Abstract:
:We report a patient with incapacitating POEMS syndrome characterized by serum monoclonal protein, polyneuropathy, organomegaly, endocrinopathy, mesangiocapillary glomerulonephritis, massive ascites formation, and pulmonary hypertension. A dramatic improvement in the clinical condition occurred after administration of thalidomide, a drug with known anti-angiogenetic, anti-proliferative, and anti-cytokine properties.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Sinisalo M,Hietaharju A,Sauranen J,Wirta Odoi
10.1002/ajh.20051keywords:
subject
Has Abstractpub_date
2004-05-01 00:00:00pages
66-8issue
1eissn
0361-8609issn
1096-8652journal_volume
76pub_type
杂志文章abstract::The levels of adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), lactic dehydrogenase (LDH), and LDH isoenzyme patterns (LD1 to LD5) have been measured in lymphocyte extract from 28 patients with B-chronic lymphocytic leukemia (B-CLL). The activities of ADA, PNP, and LDH have been correlated with two mo...
journal_title:American journal of hematology
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abstract::Red blood cells from patients with sickle cell disease (SS RBC) adhere to laminin and over-express the high-affinity laminin receptor basal cell adhesion molecule/Lutheran protein (B-CAM/LU). This receptor has recently been shown to undergo activation in vitro through a protein kinase A-dependent mechanism. Low-densit...
journal_title:American journal of hematology
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doi:10.1002/ajh.10442
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abstract::Acute myeloid leukemia (AML) is defined as ≥20% myeloblasts, representing a change from original guidelines where ≤30% blasts were considered as myelodysplastic syndromes (MDS), and 20-29% blasts classified as refractory anemia with excess blasts in transformation (RAEB-T). Whether the diagnostic bone marrow blast per...
journal_title:American journal of hematology
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abstract::We investigated the rare case of a patient with IgGlambda multiple myeloma for whom both prothrombin time and APTT were significantly prolonged. The IgG inhibited coagulation reactions upstream from prothrombin when coagulation was initiated by mRVVT, but not by FXa, as indicated by a chromogenic substrate for FXa. Th...
journal_title:American journal of hematology
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doi:10.1002/ajh.10449
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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
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更新日期:2011-04-01 00:00:00
abstract::HIV infection has been associated with an increased risk of developing several types of malignancies, including aggressive peripheral T-cell lymphomas (PTCL). However, this is a rare occurrence with no more than a hundred cases reported in the literature. The purpose of this multicenter study is to describe the charac...
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:2011-03-01 00:00:00
abstract::A discordance between p53 protein overexpression and the presence of mutations in the gene has been observed in many types of tumors, including human lymphomas. To probe this finding, we have studied a large series of 94 lymphomas of different pathologic types and histologic differentiation. Analyzing exons 5-9, we ha...
journal_title:American journal of hematology
pub_type: 杂志文章
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更新日期:1997-05-01 00:00:00
abstract::Immunoglobulin light-chain amyloidosis (AL) is a rare disorder characterized by production of a monoclonal light chain. This insoluble light chain, or a fragment thereof, deposits in tissues as amyloid and results in disruption of organ function and, ultimately, in death. Although melphalan and prednisone are benefici...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
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abstract::We experienced two rare cases of pernicious anemia that presented in the course of mycosis fungoides in elderly males. Pernicious anemia has recently been reported to be caused by autoimmune gastritis that produces autoantibodies to gastric parietal cells and intrinsic factor. Immunological abnormalities in mycosis fu...
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更新日期:1998-06-01 00:00:00
abstract::Patients presenting with Hodgkin's disease (HD) may show lung involvement characterized by contiguous spread from ipsilateral hilar lymph nodes. Lung consolidation or noncontiguous pulmonary involvement makes an alternative diagnosis more likely. This report describes a patient with HD in whom concurrent pulmonary bla...
