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Acta Haematologica

Perla R Colunga-Pedraza, Gisela B Gomez-Cruz, Julia E Colunga-Pedraza, Guillermo J Ruiz-Argüelles
In 1963 Jean Bernard introduced the concept of "geographic hematology" and distinguished 2 branches, i.e., "ethnic hematology," which deals with differences between populations, and "environmental hematology," which considers factors such as food habits, infections, and others. Both of these branches have implications in the distribution of hematological diseases worldwide. In comparison with Caucasian populations, in Mexico a significantly higher prevalence of acute lymphoblastic, acute promyelocytic, and acute megakaryoblastic leukemias has been described...
September 18, 2018: Acta Haematologica
Yeping Luo, Zhuoying Li, Lihua Huang, Jing Tian, Menglong Xiong, Zuocheng Yang
BACKGROUND/AIMS: Hereditary spherocytosis (HS) is a common pediatric hemolytic anemia caused by congenital red blood cell defects. HS due to ankyrin 1 (ANK1) mutations is the most common type. We explored an ANK1 mutation from an HS patient and reviewed the literature. METHODS: We detected the mutation in a Chinese family in which 2 members were diagnosed with HS by next-generation sequencing. The proband was diagnosed with HS in the newborn period, based on clinical manifestations, laboratory data, and family history...
September 18, 2018: Acta Haematologica
Haitao Xu, Fusheng Yao
Waldenström macroglobulinemia (WM), also known as lymphoplasmacytic lymphoma, is rare but a clinicopathologically distinct B-cell malignancy. This study assessed differentially expressed genes (DEGs) to identify potential WM biomarkers and uncover the underlying the molecular mechanisms of WM progression using gene expression profiles from the Gene Expression Omnibus database. DEGs were identified using the LIMMA package and their potential functions were then analyzed by using the gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes pathway enrichment analyses and the protein-protein interaction (PPI) network analysis by using the Search Tool for the Retrieval of Interacting Genes/Proteins database...
September 18, 2018: Acta Haematologica
Shosaku Nomura
No abstract text is available yet for this article.
September 18, 2018: Acta Haematologica
Fiorina Giona, Michelina Santopietro, Giuseppe Menna, Maria Caterina Putti, Concetta Micalizzi, Nicola Santoro, Ottavio Ziino, Rosamaria Mura, Saverio Ladogana, Grazia Iaria, Antonella Sau, Roberta Burnelli, Nadia Vacca, Sayla Bernasconi, Caterina Consarino, Fara Petruzziello, Maria Luisa Moleti, Andrea Biondi, Franco Locatelli, Robin Foà
BACKGROUND: To date, no data on the adherence to specific guidelines for children with chronic myeloid leukemia (CML) in chronic phase (CP) have been reported. METHODS: Since 2001, guidelines for treatment with imatinib mesylate (IM) and monitoring in patients younger than 18 years with CP-CML have been shared with 9 pediatric referral centers (P centers) and 4 reference centers for adults and children/adolescents (AP centers) in Italy. In this study, the adherence to these guidelines was analyzed...
September 18, 2018: Acta Haematologica
Leslie Naesens, Helena Devos, Friedel Nollet, Lucienne Michaux, Dominik Selleslag
INTRODUCTION: Myeloid sarcoma (MS), previously known as granulocytic sarcoma or chloroma, is a rare neoplastic condition defined as a tumor mass consisting of myeloblasts or immature myeloid cells occurring at an extramedullary site. Clinical presentation is diverse and determined by a tumor mass effect or local organ dysfunction. CASE REPORT: We report the case of a 25-year-old previously healthy male with rapidly progressive shortness of breath. A chest CT scan demonstrated a heterogenous anterosuperior mediastinal mass with pleural and pericardial invasion...
September 18, 2018: Acta Haematologica
Yasuyuki Inoue, Manabu Matsunawa, Fumiaki Sano, Ikuo Miura
The prognosis for patients who experience hemostatic complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT) is poor. However, no report has investigated disseminated intravascular coagulation (DIC) caused by the complications of allo-HSCT without infection. Recombinant human soluble thrombomodulin (rhTM) was used to treat 12 episodes of DIC (n = 10; group 1) caused by allo-HSCT complications such as acute graft-versus-host disease (aGVHD) or thrombotic microangiopathy (TMA), and the clinical outcomes were compared with those of historical controls (n = 9; group 2) treated for DIC without rhTM...
