Post-Transplant Cyclophosphamide Combined with Anti-Thymocyte Globulin as Graft-versus-Host Disease Prophylaxis for Allogeneic Hematopoietic Cell Transplantation in High-Risk Acute Myeloid Leukemia and Myelodysplastic Syndrome.
CONCLUSIONS: We conclude that the ATG + PTCy combination significantly improved GRFS in allogeneic HCT for high-risk AML and MDS without influencing other outcomes. PMID: 32428903 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - May 19, 2020 Category: Hematology Authors: Alanazi W, Chen S, Lipton JH, Kim DD, Viswabandya A, Kumar R, Lam W, Law AD, Al-Shaibani Z, Mattsson J, Michelis FV Tags: Acta Haematol Source Type: research

Cardiac Care of Patients with Cardiac Amyloidosis.
Abstract Cardiac amyloidosis, the majority of cases of which are due to immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), affects different aspects of the heart and cardiovascular system. Amyloid-induced cardiomyopathy, clinically manifesting with heart failure and electrophysiological abnormalities, has distinct characteristics compared to non-amyloid cardiomyopathies. Accordingly, specific management strategies are required. This paper will review the cardiovascular manifestations of patients with cardiac amyloidosis and their suggested treatment strategies, emphasizing the import...
Source: Acta Haematologica - May 14, 2020 Category: Hematology Authors: Itzhaki Ben Zadok O, Kornowski R Tags: Acta Haematol Source Type: research

The Association between Metabolic Syndrome and Multiple Myeloma.
Abstract Multiple myeloma (MM) is a haematological malignancy arising from monoclonal proliferation of plasma cells in the bone marrow, resulting in the presence of paraproteins or M-protein in serum. The involvement of paraproteins produced by malignant plasma cells in the development of hyperlipidaemia and low-HDL cholesterol has been described, as has an association with MM and obesity, hypertension, and type 2 diabetes mellitus, and insulin resistance, that is, features of the metabolic syndrome (MS). There is an association between MS components, inflammatory cytokines, and the development of MM, and some dru...
Source: Acta Haematologica - May 14, 2020 Category: Hematology Authors: Ragbourne SC, Maghsoodi N, Streetly M, Crook MA Tags: Acta Haematol Source Type: research

Chronic Pain Does Not Impact Baseline Circulating Cytokine Levels in Adults with Sickle Cell Disease.
Abstract Chronic pain affects 50% of adults with sickle cell disease (SCD). Although inflammation is thought to contribute to the pathogenesis of chronic pain, no studies have examined the differences in circulating cytokines between patients with SCD with and without chronic pain. We performed an observational cohort study using blood and urine samples from adults with SCD with and without chronic pain at their usual state of health. We tested the hypothesis that, compared to those without chronic pain, those with chronic pain would have significantly higher baseline circulating proinflammatory cytokines. A total...
Source: Acta Haematologica - May 13, 2020 Category: Hematology Authors: Karafin MS, Simpson P, Field JJ Tags: Acta Haematol Source Type: research

Venous Thromboembolism Complicated with COVID-19: What Do We Know So Far?
Abstract Coronavirus disease (COVID-19) is caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and is responsible for the ongoing 2019-2020 pandemic. Venous thromboembolism (VTE), a frequent cardiovascular and/or respiratory complication among hospitalized patients, is one of the known sequelae of the illness. Hospitalized COVID-19 patients are often elderly, immobile, and show signs of coagulopathy. Therefore, it is reasonable to assume a high incidence of VTE among these patients. Presently, the incidence of VTE is estimated at around 25% of patients hospitalized in the intensive car...
Source: Acta Haematologica - May 12, 2020 Category: Hematology Authors: Tal S, Spectre G, Kornowski R, Perl L Tags: Acta Haematol Source Type: research

