations in the reduced nicotinamide adenine dinucleotide (NADH) cytochrome b5 reductase (CYB5R) gene is an autosomal recessive inherited disease. Clinically, it can be divided into two types, namely red blood cell affected type (RCM I) and systemically affected type (RCM II). CASE PRESENTATION A 5-year-old male
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Single-Center Experience of Patients with Plasma Cell Leukemia in the Era of New Therapeutics. Acta Haematol. (IF 1.7) Pub Date : 2024-05-22 Maria Dampmann,Sarah Flossdorf,Julius Keyl,Hans Christian Reinhardt,Christine Hanoun
INTRODUCTION Plasma cell leukemia (PCL) can occur de novo as primary PCL (pPCL), or in patients with prior diagnosis of multiple myeloma (MM) as secondary PCL (sPCL). In 2021, the diagnostic criteria have been revised, establishing a new cut-off of ≥5% plasma cells in the peripheral blood. Lacking specific clinical trials, PCL is treated similarly to MM; however, outcome for patients with PCL remains
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Racial and Ethnic Characteristics and Outcomes of Patients Diagnosed with CLL/SLL in the USA. Acta Haematol. (IF 1.7) Pub Date : 2024-05-20 Debora S Bruno,Manoj Khanal,Xiaohong I Li,Maricer P Escalon,Katherine B Winfree,Lisa M Hess
INTRODUCTION This study was designed to compare outcomes among patients by race and ethnicity in the post-covalent Bruton tyrosine kinase inhibitor (cBTKi) treatment era. METHODS A nationwide electronic health record (EHR)-derived de-identified database was utilized that included patients diagnosed with CLL from 2013 to 2022 who received systemic therapy for their disease. Use of cBTKi therapy, time
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HLA-DRB5 Overexpression Promotes Platelet Reduction in Immune Thrombocytopenia Mice Model by Facilitating MHC-II-Mediated Antigen Presentation. Acta Haematol. (IF 1.7) Pub Date : 2024-05-15 Yujuan Ren,Qianqian Ying,Ying Chen,Cong Liao,Anrong Li,Qidong Ye
INTRODUCTION Major histocompatibility complex II (MHC-II)-mediated antigen presentation contributes to the pathogenesis of immune thrombocytopenia (ITP). Human leukocyte antigen (HLA)-DRB5 is an MHC-II molecule and this study aims to investigate its role and mechanisms in ITP development. METHODS Guinea pig anti-mouse platelet (PLT) serum-induced ITP mice received tail vein injection of HLA-DRB5 overexpressing
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Real-World Risk of Severe Cytopenias in Multiple Myeloma Patients Sequentially Treated with Immunomodulatory Drugs. Acta Haematol. (IF 1.7) Pub Date : 2024-05-10 Julie Barberio,Timothy L Lash,Ajay K Nooka,Ashley I Naimi,Rachel E Patzer,Christopher Kim
INTRODUCTION Most multiple myeloma (MM) patients experience cytopenias, likely driven by both disease and treatment-related factors. Immunomodulatory agents (IMiDs), which form the backbone of most anti-myeloma regimens, are known to cause higher grade cytopenias. In this context, the impact of sequential IMiD treatments on cytopenia risk is unknown. METHODS We evaluated the cumulative risks of severe
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Toxicity of CAR T-Cell Therapy for Multiple Myeloma. Acta Haematol. (IF 1.7) Pub Date : 2024-05-08 Aimaz Afrough,Pearl Rajan Abraham,Laura Turer,Gurbakhash Kaur,Aishwarya Sannareddy,Doris K Hansen,Larry D Anderson
BACKGROUND Idecabtagene vicleucel (ide-cel) and ciltacabtagene autoleucel (cilta-cel) are novel chimeric antigen receptor (CAR)-T cell therapies targeting B-cell maturation antigen (BCMA), and both have recently gained approval by the U.S. Food Drug Administration (FDA) for treatment of relapsed and refractory multiple myeloma (RRMM). SUMMARY These therapies offer unprecedented responses in RRMM but
