Abstract:
DISEASE OVERVIEW:Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). DIAGNOSIS:The diagnosis of MF or SS requires the integration of clinical and histopathologic data. RISK-ADAPTED THERAPY:TNMB (tumor, node, metastasis, and blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a "risk-adapted," multidisciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin-directed therapies is preferred, as both disease-specific and overall survival for these patients is favorable. In contrast, patients with advanced-stage disease with significant nodal, visceral or blood involvement are generally approached with biologic-response modifiers or histone deacetylase inhibitors prior to escalating therapy to include systemic, single-agent chemotherapy. Multiagent chemotherapy (e.g., CHOP) may be employed for those patients with extensive visceral involvement requiring rapid disease control. In highly selected patients, allogeneic stem-cell transplantation may be considered.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Wilcox RAdoi
10.1002/ajh.23756subject
Has Abstractpub_date
2014-08-01 00:00:00pages
837-51issue
8eissn
0361-8609issn
1096-8652journal_volume
89pub_type
杂志文章,评审abstract::We describe a newly detected alpha-thalassemia-2 (alpha-thal-2) deletion characterized by a small -2.7-kb deletion involving the alpha 1 globin gene. This deletion has thus far been observed in only one Chinese subject with Hb H disease. ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830380321
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abstract::Central nervous system (CNS) thrombotic events are a well-known complication of acute lymphoblastic leukemia (ALL) induction therapy, especially with treatments including l-asparaginase (l-ASP). Data on risk factors and clinical evolution is still lacking in adult patients. We report on the clinical evolution of 22 CN...
journal_title:American journal of hematology
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abstract::Recent advances in massively parallel sequencing technologies have provided a detailed picture of the mutational landscape in CLL and underscored the vast degree of interpatient and intratumor heterogeneities. These studies have led to the characterization of novel putative driver genes and recurrently affected biolog...
journal_title:American journal of hematology
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journal_title:American journal of hematology
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更新日期:1989-07-01 00:00:00
abstract::Karyotype is frequently used to predict response and outcome in leukemia. This post hoc exploratory analysis evaluated the relationship between baseline cytogenetics and outcome in patients with relapsed/refractory acute lymphoblastic leukemia (R/R ALL) treated with inotuzumab ozogamicin (InO), a humanized CD22 antibo...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
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abstract::We performed a laparoscopic splenectomy (LS) in 60 patients (age 9-83, 45 females) with idiopathic thrombocytopenic purpura (ITP) who did not achieve sustained remission on steroid therapy. Using a modified procedure, the mean duration of LS was 78 min (range 25-240 min) and surgery was associated with only 5% major a...
journal_title:American journal of hematology
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abstract::Post-transfusion purpura (PTP) with severe thrombocytopenia occurred eight days after transfusion in a 28-year-old woman and responded to treatment with prednisone and plasma exchange. In contrast to nearly all previously studied cases of PTP, the patient's platelets were PlA1-positive and anti-PlA1 antibody could not...
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更新日期:1986-01-01 00:00:00
abstract::In order to study the influence of oxidized low-density lipoprotein (Ox-LDL) on platelet functional morphology at an early activation stage, washed human blood platelets were stimulated by 100 micrograms/ml Ox-LDL at 37 degrees C. The settling and spreading process of stimulated and unstimulated platelets on Formvar-c...
