Abstract:
:Erythrocytes from ten patients with severe liver disease displayed low methylene blue-stimulated hexose monophosphate (HMP) shunt activity and glucose recycling despite elevated total glucose consumption when compared to controls. Heinz body formation was increased and reduced glutathione concentration significantly decreased. After hemolysis, no differences in methylene-blue estimulated HMP shunt activity or glucose recycling could be demonstrated between patients and controls. The addition of 2- and 4-mM NADP to the hemolysates produced significantly greater HMP shunt activity and glucose recycling in the patients' hemolysates. The addition of NADPH to the incubation mixture produced no significant stimulation of either HMP shunt activity or glucose recycling, unless methylene blue was also added. Omission of NAD or phosphate from the incubation mixture produced no change in shunt metabolism. The absence of supplemental ATP resulted in extremely low shunt metabolism and refractoriness to NADP stimulation in both patients and controls. In the absence of additional magnesium, a reduction of shunt metabolism was noted. These data suggest that the defect in stimulated shunt metabolism in the intact erythrocytes of patients with hepatic disease does not result from an absolute enzyme deficiency, but rather from an unavailability of NADP or other cofactor.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Smith JR,Kay NE,Gottlieb AJ,Oski FAdoi
10.1002/ajh.2830060403subject
Has Abstractpub_date
1979-01-01 00:00:00pages
313-21issue
4eissn
0361-8609issn
1096-8652journal_volume
6pub_type
杂志文章abstract::Imatinib mesylate radically changed the natural history of chronic myeloid leukemia (CML). The recent availability of alternative tyrosine kinase inhibitors (TKIs) renders the clinical management of CML more complex. In this article, we summarize our long-term single institution experience. From 2003 to 2012, 102 pati...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ajh.23804
更新日期:2014-10-01 00:00:00
abstract::The standard dose of clofarabine is 52 mg/m2 for pediatrics and 40 mg/m2 in adults. Clofarabine dosed at 52 mg/m2 was used in adult patients with refractory ALL to maximize response before allo-HSCT. All patients had a significant response to therapy. Published pharmacokinetic analysis revealed no difference in peak p...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21365
更新日期:2009-04-01 00:00:00
abstract::Despite the common practice of combining dexamethasone (Dex) with bortezomib (Bz) in patients with multiple myeloma (MM), until now there has been few prospective trials undertaken. We undertook a trial that recapitulated the original APEX study except that dexamethasone was incorporated from cycle 1. We also incorpor...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.23967
更新日期:2015-05-01 00:00:00
abstract::The role of reduced-intensity conditioning (RIC) in adult acute lymphoblastic leukemia (ALL) remains unclear because of the small sample size, short follow-up duration, various regimens for conditioning and graft-versus-host disease (GVHD) prophylaxis, and the heterogeneity of selection criteria for transplantation. W...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.23465
更新日期:2013-08-01 00:00:00
abstract::Specific anti-human thymus xenoantiserum (ATS) was utilized for characterizing a human thymus antigen (HTA) preferentially expressed on human thymocytes. Binding of ATS with different cell types was studied by immunofluorescence and immunoperoxidase techniques, as well as by radioimmunoprecipitation (RIP) followed by ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830100206
更新日期:1981-01-01 00:00:00
abstract::Decreased von Willebrand factor (VWF)-cleaving protease activity (<5%) has been implicated in patients with congenital thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (Upshaw-Schulman syndrome) and associated with mutations within the ADAMTS13 gene. In this report, we describe longitudinal studies in a p...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20221
更新日期:2004-12-01 00:00:00
abstract::A patient with Philadelphia chromosome (Ph) chronic myelogenous leukemia (CML), in chronic phase, was treated with recombinant gamma-interferon (r gamma-IFN) in a phase I clinical trial. Prior to treatment, analysis of in vitro agar culture parameters indicated hyporesponsiveness of granulocyte-macrophage colony-formi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280105
更新日期:1988-05-01 00:00:00
abstract::We studied red blood cell morphology in two marathon runners before, immediately after, and 1 day after a marathon race. A discocyte-stomatocyte transformation was found by light microscopy of wet preparations and also by scanning electron microscopy, with about one-half the erythrocytes becoming cup-shaped after the ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830190213
更新日期:1985-06-01 00:00:00
abstract::This report describes the bone marrow findings in four patients whose marrow was involved by anaplastic large-cell Ki-1 lymphoma, an uncommon event in this type of lymphoma. In the marrow aspirate smears, the involvement was subtle, and was in the form of isolated large cells with irregular nuclear configuration, coar...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370209
更新日期:1991-06-01 00:00:00
abstract::Venous thromboembolism (VTE) is a common cause of morbidity and mortality among patients with multiple myeloma (MM). The International Myeloma Working Group (IMWG) developed guidelines recommending primary thromboprophylaxis, in those identified at high-risk of VTE by the presence of risk factors. The National Compreh...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25603
更新日期:2019-11-01 00:00:00
abstract::Infantile malignant osteopetrosis (IMO) includes various genetic disorders that affect osteoclast development and/or function. Genotype-phenotype correlation studies in IMO have been hampered by the rarity and heterogeneity of the disease and by the severity of the clinical course, which often leads to death early in ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21447
更新日期:2009-08-01 00:00:00
abstract::We recently defined event-free survival at 24 months (EFS24) as a clinically relevant outcome for patients with DLBCL. Patients who fail EFS24 have very poor overall survival, while those who achieve EFS24 have a subsequent overall survival equivalent to that of the age- and sex-matched general population. Here, we de...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24223
更新日期:2016-02-01 00:00:00