journal_title:American journal of hematology
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doi:10.1002/ajh.2830430211
更新日期:1993-06-01 00:00:00
abstract::Immune dysregulation and altered T-cell hemostasis play important roles in the pathogenesis of myelodysplastic syndromes (MDS). Recent studies suggest an increased risk of MDS among patients with autoimmune diseases. Here, we investigated the prevalence of autoimmune diseases among MDS patients, comparing characterist...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24333
更新日期:2016-05-01 00:00:00
abstract:DISEASE OVERVIEW:Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS), now classified under myelodysplastic syndromes with RS (MDS-RS) and RARS with thromboc...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24637
更新日期:2017-03-01 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
pub_type: 杂志文章
doi:10.1002/ajh.23980
更新日期:2015-06-01 00:00:00
abstract::The value of menorrhagia as a predictor for mild bleeding disorders has been very little studied and the results are divergent. In the present study on 30 women with objectively verified menorrhagia, we found a significantly increased prevalence of von Willebrand's disease (20%). By keeping a strict sampling and labor...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199612)53:4<234::AID-AJH4>
更新日期:1996-12-01 00:00:00
abstract::A 36-year-old white man was found to have low erythrocyte acetylcholinesterase activity. Plasma cholinesterase activity was normal. The propositus had mild anemia and moderate elliptocytosis, but was asymptomatic. A sister, brother, father, and nephew were hematologically normal, but had slightly subnormal red cell ac...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830070403
更新日期:1979-01-01 00:00:00
abstract::Four factor XI (F XI)-deficient patients are described, all of whom formed circulating anticoagulants against F X1. In the three most severely affected patients (F XI 0%-6% activity), the anticoagulant appeared to have been stimulated by plasma infusion. However, in the milder case (25% F XI activity), no infusion had...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830260405
更新日期:1987-12-01 00:00:00
abstract::A case of a patient presenting with idiopathic concurrent erythrocytic and megakaryocytic aplasia is reported. The patient's response to immunosuppressive therapy and her bone marrow pathology clearly suggest an immune mechanism. Based on the lack of suppression of erythroid colony growth, several mechanisms are postu...
journal_title:American journal of hematology
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doi:10.1002/(SICI)1096-8652(199601)51:1<68::AID-AJH11>
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abstract::Acute lymphoblastic leukemia (ALL) with distinct fusion transcripts has unique clinical features. In this study, the incidence, clinical characteristics, early treatment response, and outcomes of 1,004 Chinese pediatric ALLs were analyzed. Patients with TEL-AML1 and E2A-PBX1 fusion genes or other B cell precursor ALLs...
journal_title:American journal of hematology
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journal_title:American journal of hematology
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更新日期:1994-06-01 00:00:00
abstract::The 2013 discovery of calreticulin (CALR) mutations in myeloproliferative neoplasms was attended by their association with longer survival in primary myelofibrosis (PMF). Subsequent studies have suggested prognostic distinction between type 1/like and type 2/like CALR mutations and detrimental effect from triple-negat...
journal_title:American journal of hematology
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更新日期:2018-03-01 00:00:00
abstract::We aimed to comprehensively study sex differences in disease and patients' characteristics, treatment and outcomes in patients with follicular lymphoma (FL) in the United States (USA) utilizing the National LymphoCare Study registry (2004-2014). Among evaluable males (n = 1277) and females (n = 1375) with FL, females ...
journal_title:American journal of hematology
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doi:10.1002/ajh.24401
更新日期:2016-08-01 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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abstract::ASXL1 and SRSF2 mutations in AML are frequently found in patients with preexisting myeloid malignancies and are individually associated with poor outcomes. In this multi-institutional retrospective analysis, we assessed the genetic features and clinical outcomes of 43 patients with ASXL1mut SRSF2mut AML and compared o...
journal_title:American journal of hematology
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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...
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abstract::Autologous stem cell transplantation (ASCT) is feasible and effective in selected older patients with Multiple Myeloma, but specific criteria for evaluating ASCT eligibility in elderly patients are lacking. We evaluated 131 patients aged 65-75 considered for ASCT at our center: The Charlson Comorbidity Index (CCI), He...
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abstract::Thrombocytopenia developing in the course of chemotherapy for malignant disease is usually attributed to drug-induced marrow suppression and/or marrow replacement by tumor. We describe two patients who developed severe thrombocytopenia and hemorrhagic symptoms while being treated with oxaliplatin, 5-fluorouracil, and ...
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abstract::Factor V G1691A (FV-Leiden) and prothrombin G20210A mutations are major inherited risk factors for venous thrombosis. Recently, it was suggested that both mutations, through stimulation of venous and placental thrombosis events, were strongly associated with recurrent idiopathic miscarriages, although other studies di...
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