Peculiar Congenital Factor VII Defect with the Proposita and Her Mother Showing the Same Compound Heterozygosity for Thr384Met and Arg413Gln.
CONCLUSIONS: The identical defect which presented in the propositaand her mother could be explained by the genetic analysis of the father and maternal grandfather of the proposita who happened to have the same mutation (Arg413Gln). PMID: 32396910 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - May 12, 2020 Category: Hematology Authors: Girolami A, Paoletti M, Ferrari S, Garcia D Tags: Acta Haematol Source Type: research

The Pathology of Amyloidosis in Classification: A Review.
Abstract BACKGROUND: The amyloidoses are a rare and heterogeneous group of disorders that are characterized by the deposition of abnormally folded proteins in tissues ultimately leading to organ damage. The deposits are mainly extracellular and are recognizable by their affinity for Congo red and their yellow-green birefringence under polarized light. Current classification of amyloid in medical practice is based on the amyloid protein type. To date, 36 proteins have been identified as being amyloidogenic in humans. SUMMARY: in clinical practice, it is critical to distinguish between treatable versus non-trea...
Source: Acta Haematologica - May 11, 2020 Category: Hematology Authors: Picken MM Tags: Acta Haematol Source Type: research

Elderly Non-GCB Diffuse Large B-Cell Lymphoma Patient Responding to Lenalidomide after Epicardial Relapse: A Case Report.
Abstract There is an unmet clinical need for elderly or unfit diffuse large B-cell lymphoma (DLBCL) patients ineligible for autologous stem cell transplantation. Lenalidomide is an immunomodulatory agent with antitumor activity in non-Hodgkin lymphoma, with an acceptable toxicity profile and manageable side effects. A 79-year-old Caucasian male with non-germinal center B-cell-like DLBCL achieved complete remission (CR) after first-line treatment with seven out of eight scheduled cycles of a polychemotherapy containing anthracycline, which had to be discontinued early due to the onset of atrial fibrillation. After ...
Source: Acta Haematologica - May 11, 2020 Category: Hematology Authors: Gentilini M, Casadei B, Broccoli A, Argnani L, Cavo M, Zinzani PL Tags: Acta Haematol Source Type: research

Treating Leukemia in the Time of COVID-19.
Abstract The coronavirus disease 2019 (COVID-19) pandemic poses several challenges to the management of patients with leukemia. The biology of each leukemia and its corresponding treatment with conventional intensive chemotherapy, with or without targeted therapies (venetoclax, FLT3 inhibitors, IDH1/2 inhibitors, Bruton's tyrosine kinase inhibitors), introduce additional layers of complexity during COVID-19 high-risk periods. The knowledge about COVID-19 is accumulating rapidly. An important distinction is the prevalence of "exposure" versus "clinical infectivity," which determine the risk vers...
Source: Acta Haematologica - May 11, 2020 Category: Hematology Authors: Paul S, Rausch CR, Jain N, Kadia T, Ravandi F, DiNardo CD, Welch MA, Dabaja BS, Daver N, Garcia-Manero G, Wierda W, Pemmaraju N, Montalban Bravo G, Thompson P, Verstovsek S, Konopleva M, Kantarjian H, Jabbour E Tags: Acta Haematol Source Type: research

T-Cell Prolymphocytic Leukemia: Long-Term Remissions Challenging!
PMID: 32392564 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - May 11, 2020 Category: Hematology Authors: Sellner L Tags: Acta Haematol Source Type: research

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Acta Haematol. 2020 May 11;143(3):189 Authors: PMID: 32392568 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - May 11, 2020 Category: Hematology Tags: Acta Haematol Source Type: research

Perspective: SARS-CoV-2, COVID-19 and Haematologists.
PMID: 32375142 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - May 6, 2020 Category: Hematology Authors: Gale RP Tags: Acta Haematol Source Type: research

What to Look Out for when Transplanting T-Cell Prolymphocytic Leukemia.
PMID: 32369824 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - May 5, 2020 Category: Hematology Authors: Wahnschaffe L, Herling M Tags: Acta Haematol Source Type: research