abstract::Invasive fungal disease (IFD) is a significant cause of morbidity and mortality in patients undergoing treatment for acute leukemia (AL). Antifungal prophylactic strategies are associated with significant toxicities and cost. We performed a retrospective study of the incidence and risk factors for IFD among patients n...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21776
更新日期:2010-09-01 00:00:00
abstract::Mutation of the VHL tumor suppressor gene is a frequent genetic event in the carcinogenesis of renal-cell carcinoma (RCC). Circulating endothelial progenitor cells (EPCs) have important role in neoangiogenesis, and mobilization of these cells is induced by various growth factors including erythropoietin (EPO). With th...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21019
更新日期:2008-02-01 00:00:00
abstract::The temporal development of the erythrocyte sedimentation rate (ESR) was studied in wide, short vacuum tubes. It was found that in about 3% of the specimens arriving in the laboratory the ESR developed in three different phases during 60 min, whereas the other showed only two. The specimens with three phases behaved s...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370310
更新日期:1991-07-01 00:00:00
abstract::A patient with acute nonlymphoblastic leukemia (FAB M4), showing a near tetraploid chromosome complement on his marrow cells, was treated with low dose cytosine arabinoside and achieved remission. During remission the near tetraploid marrow chromosome complement disappeared and reappeared upon relapse of leukemia. The...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830190412
更新日期:1985-08-01 00:00:00
abstract::Positron emission tomography-computed tomography (PET-CT) can identify bony lesions, assess disease burden, and detect extramedullary disease (EMD) in patients with multiple myeloma. We retrospectively reviewed records of patients who underwent PET-CT within 60 days of a new diagnosis (before therapy commenced) to ide...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25279
更新日期:2018-12-01 00:00:00
abstract::We aimed to determine the survival benefits of chemotherapy (CT) added to radiotherapy (RT) in different risk groups of patients with early-stage extranodal nasal-type NK/T-cell lymphoma (ENKTCL), and to investigate the risk of postponing RT based on induction CT responses. A total of 1360 patients who received RT wit...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.25878
更新日期:2020-09-01 00:00:00
abstract::Thrombospondin (TSP), a large protein found in platelet alpha-granules (as TSP-1), mediates adhesion of sickle reticulocytes to cultured vascular endothelium. To further explore the physiologic relevance of this observation, we have measured plasma TSP levels and platelet TSP-1 content in subjects with sickle cell dis...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199604)51:4<296::AID-AJH8>
更新日期:1996-04-01 00:00:00
abstract::Neutrophil elastase (NE) is a serine protease stored in the primary granules of neutrophils that proteolytically cleaves multiple cytokines and cell surface proteins on release from activated neutrophils. Recent reports of mutations in the gene encoding this enzyme in some patients with neutropenic syndromes prompted ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10434
更新日期:2003-11-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20014
更新日期:2004-04-01 00:00:00
abstract::We report the long-term follow-up results of a phase II trial of thalidomide for early-stage multiple myeloma (MM). Patients were eligible if they had smoldering multiple myeloma (SMM) or indolent MM without the need for immediate therapy. Thalidomide was initiated at a dose of 200 mg/day and adjusted as tolerated. Di...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.21821
更新日期:2010-10-01 00:00:00
abstract:DISEASE OVERVIEW:Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extracutaneous organs. DIAGNOSIS:The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23459
更新日期:2013-07-01 00:00:00
abstract::Gaucher disease (GD) is a lysosomal storage disorder; symptomatic patients with type 1 GD need long-term disease-specific therapy of which the standard of care has been enzyme replacement therapy (ERT). Thirty-eight of 40 patients (aged 9-71 years) clinically stable on ERT with imiglucerase, safely switched to a compa...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.24007
更新日期:2015-07-01 00:00:00
abstract::Lenalidomide and azacitidine are active in MDS patients, and may complement each other by targeting the bone marrow microenvironment and the malignant clone. A recent Phase I trial testing the lenalidomide and azacitidine combination yielded encouraging results; however, lenalidomide’s contribution was unclear. In thi...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.21891
更新日期:2011-01-01 00:00:00
abstract::The role of leukapheresis was explored in the management of anaemia and thrombocytopenia complicating the accumulation of lymphoid cells in patients with chronic lymphocytic leukaemia and lymphoma. The aim of this study was to determine the efficiency of this procedure in correcting these complicating features and to ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830060411
更新日期:1979-01-01 00:00:00
abstract:BACKGROUND:The histopathologic features characterizing the involvement of the bone marrow (BM) in systemic lupus erythematosus (SLE) have not been systematically analyzed to date. OBJECTIVES:The aim of this study was to assess morphologic and immunohistochemical characteristics of BM involvement in SLE. PATIENTS AND ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20593
更新日期:2006-08-01 00:00:00
abstract::Chimeric antigen receptor (CAR) T-cell therapy represents a major advancement in personalized cancer treatment. In this strategy, a patient's own T cells are genetically engineered to express a synthetic receptor that binds a tumor antigen. CAR T cells are then expanded for clinical use and infused back into the patie...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25418
更新日期:2019-05-01 00:00:00
abstract::Extramedullary meningeal hematopoiesis (EMH) represents an uncommon finding after stem-cell transplantation. We describe the case of an allogeneic bone marrow transplantation (BMT) recipient who developed EMH 1 month after radiation myelitis had been diagnosed. A 39-year-old man with multiple myeloma underwent matched...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20341
更新日期:2005-08-01 00:00:00
abstract::Sickle (Hb SS) red cells, preloaded with 45Ca by reversal of hemolysis, exhibit an incomplete 45Ca extrusion, retaining approximately four times more 45Ca than normal cells. Studies indicated that neither the reduction in Hb SS cell Ca2+-Mg2+ ATPase activity (84% of normal) nor the activation of Ca2+-Mg2+ ATPase by ca...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830080406
更新日期:1980-01-01 00:00:00