September 18, 2018: Acta Haematologica
Martin H Steinberg
No abstract text is available yet for this article.
September 18, 2018: Acta Haematologica
Mihyang Ha, Ji-Young Kim, Myoung-Eun Han, Ga Hyun Kim, Si Young Park, Dae Cheon Jeong, Sae-Ock Oh, Yun Hak Kim
BACKGROUND: Certain nuclear envelope proteins are associated with important cancer cell characteristics, including migration and proliferation. Abnormal expression of and genetic changes in nuclear envelope proteins have been reported in acute myeloid leukemia (AML) patients. Transmembrane protein 18 (TMEM18), a nuclear envelope protein, is involved in neural stem cell migration and tumorigenicity. METHODS: To examine the prognostic significance of TMEM18 in AML patients, we analyzed an AML cohort from The Cancer Genome Atlas (TCGA, n = 142)...
September 10, 2018: Acta Haematologica
Mihir Bikhchandani, Ryan Johnson, Bertrand Tuan, Ayalew Tefferi
Myeloproliferative disorders with eosinophilia may possess the FIP1L1-PDGFRα gene rearrangement. When this rearrangement is present, imatinib usually results in complete remission. In rare cases of imatinib resistance, there is poor evidence guiding second-line therapy. We present the case of a 71-year-old male who presented with abdominal discomfort, fevers, and leukocytosis with eosinophilia. The patient was diagnosed with a myeloproliferative neoplasm with eosinophilia and FIP1L1-PDGFRα rearrangement after a bone marrow evaluation revealed hypercellular marrow with eosinophilia and fluorescence in situ hybridization identified the FIP1L1-PDGFRα rearrangement...
September 5, 2018: Acta Haematologica
Yang Chen, Yanyan Xie, Min Ruan, Jinning Shi
OBJECTIVE: The aim of this work was to investigate the influence of T lymphocyte subsets and platelet-specific autoantibodies on immune thrombocytopenia (ITP) with dexamethasone therapy. METHODS: The samples were obtained from patients before therapy. T lymphocyte subsets were measured by flow cytometry, and platelet-specific autoantibodies were evaluated by modified monoclonal antibody immobilization of platelet antigen assay. RESULTS: A total of 50 ITP patients were involved in the study...
2018: Acta Haematologica
Zhara A Al-Ali, Rana K Fallatah, Esra A Aljaffer, Eman R Albukhari, Neriman Sadek Al-Ali, Ziyad T Al-Ghannam, Reem Sayeb Al-Atrash, Ahmed Alsuliman, Chittibabu Vatte
Disease severity of sickle cell anemia is highly variable, and it is commonly accepted that fetal hemoglobin (HbF) levels play a major role as an ameliorating factor. Investigation of genetic variants have identified several genes to be the principal influencers of HbF regulation. Here, we further elucidated the association of rs4527238 and rs35685045 of ANTXR1 genes in the context of HbF level variance in sickle cell anemia patients of the Arab-Indian haplotype. Samples from 630 sickle cell anemia patients were analyzed for the mutations at 2 specific locations of the ANTXR1 gene by TaqMan®-based real-time PCR...
2018: Acta Haematologica
Uri Rozovski, Michael J Keating, Zeev Estrov
The immunoglobulin heavy chain gene (IgHV) mutation status correlates with the clinical outcome of patients with chronic lymphocytic leukemia (CLL) treated with chemoimmunotherapy. Why the survival rate of patients with unmutated IgHV is worse than that of patients with mutated IgHV is unknown. CLL cells with unmutated IgHV were thought to originate from naïve B lymphocytes, whereas CLL cells with mutated IgHV were thought to arise from B cells that have undergone somatic hypermutation (SHM). Cell surface protein expression profile and gene expression studies showing that all CLL cells, regardless of their IgHV mutation status, are of postgerminal center origin, negated this hypothesis...