Conventional Therapy for Amyloid Light-Chain Amyloidosis.
Abstract The vast majority of patients with light-chain (AL) amyloidosis are not eligible for stem cell transplant and are treated with conventional chemotherapy. Conventional regimens are based on various combinations of dexamethasone, alkylating agents, proteasome inhibitors, and immunomodulatory drugs. The choice of these regimens requires a careful risk stratification, based on the extent of amyloid organ involvement, comorbidities, and the characteristics of the amyloidogenic plasma cell clone. Most patients are treated upfront with bortezomib and dexamethasone combined with cyclophosphamide or melphalan. Cyc...
Source: Acta Haematologica - April 30, 2020 Category: Hematology Authors: Milani P, Palladini G Tags: Acta Haematol Source Type: research

Belinostat in Relapsed/Refractory T-Cell Large Granular Lymphocyte Leukemia.
Abstract T-cell large granular lymphocyte (LGL) leukemia is a rare indolent neoplasm primarily treated with immunosuppression. Few therapies and no consensus exist for the optimal treatment of T-cell LGL leukemia refractory to immunosuppressive therapy. Here, we report a case of relapsed/refractory T-cell LGL treated with belinostat. A 57-year-old male presented with lymphocytosis and anemia and was found to have T-cell LGL, requiring frequent packed red blood cell transfusions. He was initially treated with methotrexate with no response after 7 months. He was then switched to cyclosporine and cyclophosphamide and...
Source: Acta Haematologica - April 29, 2020 Category: Hematology Authors: Poh C, Arora M, Ghuman S, Tuscano J Tags: Acta Haematol Source Type: research

When to Suspect a Diagnosis of Amyloidosis.
Abstract Amyloidosis is a group of complex diseases caused by extracellular deposition of pathological insoluble fibrillary protein in organs and tissues and may result in severe organ dysfunction. Despite the etiological heterogeneity of systemic amyloidosis, the clinical manifestations of the different forms of amyloidosis largely overlap and depend upon the effected organ. The signs and symptoms that should raise suspicion for the potential diagnosis of amyloidosis are usually nonspecific; therefore, establishing the diagnosis is difficult, and early diagnosis requires clinical suspicion. Light chain (AL) amylo...
Source: Acta Haematologica - April 27, 2020 Category: Hematology Authors: Vaxman I, Gertz M Tags: Acta Haematol Source Type: research

A Prospective Pilot Study of Graft-versus-Host Disease Prophylaxis with Post-Transplantation Cyclophosphamide and Ruxolitinib in Patients with Myelofibrosis.
CONCLUSION: GVHD prophylaxis with PTCy and ruxolitinib is associated with low toxicity, good acute and chronic GVHD control, and low relapse incidence. However, the relatively high rate of SPGF should be taken into account. SPGF could possibly be mitigated by ruxolitinib dose reduction. PMID: 32325461 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - April 23, 2020 Category: Hematology Authors: Morozova EV, Barabanshikova MV, Moiseev IS, Shakirova AI, Barhatov IM, Ushal IE, Rodionov GG, Moiseev SI, Surkova EA, Lapin SV, Vlasova JJ, Rudakova TA, Darskaya EI, Baykov VV, Alyanski AL, Bondarenko SN, Afanasyev BV Tags: Acta Haematol Source Type: research

Multiple Myeloma in the Time of COVID-19.
Abstract We provide our recommendations (not evidence based) for managing multiple myeloma patients during the pandemic of COVID-19. We do not recommend therapy for smoldering myeloma patients (standard or high risk). Screening for COVID-19 should be done in all patients before therapy. For standard-risk patients, we recommend the following: ixazomib, lenalidomide, and dexamethasone (IRd) (preferred), cyclophosphamide lenalidomide and dexamethasone (CRd), daratumumab lenalidomide and dexamethasone (DRd), lenalidomide, bortezomib, and dexamethasone (RVd), or cyclophosphamide, bortezomib, and dexamethasone (CyBorD)....
Source: Acta Haematologica - April 17, 2020 Category: Hematology Authors: Al Saleh AS, Sher T, Gertz MA Tags: Acta Haematol Source Type: research

