Abstract:
:Primary iron overload encompasses a variety of genetic iron overload syndromes, dominated in frequency by HFE-related, or Type 1 hemochromatosis, for which French diagnostic and therapeutic guidelines have been recently proposed. Differential diagnosis of Type 1 hemochromatosis can be made from both clinical data and genetic studies. Venesection therapy and family screening remain the basis for the curative and preventive management of most genetic iron overload diseases.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Brissot Pdoi
10.1002/ajh.21071subject
Has Abstractpub_date
2007-12-01 00:00:00pages
1140-1issue
12 Suppleissn
0361-8609issn
1096-8652journal_volume
82pub_type
杂志文章abstract::CD7 antigen, a T-cell lineage associated antigen, is expressed in a minority of patients with acute myeloid leukemia (AML). The biologic and clinical significance of this finding is not clearly established. In this retrospective study of patients with de novo acute myeloid leukemia, we have identified CD7 expression a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199808)58:4<278::aid-ajh5>
更新日期:1998-08-01 00:00:00
abstract::Cutaneous lymphoma is a disease characterized with massive skin infiltration of lymphoid malignant cells. They commonly express some T-cell markers, such as CD2, CD3, CD4, and CD7, and thus termed as CTCL (cutaneous T cell lymphoma). Here, we present a case with CD56/N-CAM-positive cutaneous lymphoma, which appears ly...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470406
更新日期:1994-12-01 00:00:00
abstract::Acute Myeloid Leukemia (AML) and myelodysplasia (MDS) with chromosome 3q abnormalities have a dismal outcome either untreated or with conventional treatments. Azacitidine (AZA) is now considered as the standard of care in high-risk MDS and oligoblastic AML patients. The objective of this study was to evaluate the impa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24099
更新日期:2015-10-01 00:00:00
abstract::Liquid culture of the mononuclear fraction of the blood of marmosets without any added stimulator resulted in the appearance of megakaryocytes. These cells could not be identified by light microscopy or by indirect immunofluorescence using antiplatelet antiserum. Ultrastructural identification was made using platelet ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830080108
更新日期:1980-01-01 00:00:00
abstract::A rare association of congenital afibrinogenemia and hereditary protein C deficiency is described in a 37-year-old female who suffered from ischemic necrosis in the left first toe. The diagnosis of afibrinogenemia was assessed by the absence of fibrinogen in clotting and immunological assays. The diagnosis of heredita...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830410111
更新日期:1992-09-01 00:00:00
abstract::Gamma inferferon (gamma IFN), alpha tumor necrosis factor (alpha TNF), and interleukin 6 (IL-60) are cytokines produced by a wide variety of cells, including T lymphocytes and NK cells. These cytokines affect B-cell proliferation and differentiation into immunoglobulin secreting cells. In addition, gamma IFN and alpha...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490403
更新日期:1995-08-01 00:00:00
abstract::Individuals with more than one defect in natural coagulant/anticoagulant systems have been postulated to be at an increased risk for thrombotic events. We report a case of combined protein S and C deficiency in a young woman, which resulted in fatal arterial mesenteric thrombosis. The role of coagulation defects in ar...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199807)58:3<246::aid-ajh17
更新日期:1998-07-01 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::Patients initially diagnosed with type 1 von Willebrand disease (VWD) have been reclassified as type 2 after a more exhaustive analysis in several studies. Our study's objectives were (1) to reanalyze patients that were previously diagnosed as type 1 to achieve a more accurate diagnosis and (2) to compare the von Will...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20470
更新日期:2005-11-01 00:00:00
abstract::A 67-year-old woman was admitted to our hospital because of lymphadenopathy and lymphocytosis. Monoclonal integration of HTLV-I provirus DNA was detected, and a diagnosis of adult T-cell leukemia (ATL) was made. Flow cytometry revealed that the ATL cells expressed CD20 as well as T-cell-associated antigens, and expres...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200101)66:1<39::AID-AJH1005>3.0.
更新日期:2001-01-01 00:00:00
abstract::Mutations in the calreticulin gene (CALR) were recently identified in approximately 70-80% of patients with JAK2-V617F-negative essential thrombocytosis and primary myelofibrosis. All frameshift mutations generate a recurring novel C-terminus. Here we provide evidence that mutant calreticulin does not accumulate effic...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24245
更新日期:2016-02-01 00:00:00
abstract::Protein S (ProS) is a physiological inhibitor of coagulation with an important function in the down-regulation of thrombin generation. ProS deficiency is a major risk factor for venous thrombosis. This study enrolled 40 ProS-deficient probands to investigate the molecular basis of hereditary ProS deficiency in Chinese...