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Historical Perspective of High-Dose Therapy Followed by Autologous Stem Cell Transplantation in Multiple Myeloma. Acta Haematol. (IF 1.7) Pub Date : 2024-05-06 Inbar Cohen,Iuliana Vaxman,Morie A Gertz
BACKGROUND High-dose therapy (HDT) followed by autologous stem cell transplantation (ASCT) has become part of standard of care (SOC) in newly diagnosed multiple myeloma. In this review, we provide a historical perspective on ASCT since its introduction in the 1990s. SUMMARY Overall survival (OS) benefit for HDT followed by ASCT was demonstrated in studies comparing HDT with ASCT to standard-dose therapy
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Cytomegalovirus Reactivation during Elotuzumab Therapy in Patients with Multiple Myeloma. Acta Haematol. (IF 1.7) Pub Date : 2024-04-24 Taku Kikuchi,Nobuhiro Tsukada,Kodai Kunisada,Moe Nomura-Yogo,Yuki Oda,Kota Sato,Tomomi Takei,Mizuki Ogura,Yu Abe,Kenshi Suzuki,Tadao Ishida
INTRODUCTION Some treatments are associated with cytomegalovirus (CMV) reactivation (CMVRA) in patients with multiple myeloma (MM). However, no reports exist on the association between elotuzumab and CMVRA. Therefore, we assessed the incidence of CMVRA in patients with MM who received elotuzumab therapy. METHODS The medical records of 85 patients who underwent elotuzumab therapy were included in the
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Long-Term Efficacy and Safety of Damoctocog Alfa Pegol Prophylaxis in Patients with Haemophilia A Aged 12-<18 Years at Enrolment into PROTECT VIII. Acta Haematol. (IF 1.7) Pub Date : 2024-04-23 Mark T Reding,Mindy Simpson,Jonathan Ducore,Pål Andrè Holme,Monika Maas Enriquez,Maria Elisa Mancuso
INTRODUCTION The phase 2/3 PROTECT VIII study demonstrated long-term efficacy and safety of damoctocog alfa pegol (BAY 94-9027; Jivi®), a B-domain-deleted recombinant factor VIII (FVIII), site-specifically PEGylated to improve its pharmacokinetic profile. We report a post hoc assessment of bleeding and safety outcomes in the subgroup of patients, aged 12-<18 years at enrolment. METHOD PROTECT VIII
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A Hematoma in the Anterior Abdominal Muscle in a Woman Receiving Venlafaxine: A Literature Review of the Reports on Similar Cases. Acta Haematol. (IF 1.7) Pub Date : 2024-04-17 Chrysoula Boutari,Evangelia Zarkada,Athanasios Vyzantiadis,Efthymia Vlachaki,Genovefa Mantzou,Cristine Karipidou,Stamatia Theodoridou
INTRODUCTION Venlafaxine (VEN) is a selective norepinephrine reuptake inhibitor (SNRI) that mainly helps treat major depressive disorder and anxiety and panic disorders. It works by inhibiting the reuptake of serotonin (5-hydroxytryptamine, 5-HT) and noradrenaline (NA) by presynaptic neurons. Additionally, VEN administration has been linked with a bleeding predisposition that may be due to the inhibition
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Prognostic Implications of Circulating Plasma Cell Percentage in Multiple Myeloma and Primary Plasma Cell Leukemia Defined by New Criteria. Acta Haematol. (IF 1.7) Pub Date : 2024-04-16 Xianghong Jin,Xianyong Jiang,Hui Li,Kaini Shen,Shuangjiao Liu,Miao Chen,Chen Yang,Bing Han,Junling Zhuang
INTRODUCTION The definition of primary plasma cell leukemia (pPCL) has been revised from ≥20% to ≥5% circulating plasma cells (CPC). However, the precise prognosis associated with CPC remains controversial. This study aimed to investigate prognostic biomarkers for myeloma patients based on CPC presence. METHODS A comprehensive analysis was conducted on 309 consecutive patients diagnosed with either