Integrative Analysis of Long Noncoding RNAs in Patients with Graft-versus-Host Disease.
CONCLUSIONS: This is the first study on the correlation between lncRNA and cGVHD using lncRNA microarray analysis. Our study provides novel enlightenment in exploring the molecular pathogenesis of cGVHD. PMID: 32289782 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - April 14, 2020 Category: Hematology Authors: Wang F, Luo L, Gu Z, Yang N, Wang L, Gao C Tags: Acta Haematol Source Type: research

Genetic Analysis of Afibrinogenemia and Hypofibrinogenemia: Novel Mutations in the FGB Gene in the Turkish Population.
r N Abstract INTRODUCTION: Congenital afibrinogenemia is a rare autosomal recessive disorder characterized by bleeding that varies from mild to severe and by complete absence or extremely low levels of plasma and platelet fibrinogen. Hypofibrinogenemia is characterized by fibrinogen levels
Source: Acta Haematologica - April 14, 2020 Category: Hematology Authors: Özkan DT, Sarper N, Akar N Tags: Acta Haematol Source Type: research

Venetoclax and BCR-ABL Tyrosine Kinase Inhibitor Combinations: Outcome in Patients with Philadelphia Chromosome-Positive Advanced Myeloid Leukemias.
CONCLUSIONS: Combination therapy of venetoclax with TKI-based regimens shows encouraging activity in very heavily pretreated, advanced Ph+ leukemias, particularly CML-MBP. PMID: 32289808 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - April 14, 2020 Category: Hematology Authors: Maiti A, Franquiz MJ, Ravandi F, Cortes JE, Jabbour EJ, Sasaki K, Marx K, Daver NG, Kadia TM, Konopleva MY, Masarova L, Borthakur G, DiNardo CD, Naqvi K, Pierce S, Kantarjian HM, Short NJ Tags: Acta Haematol Source Type: research

Patterns of Late Relapse after Allogeneic Hematopoietic Stem Cell Transplantation in Patients with T-Cell Prolymphocytic Leukemia.
Abstract Initial treatment with the monoclonal anti-CD52 antibody alemtuzumab induces responses in the majority of patients with T-cell prolymphocytic leukemia (T-PLL). In eligible patients, allogeneic hematopoietic stem cell transplantation (allo-HSCT) is an option to consolidate hematological remissions. Here, we report our experience with 10 patients who received allo-HSCT against T-PLL. Notably, 3 patients with complete remission at transplantation and durable full-donor chimerism relapsed at months 12, 59, and 84 after transplantation, respectively. This relapse was associated with rapid progressive leukemia ...
Source: Acta Haematologica - April 7, 2020 Category: Hematology Authors: Shumilov E, Hasenkamp J, Szuszies CJ, Koch R, Wulf GG Tags: Acta Haematol Source Type: research

High-Dose Melphalan and Autologous Peripheral Blood Stem Cell Transplantation in AL Amyloidosis.
Abstract AL amyloidosis is a systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. High-dose intravenous melphalan and autologous stem cell transplantation was developed for the treatment of AL amyloidosis in the early 1990s and was prompted by its success in myeloma. This application has evolved significantly over the past three decades. This review provides a comprehensive assessment of eligibility criteria, stem cell collection, and mobilization strategies and regimens, risk-adapted melphalan dosing, role for induction and consolidation therapies as well as long-term outcome with resp...
Source: Acta Haematologica - April 3, 2020 Category: Hematology Authors: Sanchorawala V Tags: Acta Haematol Source Type: research

Supportive Care in AL Amyloidosis.
N, Bladé J Abstract Immunoglobulin light-chain (AL) amyloidosis is a systemic disease characterized by the production and deposition of light chain-derived amyloid fibrils in different organs. Prompt treatment directed to the underlying plasma cell clone is crucial in order to achieve a rapid, deep and durable hematologic response. The decrease in the production of the amyloidogenic light chains is a required condition to obtain the organ response, which is commonly delayed. Meanwhile, supportive treatment is aimed to maintain quality of life of these patients and preserve their involved organs' function. F...
Source: Acta Haematologica - April 1, 2020 Category: Hematology Authors: Cibeira MT, Ortiz-Pérez JT, Quintana LF, Fernádez de Larrea C, Tovar N, Bladé J Tags: Acta Haematol Source Type: research

