Abstract:
:A 63-year-old white man with myasthenia gravis and red cell aplasia was found to have hepatic iron overload. The differential diagnosis included hereditary hemochromatosis and secondary iron overload related to red cell aplasia. Family studies of siblings, including HLA typing, initially suggested a diagnosis of secondary iron overload. The investigation of a remote sibling led to the final diagnosis of hereditary hemochromatosis. The case illustrates the importance of complete family investigations in the differentiation between hereditary hemochromatosis and secondary iron overload and the potential hazard of using HLA typing as an indirect genetic test for hereditary hemochromatosis.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Adams PCdoi
10.1002/ajh.2830450314subject
Has Abstractpub_date
1994-03-01 00:00:00pages
260-1issue
3eissn
0361-8609issn
1096-8652journal_volume
45pub_type
杂志文章abstract::Transformation to secondary myelofibrosis (MF) occurs as part of the natural history of polycythemia vera (PPV-MF) and essential thrombocythemia (PET-MF). Although primary (PMF) and secondary MF are considered similar diseases and managed similarly, there are few studies specifically focused on the latter. The aim of ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24377
更新日期:2016-07-01 00:00:00
abstract::This report describes studies investigating the use of a collagen binding assay to improve the laboratory monitoring of desmopressin (DDAVP) therapy in patients with von Willebrand's disease (vWD). We evaluated the response of seven patients with vWD (four type I, three type IIA) to DDAVP, administered using a standar...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450303
更新日期:1994-03-01 00:00:00
abstract::Cytotoxic agents like Hydroxyurea, Busulfan and Interferon-alpha are to date the most commonly used therapeutic approaches in Essential Thrombocythemia (ET). However, few data on the efficacy and safety of these agents in the long-term are currently available. We report a retrospective analysis of the long-term outcom...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21360
更新日期:2009-04-01 00:00:00
abstract::Polycythemia vera (PV) and essential thrombocythemia (ET) are common types of myeloproliferative disorders (MPD), the prevalence of which has not been well documented in the United States. Recent breakthroughs in the molecular etiology of these disorders and the accelerated development of targeted pharmacotherapeutics...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21129
更新日期:2008-05-01 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::Although large vessel thrombi are occasionally reported in patients with homozygous sickle cell disease, the role of intravascular coagulation in typical pain crises is controversial. Therefore, we studied 24 sickle cell patients during and between episodes of pain crisis, using several sensitive tests of hemostasis. ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830230403
更新日期:1986-12-01 00:00:00
abstract:DISEASE OVERVIEW:Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 8500 new patients annually and representing approximately 10.2% of all lymphomas in the United States. DIAGNOSIS:HL is composed of two distinct disease entities: classical HL and nodular lymphocyte predominant HL. Nodular sclero...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25071
更新日期:2018-05-01 00:00:00
abstract::Thalassemia is an inherited blood disorder that requires lifelong adherence to a complicated and burdensome medical regimen which could potentially impact emotional functioning of patients. The importance of understanding and promoting healthy emotional functioning is crucial not only to psychological well-being, but ...
journal_title:American journal of hematology
pub_type: 信件,多中心研究
doi:10.1002/ajh.21826
更新日期:2010-10-01 00:00:00
abstract::We present a female patient with T-gamma LGL leukemia, who was followed for the last 20 years. Over these years she developed several autoimmune disorders, including Sjögren's syndrome, Hashimoto's thyroiditis, premature ovarian failure (compatible with type II autoimmune polyglandular syndrome), amegakaryocytic throm...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10024
更新日期:2002-02-01 00:00:00
abstract::Conventional treatment of mantle cell lymphoma (MCL) yields modest responses and short remissions. We report 30 hematopoietic stem cell transplants (HSCT) for MCL: 13 autologous, 10 allogeneic myeloablative, and 7 nonablative. After a median 1.2 years from diagnosis (range 0.5 to 4.7) and a median of 2 pre-HSCT chemot...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20646
更新日期:2006-07-01 00:00:00
abstract::Several histone deacetylase inhibitors (HDACi), including vorinostat, have been approved for the therapy of cutaneous T-cell lymphoma (CTCL). Emerging data suggest that HDACi may exert immune suppressive effects which would be disadvantageous for therapy of CTCL. We describe a patient with Sezary syndrome who was moni...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22231
更新日期:2012-02-01 00:00:00
abstract::The erythrocytes of 90 pregnant women were evaluated for the presence of in vivo or in vitro oxidant damage. The reduced glutathione (P less than 0.005) and the membrane reduced sulfhydryl (P less than 0.001) concentrations were decreased in fresh erythrocytes. Following incubation with acetylphenylhydrazine, Heinz bo...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830180204
更新日期:1985-02-01 00:00:00
abstract::A 1750 base pair cDNA to human terminal deoxynucleotidyl transferase (TdT) has been cloned. This cDNA detects a dominant 2200 base pair messenger RNA species in normal and leukemic cells synthesizing the enzyme. A quantitative dot blot assay was utilized to survey a number of clinical samples from patients with TdT po...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830250305