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Efficacy, Safety and Quality of Life of Pegcetacoplan in Japanese Patients With Paroxysmal Nocturnal Hemoglobinuria Treated Within the Phase 3 PEGASUS Trial. Acta Haematol. (IF 1.7) Pub Date : 2024-04-12 Hisakazu Nishimori,Hideyuki Nakazawa,Shinobu Tamura,Toshiki Uchida,Kensuke Usuki,Johan Szamosi,Régis Peffault de Latour,Alexander Röth,Jens Panse
INTRODUCTION Pegcetacoplan, the first approved proximal complement C3 inhibitor, showed superiority to eculizumab in improving hemoglobin levels and clinical outcomes in the phase 3 PEGASUS study in patients with paroxysmal nocturnal hemoglobinuria (PNH) and inadequate response to eculizumab. METHODS This analysis evaluates the efficacy and safety of pegcetacoplan for Japanese patients in PEGASUS,
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Failure of treatment-free remission after a prolonged deep molecular response in patients with chronic myeloid leukemia. Acta Haematol. (IF 1.7) Pub Date : 2024-04-04 Patrice E Nasnas,Elias J Jabbour,Koji Sasaki,Ghayas C Issa,Lucia Masarova,Nicholas J Short,Fadi G Haddad
Patients with chronic myeloid leukemia in chronic phase (CML-CP) can have a normal life expectancy when treated with the BCR::ABL1 tyrosine kinase inhibitors. In recent years, treatment discontinuation and treatment-free remission (TFR) emerged as the new goal of therapy in patients with CML-CP. Deep and sustained molecular remissions for more than 3 to 5 years are associated with higher chances of
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Consensus-Based Expert Recommendations on the Management of Hemophilia A in the Gulf Region. Acta Haematol. (IF 1.7) Pub Date : 2024-04-02 Hazzaa Abdulla Alzahrani,Ashraf Warsi,Ali Mullah-Ali,Ghazi Alotaibi,Mahmoud Abu-Riash,Mohammad Alshahrani,Mustaqeem Siddiqui,Tarek Owaidah,Shahrukh Hashmi
BACKGROUND Hemophilia A presents a significant health challenge in the Gulf region, where it has an especially high prevalence. There are several unmet needs associated with the management of hemophilia A in the region. The aim of this manuscript was to contextualize unmet management needs, provide recommendations to optimize care, and specify requirements for the establishment of gene therapy centers
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Utility of echocardiogram in neutropenic patients with gram positive bacteremia - a retrospective study. Acta Haematol. (IF 1.7) Pub Date : 2024-03-25 Neta Sternbach,Haim Ben-Zvi,Ofir Wolach,Moshe Yeshurun,Pia Raanani,Dafna Yahav,Liat Shargian
INTRODUCTION Hemato-oncology patients are vulnerable to bloodstream infections due to immunocompromised state and use of intravascular catheters. Data regarding risk of infective endocarditis (IE) among those with gram positive bacteremia is limited. We aimed to evaluate the incidence of IE among neutropenic hemato-oncology patients, and to explore the yield of echocardiogram in this population. METHODS
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The ratio of serum urea nitrogen to albumin is a better predictor of OS in MM patients than urea nitrogen alone. Acta Haematol. (IF 1.7) Pub Date : 2024-03-25 Jiaqi Shao,Enfan Zhang,Haoguang Chen,Zhen Cai,Mengmeng Dong
Introduction Multiple myeloma (MM) is a malignant proliferative disease of plasma cells. Abnormally cloned plasma cells secrete large amounts of monoclonal immunoglobulins in the bone marrow of MM patients. Serum urea nitrogen (sUN) is a byproduct of protein metabolism, and its effect on MM patients' prognoses remains unknown. Therefore, we analysed MM patients' clinical data to explore the role of
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Long Non-coding RNA RP11-252C15.1 is a Potential Biomarker of Prognosis and Hallmark for Leukemogenesis in Children with B-cell Precursor Acute Lymphoblastic Leukemia. Acta Haematol. (IF 1.7) Pub Date : 2024-03-25 Lili Pan,Yongzhi Zheng,Hao Zheng