Identification of a Patient Cohort with Relapsing Diffuse Large B-Cell Lymphoma with a Low International Prognostic Index in PET/CT Using a 2-Gene (LMO2/TNFRSF9) Scoring System.
PMID: 32187599 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - March 18, 2020 Category: Hematology Authors: Omidvar N, Tekin N, Conget P, Bruna F, Timar B, Gagyi E, Basak R, Auewarakul C, Sritana N, Cerci JJ, Dimamay MP, Gyorke T, Redondo F, Nair R, Gorospe C, Paez D, Fanti S, Ozdag H, Padua RA, Carr R Tags: Acta Haematol Source Type: research

Desensitization in Iron Product Allergy.
Abstract Iron deficiency is the main cause of anemia in both sexes, with women being more commonly affected. Iron therapy is currently considered an effective and safe remedy to replenish the iron storages. Iron can be administrated both orally and intravenously. In particular, intravenous (IV) iron therapy is widely used when oral iron preparations are either not tolerated or ineffective. Indeed, IV iron improves iron stores more rapidly. Two main immunological responses have been described for iron hypersensitivity reactions (HSRs): IgE-mediated allergy and complement activation-related pseudo-allergy. Here, we ...
Source: Acta Haematologica - March 17, 2020 Category: Hematology Authors: Di Girolamo A, Albanesi M, Loconte F, Di Bona D, Caiaffa MF, Macchia L Tags: Acta Haematol Source Type: research

Clinical Benefit Derived from Decitabine Therapy for Advanced Phases of Myeloproliferative Neoplasms.
Abstract Treatment options are limited for patients with advanced forms of myeloproliferative neoplasms (MPN) including blast-phase disease (MPN-BP). Decitabine has frequently been deployed but its efficacy and safety profile are not well described in this population. We retrospectively reviewed 42 patients treated with decitabine either alone or in combination with ruxolitinib at our institution: 16 with MPN-BP, 14 with MPN accelerated-phase (MPN-AP), and 12 with myelofibrosis with high-risk features (MF-HR). The median overall survival (OS) for the MPN-BP patients was 2.6 months, and for those who received ≥2...
Source: Acta Haematologica - March 11, 2020 Category: Hematology Authors: Zhou S, Tremblay D, Hoffman R, Kremyanskaya M, Najfeld V, Li L, Moshier E, Mascarenhas J Tags: Acta Haematol Source Type: research

Noonan Syndrome and Acute Myeloid Leukemia in Adults: The Importance of a Correct Multidisciplinary Approach during Childhood.
PMID: 32155617 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - March 10, 2020 Category: Hematology Authors: Timeus F Tags: Acta Haematol Source Type: research

A Unique Case of Acquired Hemophilia A Presenting with Transient Ischemic Attack.
In this report, we describe the case of an elderly patient presenting a transient ischemic attack concurrently with AHA. A thrombotic event in AHA is occasionally associated with the use of bypassing agents for treatment, but a spontaneous thrombotic event has not ever been described. PMID: 32155618 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - March 10, 2020 Category: Hematology Authors: Beauverd Y, Boehlen F, Fisch L, Sztajzel R, Fontana P, Casini A Tags: Acta Haematol Source Type: research

Treating Plasma Cell Myeloma in Developing Countries: Does Everyone Need the Newest Drugs?
PMID: 32155631 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - March 10, 2020 Category: Hematology Authors: Gale RP Tags: Acta Haematol Source Type: research

The Prognostic Role of Lactate Dehydrogenase at First Relapse of Multiple Myeloma.
PMID: 32101854 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - February 26, 2020 Category: Hematology Authors: Lee H, Jimenez-Zepeda VH Tags: Acta Haematol Source Type: research