journal_title:American journal of hematology
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journal_title:American journal of hematology
pub_type: 杂志文章
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更新日期:1987-07-01 00:00:00
abstract::Although infectious complications represent a relevant cause of morbidity and mortality in patients with myelofibrosis (MF), little is known about their incidence, outcome and risk factors. We retrospectively evaluated a cohort of 507 MF patients, diagnosed between 1980 and 2014 in five Italian hematology centers, to ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
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更新日期:2017-01-01 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
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更新日期:2011-12-01 00:00:00
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
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更新日期:2016-07-01 00:00:00
abstract::Granulysin is a newly identified CTL/NK cell-related cytotoxic protein, which is secreted in both constitutive and Ca-dependent manner. To evaluate its significance in stem-cell transplantation (SCT), serum granulysin was measured by newly established ELISA method in 26 patients undergoing SCT (21 allogeneic and 5 aut...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20570
更新日期:2006-05-01 00:00:00
abstract::In this descriptive study, all 425 reports were included concerning drug-associated agranulocytosis as registered between 1974 and 1994 in the files of the Drug Safety Unit of the Dutch Inspectorate for Health Care. All reports were analysed as to the probability of agranulocytosis or neutropenia according to previous...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199803)57:3<206::aid-ajh4>
更新日期:1998-03-01 00:00:00
abstract::Chimeric antigen receptors (CARs) can be introduced into T-cells redirecting them to target specific tumor antigens. CAR-modified T cells targeting CD19 have shown remarkable activity against CD19+ malignancies including B cell acute lymphocytic leukemia (ALL), chronic lymphocytic leukemia (CLL), and non-Hodgkin lymph...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24238
更新日期:2016-01-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20949
更新日期:2007-09-01 00:00:00
abstract::Hb Saint Nazaire [beta 103 (G5) Phe-->Ile] was found in four apparently unrelated French families. The five patients carrying this hemoglobin have been detected because of a moderate erythrocytosis. The structural abnormality of Hb Saint Nazaire concerns the same residue as in Hb Heathrow [beta 103 (G5) Phe-->Leu). A ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830440105
更新日期:1993-09-01 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
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更新日期:2015-07-01 00:00:00
abstract::Quantification of sickle hemoglobin (HbS) in patients with sickle cell disease (SCD) undergoing hydroxyurea or chronic transfusion therapy is essential to monitoring the effectiveness of these therapies. The clinical monitoring of %HbS using conventional laboratory methods is limited by high per-test costs and long tu...
journal_title:American journal of hematology
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abstract::Pure red cell aplasia (PRCA) sometimes accompanies thymoma. Herein, we report a PRCA patient with thymoma with a clonal disorder of T cells. A 55-year-old man presented with anemia and anterior mediastinum tumor. The laboratory study revealed hemoglobin 8.2 g/dl; leukocytes 15.8 x 10(9)/L with 76.5% neutrophils, 20.0%...
journal_title:American journal of hematology
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journal_title:American journal of hematology
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更新日期:2012-09-01 00:00:00
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journal_title:American journal of hematology
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更新日期:1988-10-01 00:00:00
abstract::Five adult SS patients from Qatar, Turkey, and South Africa with mild disease, had greatly elevated Hb F and specific patterns of polymorphic sites on their beta S chromosomes. One subject had an alpha-thalassemia (-alpha/-alpha). The haplotypes were the common type #19, associated with severe disease, and type #31, n...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830200313
更新日期:1985-11-01 00:00:00
abstract::Some patients suffering from malignancies may benefit of myeloablative chemotherapy followed by hematological reconstitution with autologous peripheral blood reinfusion. A quick evaluation of the number of hematopoietic progenitors present in leukapheresis blood samples is necessary to ensure the collection of a suffi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830390304
更新日期:1992-03-01 00:00:00
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journal_title:American journal of hematology
pub_type: 杂志文章
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更新日期:1983-08-01 00:00:00
abstract::The treatment of corticosteroid- and/or splenectomy-refractory immune thrombocytopenic purpura (ITP) includes vinca alkaloids, immunosuppressives, Danazol, intravenous gammaglobulin, and alpha-interferon. However, these treatments have often been associated with toxic side effects. Brox et al. (Br J Haematol 70:341-34...
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更新日期:1993-05-01 00:00:00
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journal_title:American journal of hematology
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更新日期:1995-05-01 00:00:00
abstract::A 49-year-old Caucasian woman presented with features suggestive of thrombotic microangiopathy (TMA). She did not respond to treatment with repeated plasma exchange and corticosteroids. A bone marrow biopsy revealed presence of metastatic carcinoma. A limited autopsy revealed presence of breast cancer with rib metasta...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
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更新日期:2007-04-01 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
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更新日期:2013-07-01 00:00:00
abstract::Chronic myeloid leukemia (CML) is considered to be a pleuripotential stem cell disorder with the capacity to differentiate into myeloid, erythroid, megakaryocytic, and lymphoid cell lines. Consequently, blast crisis (BC) involving each of the above lineages has been well described. Among lymphoblastic crises, differen...
journal_title:American journal of hematology
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更新日期:1991-02-01 00:00:00