INTRODUCTION Improved understanding of the prognostic biomarkers associated with childhood acute lymphoblastic leukemia (ALL) is needed for accurate risk group stratification. This study aimed to identify potential long-non coding RNA (lncRNA) markers and evaluate their prognostic value in children with ALL. METHODS We selected 50 children with newly diagnosed ALL and 20 age-matched patients with idiopathic
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The IRE1α-endonuclease plays a dual role in regulating the XBP1/miRNA-34a axis and PD-1 expression within Natural Killer cells in Hodgkin Lymphoma. Acta Haematol. (IF 1.7) Pub Date : 2024-03-13 Karolina Bednarska,Gayathri Thillaiyampalam,Sally Mujaj,Jamie Nourse,Jay Gunawardana,Muhammed B Sabdia,Soi C Law,Anna Pilaar,Qingyan Cui,Lilia M de Long,Frank Vari,Maher K Gandhi,Alexandre S Cristino
INTRODUCTION Hodgkin Lymphoma (HL) is deficient in Major Histocompatibility Complex-class I, rendering it susceptible to anti-tumoral immunity by Natural Killer (NK)-cells. Despite the functional impairment of PD-1+ NK-cells in HL, the underlying mechanisms of NK-cell dysfunction remain unclear. METHODS This study involved 14 HL patients and SNK10/KHYG-1 cell lines to assess NK-cell activation against
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Efficacy of preexposure prophylaxis with monoclonal-antibody tixagevimab-cilgavimab against emerging SARS-CoV-2 resistant variants in patients with chronic lymphocytic leukemia. Acta Haematol. (IF 1.7) Pub Date : 2024-03-12 Ohad Benjamini,Tamar Tadmor,Abraham Avigdor,Rotem Gershon,Limor Kliker,Florin Fares,Nofar Atari,Ilana Laevsky,Bayan Abd Elkader,Tammy Hod,Orit Golan-Shany,Michal Mandelboim,Galia Rahav
Introduction Pre exposure prophylaxis with monoclonal antibodies (mAbs) were developed in addition to COVID19 vaccine for immunocompromised and those with insufficient immune response, among them patients with CLL. Omicron variant and its sublineages evolved mutations that escape mAbs neutralizing effect, yet the extent of which was not studied. Methods We evaluated anti-spike titters and neutralization
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The effect of mind-body intervention on lymphocyte doubling time and treatment free survival in treatment-naïve chronic lymphocytic leukemia patients. Acta Haematol. (IF 1.7) Pub Date : 2024-03-08 Shirley Shapira,Naama Hirschberger,Yishai Ofran,Barak Mizrahi,Esti Mandel,Ohad Benjamini,Noa Rabinowicz,Adi Zoref-Lorenz
INTRODUCTION Mind-Body Intervention (MBI) serves as supportive aid in oncology. We hypothesized that MBI could impact the progression of Chronic Lymphocytic Leukemia (CLL) in the 'watch and wait' (w&w) phase. METHODS MBI was utilized in a non-randomized prospective controlled study between 02/2020-02/2022 in 76 treatment-naïve CLL patients in the w&w phase (37 intervention and 39 control patients)
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Peeling Back the Layers: Recurrent Talquetamab Skin Toxicity After Supportive Stem Cell Boost in Multiple Myeloma. Acta Haematol. (IF 1.7) Pub Date : 2024-03-06 Alexander D Heini,Vera Ulrike Bacher,Dilara Akhoundova,Katja Seipel,Thomas Pabst
Bispecific antibodies have meaningfully expanded the therapeutic armentarium in multiple myeloma. Talquetamab is a CD3+ T cell redirecting antibody targeting GPRC5D, which is expressed on multiple myeloma plasma cells as well as in keratinized tissues. Due to the expression pattern, toxicity of talquetamab involves skin toxicity. Here we report the case of a patient who was treated with talquetamab