The Spectrum of MYC Alterations in Diffuse Large B-Cell Lymphoma.
In this study, we comprehensively summarize the different alterations of MYC in DLBCL, including MYC overexpression, MYC translocations, MYC mutations, and increased gene copy number of MYC. Noteworthy, lone MYC overexpression or MYC translocation is not significantly associated with poor clinical outcomes, and their detrimental effects depend on the genetic alterations of BCL2 or BCL6. Both double-expressor DLBCL (DE-DLBCL), defined as overexpression of MYC and BCL2 proteins, and double-hit lymphoma (DHL), defined as a dual translocation of MYC together with BCL2 or BCL6, represent the distinct subgroups of DLBCL with inf...
Source: Acta Haematologica - February 19, 2020 Category: Hematology Authors: Xia Y, Zhang X Tags: Acta Haematol Source Type: research

Tisagenlecleucel in Non-Hodgkin Lymphoma: The Restricted Mean Survival Time as a Tool for Estimating Progression-Free Life Expectancy Better than the Median.
PMID: 32074609 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - February 19, 2020 Category: Hematology Authors: Chiumente M, Mengato D, Messori A Tags: Acta Haematol Source Type: research

WhatsApp in Acta Haematologica?
PMID: 32050195 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - February 12, 2020 Category: Hematology Authors: Raanani P Tags: Acta Haematol Source Type: research

Treatment of Persons with Multiple Myeloma in Underprivileged Circumstances: Real-World Data from a Single Institution.
CONCLUSION: In this series, a regimen incorporating low-cost novel agents and outpatient HCT was associated with excellent long-term survival in the treatment of MM patients. This approach may be a model for MM treatment in underprivileged circumstances. PMID: 32045907 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - February 11, 2020 Category: Hematology Authors: Murrieta-Álvarez I, Steensma DP, Olivares-Gazca JC, Olivares-Gazca M, León-Peña A, Cantero-Fortiz Y, García-Navarrete YI, Cruz-Mora A, Ruiz-Argüelles A, Ruiz-Delgado GJ, Ruiz-Argüelles GJ Tags: Acta Haematol Source Type: research

Combined Congenital Hypodysfibrinogemia and Factor XI Deficiency in a Chinese Family.
Abstract Both congenital hypodysfibrinogenemia and factor XI deficiency are rare coagulopathies caused by mutations within the fibrinogen and F11 genes, respectively. To investigate the pathogenesis of combined congenital hypodysfibrinogenemia with factor XI (FXI) deficiency in a Chinese family, coagulation assays, FXI activity (the 1-stage method), fibrinogen activity (the Clauss method), and antigen (prothrombin time [PT]-derived method) were performed. The sequences of fibrinogen genes and F11 were amplified by PCR and analyzed by direct sequencing. The proband as well as his grandmother, father, aunt, and sist...
Source: Acta Haematologica - January 24, 2020 Category: Hematology Authors: Peng Y, Nie L, Qin C, Wan L, Zhou P Tags: Acta Haematol Source Type: research

Coexistence of Pure Red Cell Aplasia and Autoimmune Haemolytic Anaemia Associated with Thymoma.
Abstract Thymoma is an uncommon neoplasia derived from the epithelial cells of the thymus, which leads to immune dysregulation and is associated with a series of autoimmune diseases. However, the concurrence of these disease entities is rare, and the exact mechanisms of these diseases are still unclear. We have admitted several cases who were diagnosed with thymoma, autoimmune haemolytic anaemia, and pure red cell aplasia. These cases were the first to report the concurrence of these three disorders. After thymectomy, anaemia improved, haemolytic cells decreased, and haemoglobin was normalized. PMID: 31962320...
Source: Acta Haematologica - January 21, 2020 Category: Hematology Authors: Wang W, Chen LY, Zhao W, Ren Y, Wang L, Li X, Jiang Y, Gao H Tags: Acta Haematol Source Type: research

