Hemoglobin Beth Israel [HBB:c.308A & gt;G (p.Asn103Ser)]: an ultra-rare, low oxygen-affinity, non-methemoglobinemic hemoglobin diagnosed on targeted resequencing as cause of dominantly inherited benign cyanosis
(Source: Annals of Hematology)
Source: Annals of Hematology - October 1, 2022 Category: Hematology Source Type: research

Anti-CD38 antibody therapy for patients with relapsed/refractory multiple myeloma: differential mechanisms of action and recent clinical trial outcomes
AbstractCD38 is a transmembrane glycoprotein that functions both as a receptor and an ectoenzyme, playing key roles in the regulation of calcium signaling and migration of immune cells to tumor microenvironments. High expression on multiple myeloma (MM) cells and limited expression on normal cells makes CD38 an ideal target for the treatment of MM patients. Two monoclonal antibodies directed at CD38, isatuximab and daratumumab, are available for use in patients with relapsed and/or refractory MM (RRMM); daratumumab is also approved in newly diagnosed MM and light-chain amyloidosis. Clinical experience has shown that anti-C...
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Bosutinib-induced late-onset severe liver injury preceded by eosinophilia
AbstractTyrosine kinase inhibitors (TKIs) have dramatically changed the way chronic myeloid leukemia (CML) is treated. Although TKI therapy improves the overall survival of patients with CML, the management of various adverse events (AEs) from the therapy has become a major barrier to its adherence. Bosutinib is a second-generation TKI and an effective first-line treatment for CML. The most frequent AE is diarrhea; however, liver toxicity is also a major complication. In general, drug-induced hepatic enzyme elevation occurs within 1 –2 weeks after treatment initiation. However, we noticed that, in a certain number of pa...
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

CD277 agonist enhances the immunogenicity of relapsed/refractory acute myeloid leukemia towards V δ2+ T cell cytotoxicity
AbstractRelapse and refractoriness remain the major obstacles in clinical treatment of acute myeloid leukemia (AML). Efficacy of current therapeutic strategies for relapsed/refractory (R/R) AML is generally unsatisfying. V δ2+ T cells have become an attractive candidate for immunotherapy of various types of tumors. However, the results were not exciting in some pilot studies utilizing V δ2 cell-based protocols to treat R/R AML. Functional receptors on Vδ2 cells and immunogenic ligands on leukemia cells are both critical to the anti-AML effect of Vδ2 cells, which have not been characterized in the context of R/R AML. CD...
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Rituximab plus cladribine versus R-CHOP in frontline management of marginal zone lymphoma in China: a propensity-score matched multicenter study
AbstractMarginal zone lymphoma (MZL) is an uncommon subtype of non-Hodgkin lymphoma (NHL). Combination of rituximab and cladribine (R-2CdA) is a potential option for indolent NHL (iNHL) and mantle cell lymphoma (MCL) patients. The goal of this multicenter retrospective study was to assess the efficacy and safety of R-2CdA in MZL to support consensus-reaching in first-line therapy in advanced-stage patients. We searched electronic medical records databases of eight centers in China. Between November 2014 and December 2019, 183 symptomatic advanced MZL patients (42 treated with R-2CdA and 141 with rituximab plus cyclophospha...
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Functional association of NR4A3 downregulation with impaired differentiation in myeloid leukemogenesis
AbstractThe coincident downregulation ofNR4A1 andNR4A3 has been implicated in myeloid leukemogenesis, but it remains unknown how these two genes function in myeloid cells and how their combined downregulation promotes myeloid leukemogenesis. SinceNR4A1 abrogation is thought to confer a survival and proliferation advantage to myeloid cells, we hypothesized that downregulation ofNR4A3 may have a complementary effect on myeloid cell differentiation. First, we tested the association between differentiation status of leukemic cells andNR4A3 expression using two large clinical datasets from patients with different acute myeloid ...
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Cut-off values for diagnosis of G6PD deficiency by flow cytometry in Thai population
This study established flow cytometric cut-off values for screening of G6PD deficiency in hemizygous males and heterozygous or homozygous females. We studied 205 (125 females, 80 males) leftover blood samples from quantitative methemoglobin reduction (MR) screening. G6PD gene mutations determined by multiplex amplification refractory mutation system-polymerase chain reaction and direct DNA sequencing were used as the gold standard reference. Accuracy of the test, including the sensitivity, specificity, and positive and negative predictive values, was analyzed using MedCalc software. The optimal cut-off values for classific...
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Low-risk polycythemia vera treated with phlebotomies: clinical characteristics, hematologic control and complications in 453 patients from the Spanish Registry of Polycythemia Vera
AbstractHematological control, incidence of complications, and need for cytoreduction were studied in 453 patients with low-risk polycythemia vera (PV) treated with phlebotomies alone. Median hematocrit value decreased from 54% at diagnosis to 45% at 12  months, and adequate hematocrit control over time (<  45%) was observed in 36%, 44%, and 32% of the patients at 6, 12, and 24 months, respectively. More than 5 phlebotomies per year in the maintenance phase were required in 19% of patients. Worsening thrombocytosis, age >  60 years, and microvascular symptoms constituted the main indications for starting cyt...
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Real-life analysis on safety and efficacy of asciminib for ponatinib pretreated patients with chronic myeloid leukemia
AbstractFailure of second-generation tyrosine kinase inhibitors (2GTKI) is a challenging situation in patients with chronic myeloid leukemia (CML). Asciminib, recently approved by the US Federal Drug Administration, has demonstrated in clinical trials a good efficacy and safety profile after failure of 2GTKI. However, no study has specifically addressed response rates to asciminib in ponatinib pretreated patients (PPT). Here, we present data on responses to asciminib from 52 patients in clinical practice, 20 of them (38%) with prior ponatinib exposure. We analyzed retrospectively responses and toxicities under asciminib an...
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Rapid and deep response to avapritinib in heavily treated acute myeloid leukemia with t (8;21) and KIT mutation
(Source: Annals of Hematology)
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Hemophagocytic lymphohistiocytosis in HIV-associated lymphoproliferative disorders
In conclusion HLH appears to be more frequent in HIV-associated lymphomas than in HIV-negative lymphomas. The probability of developing HLH was particularly hi gh in patients with Hodgkin lymphoma, lymphoma with bone marrow infiltration and HHV-8 associated lymphoma. Mortality was significantly increased in the presence of HLH. (Source: Annals of Hematology)
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Effective treatment with polatuzumab vedotin of relapsed and refractory primary cutaneous diffuse large B-cell lymphoma leg type
(Source: Annals of Hematology)
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

First Latin America report on the diagnostic utility of the study of the MYD88 L265P gene mutation in patients with Waldenstr öm Macroglobulinemia
(Source: Annals of Hematology)
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Forodesine maintenance therapy for newly diagnosed peripheral T-cell lymphoma: a single-institutional, observational, retrospective analysis
(Source: Annals of Hematology)
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research

Whole exome sequencing identifies a novel SPTB frameshift mutation causing hereditary spherocytosis in the Chinese population
(Source: Annals of Hematology)
Source: Annals of Hematology - September 13, 2022 Category: Hematology Source Type: research