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Primary nodal Epstein-Barr virus-positive T-cell/NK-cell lymphoma: Real-world experience. Acta Haematol. (IF 1.7) Pub Date : 2024-03-03 Dae-Ho Choi,Sang Eun Yoon,Junhun Cho,Seok Jin Kim,Won Seog Kim
PTCL-EBV is a disease entity newly recognized in the WHO-HAEMS5 and the ICC of Mature Lymphoid neoplasms classification. Previously, it was classified as a subtype within PTCL-NOS and was known to have a poor prognosis. However, the clinical feature and treatment outcomes are not well known. This retrospective observational study was conducted on patients diagnosed with PTCL-EBV at Samsung Medical
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Venous thromboembolism prophylaxis in inflammatory bowel disease inpatients - Systematic review and meta-analysis. Acta Haematol. (IF 1.7) Pub Date : 2024-03-01 Rotem McNeil,Danielle Fredman,Ofir Eldar,Anat Gafter-Gvili,Tomer Avni
INTRODUCTION Inflammatory bowel disease (IBD) patients are three-times more likely to develop venous thromboembolism (VTE), and guidelines recommend prophylaxis during all hospitalizations. In this systematic review we sought to assess for the benefits and risks of VTE prophylaxis in hospitalized IBD patients. METHODS We performed a systematic review and meta-analysis. We searched MEDLINE and others
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Impacts of Posttransplant Cyclophosphamide Dose on Graft-versus-leukemia Effects via HLA-B Leader in HLA-haploidentical Peripheral Blood Stem Cell Transplantation. Acta Haematol. (IF 1.7) Pub Date : 2024-03-01 Makoto Moriguchi,Kentaro Ido,Hiroshi Okamura,Mika Nakamae,Kazuki Sakatoku,Yosuke Makuuchi,Masatomo Kuno,Teruhito Takakuwa,Asao Hirose,Mitsutaka Nishimoto,Yasuhiro Nakashima,Hideo Koh,Masayuki Hino,Hirohisa Nakamae
INTRODUCTION The graft-versus-leukemia effect of HLA-B leader dimorphism, i.e. methionine (M) or threonine (T) at position -21 of the leader sequence, has been observed in HLA-haploidentical hematopoietic cell transplantation with posttransplant cyclophosphamide (PTCy-haplo). However, the biological mechanism has been unclear, and the contributions of HLA-B leader genotype to risk reduction of relapse
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Impact of COVID-19 on patients with beta-thalassemia major: an observational study. Acta Haematol. (IF 1.7) Pub Date : 2024-02-28 Nadia Vacca,Cristian Locci,Fabiola Serra,Elena Chicconi,Mariangela V Puci,Giovanni Sotgiu,Antonio Matteo Amadu,Roberto Antonucci
INTRODUCTION The prevalence of COVID-19 is slightly lower, and its mortality is higher in beta-thalassemia patients than in the general population. We evaluated the impact of COVID-19 in terms of incidence, clinical course, management, and specific antibody response to vaccination in a cohort of patients with beta-thalassemia major. METHODS We retrospectively enrolled all transfusion-dependent beta-thalassemia
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Oncogenic MTOR signaling axis compensates BTK inhibition in a Chronic Lymphocytic Leukemia patient with Richter Transformation: A Case Report and Review of the Literature. Acta Haematol. (IF 1.7) Pub Date : 2024-02-24 Thomas Parigger,Stephan Drothler,Christian Scherhäufl,Franz Josef Gassner,Maria Schubert,Markus Steiner,Jan Philip Höpner,Alexandra Hödlmoser,Lena Schultheis,Aryunni Abu Bakar,Daniel Neureiter,Lisa Pleyer,Alexander Egle,Richard Greil,Roland Geisberger,Nadja Zaborsky
INTRODUCTION Targeting the B cell receptor (BCR) pathway via ibrutinib, a specific inhibitor of Bruton's tyrosine kinase, has shown marked clinical efficacy in treatment of patients with chronic lymphocytic leukemia (CLL), thus becoming a preferred first line option independent of risk factors. However, acquired resistance to ibrutinib poses a major clinical problem and requires the development of