Alpha-Thalassemia Carrier due to - α3.7 Deletion: Not So Silent.
CONCLUSION: Heterozygosity for the -α3.7 deletion was associated with clinically significant microcytosis and mild anemia in our pediatric population. In the absence of iron deficiency and β-thalassemia, this finding provides a diagnosis for mild microcytic anemia, making additional investigations of microcytosis unnecessary. PMID: 31935715 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - January 14, 2020 Category: Hematology Authors: Gilad O, Steinberg-Shemer O, Dgany O, Krasnov T, Noy-Lotan S, Tamary H, Yacobovich J Tags: Acta Haematol Source Type: research

Exosomes in Chronic Myeloid Leukemia: Are We Reading a New Reliable Message?
PMID: 31922494 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - January 9, 2020 Category: Hematology Authors: Bernardi S, Malagola M, Polverelli N, Russo D Tags: Acta Haematol Source Type: research

Impact of Platelet Counts, Surgical Methods, and Preoperative Platelet Transfusion on the Outcome of Splenectomy for Immune Thrombocytopenia.
CONCLUSIONS: Baseline and postoperative platelet counts are apparently associated with the treatment response to splenectomy but the difference did not reach statistical significance. Preoperative platelet transfusion did not overcome the disadvantage of thrombocytopenia and was not recommended when other preparative measures are available. PMID: 31910413 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - January 7, 2020 Category: Hematology Authors: Ong YC, Chang H, Yeh TS, Kuo MC, Li PL, Wang PN, Lin TL, Wu JH, Hung YS Tags: Acta Haematol Source Type: research

Rapid Complete Response to Single-Agent Bcl-2 Inhibitor Venetoclax in a Heart-Transplanted Patient with Triple Refractory Immunoglobulin Light-Chain Amyloidosis.
Abstract Immunoglobulin light-chain amyloidosis (AL) is a disease with limited treatment options due to the frailty of patients caused by organ damage. Since the clonal plasma cells often contain the cytogenetic aberration t(11;14), the Bcl-2 inhibitor venetoclax is suggested to have a role in the treatment of AL. Here, we report of a heart-transplanted patient, refractory to multiple therapies, reaching a rapid complete response with single-agent venetoclax. PMID: 31896106 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - January 2, 2020 Category: Hematology Authors: Gran C, Borg Bruchfeld J, Ellin F, Nahi H Tags: Acta Haematol Source Type: research

Avoiding the Thorns of the Gifted Red Rose: Case Report of Late Diagnosis of Polycythemia Rubra Vera in a Sibling Bone Marrow Transplantation Donor for a Patient with Chronic Myeloid Leukemia.
PMID: 31851977 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - December 18, 2019 Category: Hematology Authors: Amitai I, Lipton JH Tags: Acta Haematol Source Type: research

Hemophilia A in Females: Considerations for Clinical Management.
Abstract Approximately 50% of female carriers of hemophilia A have factor VIII (FVIII) levels below 0.5 IU/dL and may be categorized as having mild hemophilia. Females with hemophilia may go undiagnosed for years because the most common symptoms - menorrhagia and bleeding after childbirth - also occur in females without hemophilia. Females with hemophilia can exhibit increased bleeding tendencies despite current guidelines of expected, adequate FVIII levels. The cases described illustrate the clinical variability and presentation of hemophilia in females and highlight the importance of a timely diagnosis, effectiv...
Source: Acta Haematologica - December 11, 2019 Category: Hematology Authors: Bryant P, Boukouvala A, McDaniel J, Nance D Tags: Acta Haematol Source Type: research

Risk Factors for Acute Kidney Injury and Chronic Kidney Disease following Allogeneic Hematopoietic Stem Cell Transplantation for Hematopoietic Malignancies.
In this study, 114 patients who had undergone allo-HSCT were retrospectively analyzed to investigate the risk factors for onset of posttransplant AKI and CKD as defined by the new Kidney Disease Improving Global Outcomes criteria. RESULTS: Seventy-four patients (64.9%) developed AKI and 25 (21.9%) developed CKD. The multivariate analysis showed that the risk factors for developing stage 1 or higher AKI were age ≥46 years at the time of transplant (p = 0.001) and use of ≥3 nephrotoxic drugs (p = 0.036). For CKD, the associated risk factors were disease status other than complete remission at the time of transplan...
Source: Acta Haematologica - December 10, 2019 Category: Hematology Authors: Sakaguchi M, Nakayama K, Yamaguchi H, Mii A, Shimizu A, Inai K, Onai D, Marumo A, Omori I, Yamanaka S, Fujiwara Y, Fukunaga K, Ryotokuji T, Hirakawa T, Okabe M, Tamai H, Okamoto M, Wakita S, Yui S, Tsuruoka S, Inokuchi K Tags: Acta Haematol Source Type: research