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.23525
更新日期:2013-10-01 00:00:00
abstract::Autoimmune hemolytic anemia is thought to be mediated via auto-antibodies produced by lymphoid B cells. This may be an idiopathic process or secondary to an underlying infection or lymphoproliferative disorder. Conventional treatment comprises immunosuppression with corticosteroids and, in some cases, splenectomy. A p...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20220
更新日期:2005-02-01 00:00:00
abstract::We describe a 25-year-old black woman who presented with a long history of anemia requiring transfusions during childhood and adolescence. Molecular analysis revealed her to be a compound heterozygote for the sickle mutation and the approximately 22.7 kb deletion associated with hemoglobin Kenya. This patient's clinic...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830410413
更新日期:1992-12-01 00:00:00
abstract::Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in β-thalassemia major. We investigated the mechanical and endocrine function of the left atrium and ventricle to identify early signs of dysfunction. We studied 90 patients (mean age: 2...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23581
更新日期:2014-01-01 00:00:00
abstract::AL amyloidosis, a systemic disorder characterized by widespread deposition of amyloid fibrils derived from monoclonal Ig light chains in organs and soft tissues, is typically caused by an underlying plasma cell dyscrasia. However, this disease can also be associated rarely with a B-cell lymphoproliferative disorder. I...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20635
更新日期:2006-09-01 00:00:00
abstract::Philadelphia chromosome (Ph1) positive, or "adult-type," chronic granulocytic leukemia (CGL) differs in many clinical and laboratory aspects from PH1-negative, or "juvenile," CGL. Because of these differences, the disorders have been considered two distinct disease entities. We present a child with Ph1-positive CGL wh...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830160114
更新日期:1984-01-01 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::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::We report a case of a 61-year-old man with head and neck cancer who presented with pancytopenia two months after the completion of his chemotherapy and was diagnosed with myelodysplasia on the basis of two bone marrow examinations, before the correct diagnosis of visceral leishmaniasis was established with splenectomy...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10408
更新日期:2003-11-01 00:00:00
abstract::Initially described in 1948 by Hertert thromboelastography (TEG) provides a real-time assessment of viscoelastic clot strength in whole blood. Rotational thromboelastometry (ROTEM) evolved from TEG technology and both devices generate output by transducing changes in the viscoelastic strength of a small sample of clot...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23599
更新日期:2014-02-01 00:00:00
abstract::Various in vitro studies and clinical observations suggest that Fanconi's anemia (FA) patients are unable to detoxify adequately superoxide anions (O2-) released by activated phagocytes. Recent studies have shown that certain lymphokines such as tumor necrosis factor-alpha (TNF-alpha) and interferon-gamma (IFN-gamma) ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830420211
更新日期:1993-02-01 00:00:00
abstract::Clopidogrel is a widely used antiplatelet agent that irreversibly inhibits platelet P2Y12 ADP receptors after conversion to an active metabolite. There are a number of laboratory tests capable of detecting clopidogrel-induced platelet inhibition and published literature correlates suboptimal clopidogrel response to ad...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.22112
更新日期:2011-12-01 00:00:00
abstract::To monitor successful engraftment after bone marrow transplantation, we performed Southern hybridization analysis or dot blot analysis of DNA in a set of sex-mismatched cases using a Y-chromosome-specific DNA probe (pHY10). This method was extremely sensitive and rapid for checking which cells contain the Y-chromosome...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830270108
更新日期:1988-01-01 00:00:00
abstract::The purpose of this study was to determine if therapeutic levels of Rituximab could be achieved in a patient with renal failure being dialyzed and if Rituximab is removed by hemodialysis. A 54-year-old man with low-grade lymphoma and renal failure on hemodialysis received 8 weekly treatments of Rituximab at 375 mg/M(2...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10213
更新日期:2002-11-01 00:00:00
abstract::Neonatal alloimmune thrombocytopenia is an uncommon but important cause of thrombocytopenia in infants. Because of the severity of the thrombocytopenia, some of these infants will have intracranial hemorrhage with resultant long-term disability. Obstetricians and neonatologists have recommended delivery by caesarean s...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280207
更新日期:1988-06-01 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::The specificity of autoantibodies in autoimmune hemolytic anemia (AIHA) has been studied using the serological procedure and immunoprecipitation technique with rare phenotype red cells. We attempted to analyze specificity using recombinant rhesus (Rh) blood group and band3 antigens expressed on erythroleukemic cell li...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.1161
更新日期:2001-10-01 00:00:00
abstract::An adolescent male patient developed functional asplenia in the course of graft versus host disease (GVHD) after successful allogeneic bone marrow transplantation (BMT) for aplastic anemia. Coincident with the onset of the asplenia, amelioration of the ongoing GVHD was observed. Unexpectedly, after 34 months of functi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120112
更新日期:1982-02-01 00:00:00
abstract::This review focuses on the initial clinical descriptions and subsequent investigation of the syndrome of recurrent infections associated with neutrophil membrane glycoprotein deficiencies. Characterization of the missing group of three glycoprotein heterodimers and their role in adhesion-related neutrophil function is...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830310416
更新日期:1989-08-01 00:00:00