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Evaluating the economic burden of acute myeloid leukemia in Canada. Acta Haematol. (IF 1.7) Pub Date : 2024-02-21 Jean Lachaine,Catherine Beauchemin,Fatéma Dodat,Yunghan Au,William K Evans,Brian Leber,Kristjan Paulson,Andre Schuh,John Storring
Background Acute myeloid leukemia (AML) represents a significant burden for patients and their families, and to the healthcare system. This study estimated the total cost of illness associated with newly diagnosed AML patients in Canada. Methods The economic burden of AML was estimated using an incidence-based model, analyzing different types of AML cases in Canada. Direct and indirect costs were calculated
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Congenital Hypodysfibrinogenemia due to γ326Cys→Tyr Mutation: Third Ever-Described Case Associated with Recurrent Venous Thrombosis and COVID Vaccine. Acta Haematol. (IF 1.7) Pub Date : 2024-02-17 Kumanan Rune Nanthan,Inge Søkilde Pedersen,Dorte Terp Andersen,Mustafa Vakur Bor
INTRODUCTION Congenital fibrinogen disorders are a heterogenous group of fibrinogen defects. CASE PRESENTATION Here, we describe hypodysfibrinogenemia in a 33-year-old female patient with provoked recurrent deep vein thrombosis (DVT) diagnosed based on decreased functional and antigenic fibrinogen levels with a decreased functional/antigenic fibrinogen ratio. Definitive diagnosis of congenital hypodysfibrinogenemia
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Clinical outcomes, survival, and predictors in lower-risk myelodysplastic syndrome patients treated with cyclosporine A. Acta Haematol. (IF 1.7) Pub Date : 2024-02-17 Yingjia Lu,Lina Zhang,Weiying Qu,Zhou Feng,Yuan Deng,Lin Zhao
INTRODUCTION Therapeutic options to improve myelodysplastic syndrome (MDS)-related cytopenias in patients with lower-risk MDS are limited, and cyclosporin A (CSA) is an available option. METHODS We retrospectively analysed the clinical data of 153 consecutive patients with lower-risk MDS at our institution from July 1997 to October 2017. Propensity score matching method was used to balance the influence
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Acquired von Willebrand syndrome associated with a smoldering multiple myeloma, successfully treated by daratumumab, lenalidomide and dexamethasone. Acta Haematol. (IF 1.7) Pub Date : 2024-02-15 Michael Iarossi,Marie-Christiane Madeleine Vekemans,Nicolas Weynants,Cedric Hermans
INTRODUCTION Acquired von Willebrand syndrome (AvWS) is a rare entity with approximately 700 cases described in the literature. A number of etiologies are responsible for this condition, mainly lymphoproliferative, myeloproliferative syndromes and cardiac diseases. Management is aimed at preventing and treating bleeds, as well as treating the underlying pathology. In the case of a monoclonal gammopathy
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Persistent COVID-19: a case report of an immunocompromised patient and a literature review. Acta Haematol. (IF 1.7) Pub Date : 2024-02-15 Sirine Bekkaoui,Geoffroy Venton,Fannie Bretelle,Victoria Garrido,Victor Chabbert,Stéphane Gayet,Paul Dalmas,Antoine Tichadou,Pierre-André Jarrot,Patrick Villani,Aurélie Daumas,Robin Arcani
Introduction Immunocompromised patients can show prolonged shedding of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and persistent symptoms, which is called persistent COVID-19. Case presentation We report a case of an immunocompromised patient who was treated for mantle cell lymphoma and was suffering from B-cell depletion. The patient developed persistent COVID-19, which was confirmed