Kidney Disease after Allogeneic Hematopoietic Cell Transplantation: In Search of the Truth.
PMID: 31794970 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - December 3, 2019 Category: Hematology Authors: Gavriilaki E, Sakellari I Tags: Acta Haematol Source Type: research

Reexamining 1-Gene Deletion α-Thalassemia: Is the Silent Carrier of Clinical Significance?
Reexamining 1-Gene Deletion α-Thalassemia: Is the Silent Carrier of Clinical Significance? Acta Haematol. 2019 Nov 27;:1-2 Authors: Andolina JR PMID: 31775142 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - November 27, 2019 Category: Hematology Authors: Andolina JR Tags: Acta Haematol Source Type: research

FLAG and Etoposide as Salvage Treatment in Acute Myeloid Leukemia.
PMID: 31751992 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - November 21, 2019 Category: Hematology Authors: Papadantonakis N Tags: Acta Haematol Source Type: research

Extended Follow-up of an Educational Intervention Encouraging Appropriate Use of Blood Transfusions.
Abstract INTRODUCTION: A restrictive transfusion strategy of packed red blood cells (PRBCs) has been associated with at least non-inferior patient outcomes in a variety of clinical settings. In December 2014, we conducted an educational intervention which consisted of an oral presentation and computerized notifications at a single tertiary medical center. OBJECTIVE: The aim of this study was to examine the long-term effects of a simple and low-cost educational intervention aimed to promote awareness to transfusion guidelines. METHODS: We retrospectively analyzed all PRBC transfusions ordered between 2014...
Source: Acta Haematologica - November 21, 2019 Category: Hematology Authors: Pasvolsky O, Shepshelovich D, Berger T, Tadmor B, Shochat T, Raanani P, Yahalom V, Gafter-Gvili A Tags: Acta Haematol Source Type: research

Weekly Cyclophosphamide-Bortezomib-Dexamethasone Induction Performs Comparably to Twice-Weekly Dosing with Respect to Both Response Rates and Survival after Autologous Transplant.
PMID: 31689702 [PubMed - as supplied by publisher] (Source: Acta Haematologica)
Source: Acta Haematologica - November 5, 2019 Category: Hematology Authors: Thirunavukarasu C, Weber N, Morris K, Subramonia Pillai E, Curley C, Butler J, Kennedy G Tags: Acta Haematol Source Type: research

A Retrospective Study of the Combination of Rituximab, Cyclophosphamide and Dexamethasone for the Treatment of Relapsed/Refractory Warm Antibody Autoimmune Hemolytic Anemia.
Abstract The combination of rituximab, cyclophosphamide, and dexamethasone (RCD) is highly effective in the treatment of warm autoimmune hemolytic anemia (WAIHA) associated with chronic lymphocytic leukemia (CLL). We treated a cohort of patients with relapsed/refractory WAIHA, without CLL, with RCD. The primary objective was to evaluate the overall response (OR) of RCD therapy. Complete response (CR) was defined as a hemoglobin (Hgb) ≥12 g/dL. Partial response (PR) was defined as Hgb 10-11.9 g/dL or ≥2 g/dL increase in Hgb. Sustained response was defined as Hgb ≥10 g/dL with no treatment changes. A total ...
Source: Acta Haematologica - October 30, 2019 Category: Hematology Authors: Piatek CI, Bocian H, Algaze S, Weitz IC, O Apos Connell C, Liebman HA Tags: Acta Haematol Source Type: research