2018: Acta Haematologica
Iuliana Vaxman, Daniel Shepshelovich, Lucille Hayman, Pia Raanani, Meir Lahav
Currently, there are only 2 case reports of Waldenström macroglobulinemia (WM) associated with severe neutropenia. This is a case report of a woman with a past medical history of WM who presented with neutropenic fever. The patient's febrile neutropenia resolved after RCD chemotherapy (cyclophosphamide 750 mg/m2, dexamethasone 20 mg, and rituximab 375 mg/m2). Fourteen days after administration, the neutrophil level had started to rise and normalized after 6 days. To the best of our knowledge, this is the 3rd reported case of agranulocytosis due to WM...
2018: Acta Haematologica
Jing Jia, Huijuan Wang, Miaomiao Wu, Feilong Zhang, Xuelin Liu, Wenming Chen, Aijun Liu
A 62-year-old female presenting with anemia and extensive hemorrhages is reported. Coagulation tests showed significantly prolonged prothrombin time (PT) and activated partial thromboplastin time. Decreased levels of clotting factor X activity were determined (5.4%). Bone marrow biopsy revealed neoplastic plasma cells. Serum and urine protein electrophoresis were both negative for monoclonal gammopathy, and both bone marrow and abdominal fat biopsies were negative for amyloid deposition. The patient was diagnosed as suffering from nonsecretory multiple myeloma (MM) complicated by acquired factor X deficiency...
2018: Acta Haematologica
Kanji Miyazaki, Kenshi Suzuki
No abstract text is available yet for this article.
2018: Acta Haematologica
Jianyun Wen, Qareen Haque, Fuyu Pei, Libai Chen, Yongsheng Ruan, Xuan Liu, Yuelin He, Xiaoqin Feng, Chunfu Li, Xuedong Wu
BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is currently the only curative treatment for thalassemia majorTM. Graft rejection (GR) and graft-versus-host disease (GVHD) are the primary obstacles to a successful outcome. METHODS: We conducted a retrospective study of HSCT in 29 children (median age at transplantation: 6 years) with Beta-thalassemia (β-TM) after the combined infusion of granulocyte colony-stimulating factor-primed bone marrow (G-BM) and cord blood (CB) from the human leukocyte antigen (HLA)-identical sibling donors...
2018: Acta Haematologica
Iman Abou Dalle, Jorge E Cortes, Pramod Pinnamaneni, Betty Lamothe, Adolfo Diaz Duque, Jasleen Randhawa, Naveen Pemmaraju, Elias Jabbour, Alessandra Ferrajoli, William G Wierda, Zeev Estrov, Marina Konopleva, Farhad Ravandi, Yesid Alvarado, Gautam Borthakur, Varsha Gandhi, Hagop M Kantarjian
Erlotinib, an epidermal growth factor receptor (EGFR) inhibitor, may have off-target activity inducing acute myeloid leukemia (AML) differentiation, possibly through SYK inhibition. We investigated erlotinib in a pilot phase II study for efficacy in relapsed/refractory AML patients at a dose of 150 mg once daily in 28-day cycles. Twenty-nine patients were treated for a median of 29 days (range 12-142 days). Seven patients (24%) received > 1 cycle of therapy and 12 (41%) discontinued treatment before day 28 due to disease progression...
2018: Acta Haematologica
Tsuyoshi Takahashi
No abstract text is available yet for this article.
2018: Acta Haematologica
Toyomi Kamesaki, Eiji Kajii
BACKGROUND: Direct antiglobulin test (DAT)-negative warm autoimmune hemolytic anemia (AIHA) is mainly caused by three mechanisms: red blood cell (RBC)-bound immunoglobulin (Ig)G below the detection limit of routine DAT; RBC-bound IgA or IgM; or low-affinity autoantibodies. Although most cases of DAT-negative AIHA are thought to be caused by RBC-bound IgG, and combinatory serological analyses are recommended, the relative ratios of each mechanism have not been clarified. METHODS: Two groups of patients with undiagnosed hemolytic anemia and negative conventional tube method-DAT (TM-DAT) were investigated using anti-IgA and anti-IgM sera, or column agglutination method-DAT (CM-DAT), respectively, in addition to radioimmunological quantitation of RBC-bound IgG...
2018: Acta Haematologica
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