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Lenalidomide treatment of isolated central nervous system relapse in acute lymphoblastic leukemia after HSCT and CAR-T cell therapy. Acta Haematol. (IF 1.7) Pub Date : 2024-02-15 Qing Zhang,Yi Dong,Zhimin Zhai,Lili Tao,Qianshan Tao
INTRODUCTION Hematopoietic stem cell transplantation (HSCT) and chimeric antigen receptor T (CAR-T) cell are effective treatments for acute lymphoblastic leukemia (ALL). Various forms of intra- and extra-medullary relapses have been reported after HSCT and CAR-T cell therapy for ALL; however, no reports have investigated isolated central nervous system (CNS) relapse after HSCT and CAR-T cell therapy
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Prevalence, risk factors, and prognostic value of anxiety and depression in acute myeloid leukemia. Acta Haematol. (IF 1.7) Pub Date : 2024-02-11 Tao Zhong,Dan Xu,Wenchao Li
INTRODUCTION Limited studies report anxiety and depression prevalence and their correlations with prognosis in acute myeloid leukemia (AML). Even worse, their risk factors in AML remained unclear. This study aimed to investigate the prevalence, risk factors, and prognostic value of anxiety and depression in AML patients. METHODS Totally, 132 de novo AML patients, 60 non-malignant hematological disease
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Multiplex proteomics in the identification of potential biomarkers of very severe sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) in allogeneic hematopoietic cell transplant patients treated with defibrotide. Acta Haematol. (IF 1.7) Pub Date : 2024-02-08 Ram Vasudevan Nampoothiri,Lisa Avery,Ivan Pasic,Ioannis Prassas,Eleftherios Diamandis,Fotios V Michelis
Introduction Despite well-established clinical criteria for diagnosis of SOS/VOD following allogeneic HCT, there is a lack of established diagnostic protein biomarkers. Methods Prospective samples were collected from patients with very severe SOS/VOD at diagnosis and days +3, +7, +14, and +30 post-initiation of defibrotide. Samples from age-matched controls with no VOD were collected at day +14, +30
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Attitudes towards COVID-19 vaccination in adults with haematological malignancies. Acta Haematol. (IF 1.7) Pub Date : 2024-01-30 Richard Blennerhassett,Nada Hamad,Lisa Grech,Alastair Kwok,Tammie Choi,Cecily Forsyth,Jacqueline Jagger,Stephen Opat,Sam Harris,Bryan Anthony Chan,Mike Nguyen,Nathan Bain,Daphne Day,Eva Segelov,
INTRODUCTION Despite people with haematological malignancies being particularly vulnerable to severe COVID-19 infection and complications, vaccine hesitancy may be a barrier to optimal vaccination. This study explored attitudes towards COVID-19 vaccination in people with haematological malignancies. METHODS People with haematological malignancies at nine Australian health services were surveyed between
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Prognostic Factors of Adult Hemophagocytic Lymphohistiocytosis and Clinical Utility of HLH-2004 Diagnostic Criteria and HScore: A Real-World Multicenter Study from Thailand. Acta Haematol. (IF 1.7) Pub Date : 2024-01-23 Pitchayaporn Jongdee,Jakrawadee Julamanee,Ekarat Rattarittamrong,Sarita Mukura,Chinadol Wanitpongpun,Rawisut Deoisares,Anoree Surawong,Thunyamon Chajuwan,Chantiya Chanswangphuwana
INTRODUCTION Adult hemophagocytic lymphohistiocytosis (HLH) is a rare disease with a dismal prognosis. Early diagnosis and prompt management are necessary for improved outcomes. METHODS This multicenter retrospective study investigated the etiologies, survival, and prognostic factors of HLH, including the utility of HLH-2004 criteria and HScore in real-life clinical practice. RESULTS A total of 147