更新日期:1987-07-01 00:00:00
abstract::Children with sickle cell anemia (SCA) and a primary overt stroke are at high risk of recurrent (secondary) stroke. Chronic transfusion therapy dramatically reduces but does not eliminate this high risk, and inevitably results in transfusion-related hemosiderosis. We previously reported the use of hydroxyurea/phleboto...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21986
更新日期:2011-04-01 00:00:00
abstract::The objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myeloid metaplasia (AMM). We searched the literature (Medline, 1970-1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or trans...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830330210
更新日期:1990-02-01 00:00:00
abstract::Autoantibody production following allogeneic stem-cell transplantation is common and is often ascribed to the immune dysregulation associated with graft-versus-host disease. Recent data suggests that donor-memory B cells can be reactivated on exposure to antigen and result in antibody production in the recipient ident...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20350
更新日期:2005-08-01 00:00:00
abstract:DISEASE OVERVIEW:Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, and lymphadenopathy. DIAGNOSIS:Presence of IgM monoclonal protein associated with 10% clonal lymphoplasmacytic cell...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.22014
更新日期:2011-05-01 00:00:00
abstract::Zoledronic acid (ZOL), an intravenous bisphosphonate, has been shown to reduce and delay the incidence of skeletal-related events (SREs) in multiple myeloma (MM) patients with bone disease. A retrospective claims-based analysis was conducted that used two distinct US managed care databases to examine the relationship ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23164
更新日期:2012-05-01 00:00:00
abstract::Thrombotic complications occur at high rates in hospitalized patients with COVID-19, yet the impact of intensive antithrombotic therapy on mortality is uncertain. We examined in-hospital mortality with intermediate- compared to prophylactic-dose anticoagulation, and separately with in-hospital aspirin compared to no a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.26102
更新日期:2021-01-21 00:00:00
abstract::Splenectomy is a time-honoured well established approach for patients with steroid-resistant immune thrombocytopenia (ITP). However, due to the more recent availability of therapeutic options alternative to splenectomy, such as rituximab and agonists of the thrombopoietin-receptor, the choice of second-line therapy is...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23823
更新日期:2014-11-01 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::To better assess the efficacy and safety of rituximab in adults' warm antibody autoimmune hemolytic anemia (wAIHA), we conducted a retrospective study including 27 adults (mean age 49.7 +/- 21 years) with either primary (n = 17) or secondary (n = 10) wAIHA. On average, the patients received 2.1 +/- 1.4 treatment lines...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.21341
更新日期:2009-03-01 00:00:00
abstract::Seventeen adults received the antifibrinolytic drug tranexamic acid during cardiac surgery utilizing extracorporeal circulation (ECC). In 8 patients, drug administration began prior to skin incision (pre-ECC); infusions commenced after ECC and protamine administration in another 9 patients (post-ECC). Compared with th...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ajh.2830380208
更新日期:1991-10-01 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::Hypereosinophilic syndrome (HES) is a rare disorder that can manifest in various organ systems. We report the case of a 54-year-old woman with a remote history of seizure disorder who presented with early signs of right-sided heart failure. Laboratory studies showed significant eosinophilia (8 x 10(9) l(-1)). Computed...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20933
更新日期:2007-10-01 00:00:00
abstract::An association between thrombocytopenia and thyrotoxicosis in a single individual is well documented, and the theories for this event include a common immunologic cause or a thyrotoxic-induced decrease in platelet survival. We report the first description of the coexistence of autoimmune thrombocytopenic purpura (AITP...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830390411
更新日期:1992-04-01 00:00:00
abstract::Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 x 10(9)/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor th...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.10255
更新日期:2003-02-01 00:00:00
abstract::Cultured endothelial cells from bovine aorta were exposed to oxidized low density lipoprotein and examined by electron microscopy. The endothelial cells contracted slightly and the intercellular junctions became unclear. Some osmiophilic material increased in the cytoplasm. The oxidized low density lipoprotein appears...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490315
更新日期:1995-07-01 00:00:00
abstract::Familial Pseudohyperkalemia (FP) is a dominant red cell trait characterized by increased serum [K(+)] in whole blood stored at or below room temperature, without additional hematological abnormalities. Functional gene mapping and sequencing analysis of the candidate genes within the 2q35-q36 critical interval identifi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23357
更新日期:2013-01-01 00:00:00
abstract::Lysosomes (granules) of rabbit PMN leukocytes were extracted with either HCl or H2SO4, and the extracts were chromatographed over Sephadex to separate protein constituents. Some of the low molecular weight cationic proteins homogeneous on SDS PAGE (8% and 12.5% gels) were characterized by electrophoretic mobility in a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830070309
更新日期:1979-01-01 00:00:00