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Outcome and prognostic factors of haploidentical allogeneic hematopoietic stem cell transplantation in pediatric relapsed or refractory ETV6/RUNX1-positive acute lymphoblastic leukemia. Acta Haematol. (IF 1.7) Pub Date : 2024-01-20 Guan-Hua Hu,Xiao-Hui Zhang,Kai-Yan Liu,Lan-Ping Xu,Yu Wang,Yi-Fei Cheng,Xiao-Jun Huang
INTRODUCTION the role of haploidentical allogeneic hematopoietic stem cell transplantation (haplo-HSCT) in pediatric patients with relapsed or refractory (R/R) ETV6/RUNX1-positive acute lymphoblastic leukemia (ALL) is unclear. This study aimed to identify prognostic factors and explore the role of haplo-HSCT in the treatment of ETV6/RUNX1-positive ALL. METHODS we analyzed the clinical characteristics
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Efficiency and Toxicity of Imatinib Mesylate combined with Atorvastatin Calcium in the Treatment of Steroid-Refractory Chronic Graft-Versus-Host Disease: A Single-center, Prospective, Single-arm, Open-label study. Acta Haematol. (IF 1.7) Pub Date : 2024-01-17 Ting Chen,JiaLi Li,Xiao Wei,Han Yao,LiDan Zhu,Jia Liu,YuQing Liu,Ping Wang,YiMei Feng,ShiChun Gao,HuanFeng Liu,Lu Wang,Lu Zhao,Li Gao,Cheng Zhang,Lei Gao,Xi Zhang,PeiYan Kong
INTRODUCTION Steroid-refractory cGVHD (SR-cGVHD) presents new great challenges for treatment. We have reported imatinib monotherapy was effective to SR-cGVHD, but the CR rate was not satisfactory and the benefit was not showed specific to some target organs, previously. Imatinib and statin drugs have been recognized to regulate T-cell function, statins also have been demonstrated endothelia protection
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Clinical features of hepatic manifestations among adult patients with hemophagocytic lymphohistiocytosis: a retrospective study. Acta Haematol. (IF 1.7) Pub Date : 2024-01-16 Qiongling Bao,Zhengqing Xu,Fengling Yang,Juan Lu
INTRODUCTION Liver dysfunction is common in patients with hemophagocytic lymphohistiocytosis (HLH). However, whether the severity of liver injury is associated with the prognosis of patients with HLH remains to be determined. This study aims to assess the association of the severity of liver involvement with short-term prognosis among adult patients with HLH. METHODS A retrospective study was performed
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Successful treatment with daratumumab of a patient with monoclonal lambda light chain disease presenting as nephrotic syndrome and crescentic glomerulonephritis. Acta Haematol. (IF 1.7) Pub Date : 2024-01-16 Alon Bnaya,Chezi Ganzel,Linda Shavit
Monoclonal immunoglobulin deposition disease (MIDD) are a group of systemic diseases, characterized by deposition of monoclonal immunoglobulin predominantly in the kidney. In the absence of overt hematologic disease, MIDD are classified as a part of monoclonal gammopathy of renal significance. Patients with MIDD may present with a nephrotic syndrome and kidney function impairment. Treatment usually
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Health-Related Complications during Follow-Up and Their Impact on Blood Cancer Survivors: Results from the "Aftercare in Blood Cancer Survivors" (ABC) Study. Acta Haematol. (IF 1.7) Pub Date : 2024-01-16 Julia Baum,Hildegard Lax,Nils Lehmann,Anja Merkel-Jens,Dietrich W Beelen,Karl-Heinz Jöckel,Ulrich Dührsen
INTRODUCTION Blood cancer survivors are at increased risk for medical complications. METHODS Our questionnaire-based study involved 1,551 blood cancer survivors with a ≥3-year interval since the last intense treatment. Its goal was to quantify health-related complications during follow-up and assess their impact on the patients' lives. RESULTS A total of 20.4% of the responding survivors reported a