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 cells in bone marrow confirms the diagnosis. RISK STRATIFICATION:Age, hemoglobin level, platelet count, b2-microglobulin, and monoclonal IgM concentrations are characteristics required for prognosis. RISK ADAPTED THERAPY:Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Rituximab-based therapy is used in virtually all US patients with WM and can be combined with alkylating agent or purine nucleoside analogue, or both. The preferred Mayo Clinic nonstudy therapeutic induction is rituximab, cyclophosphamide, and dexamethasone. Future stem cell transplantation should be considered in induction therapy selection. MANAGEMENT OF REFRACTORY DISEASE:Bortezomib, thalidomide, lenalidomide, and bendamustine have all been shown to have activity in WM. Given WM’s natural history, reduction of complications will be a priority for future treatment trials.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Gertz MAdoi
10.1002/ajh.22014subject
Has Abstractpub_date
2011-05-01 00:00:00pages
411-6issue
5eissn
0361-8609issn
1096-8652journal_volume
86pub_type
杂志文章,评审abstract::In some cases, bone marrow aplasia has been thought to result from immunologic abnormalities. Our patient had severe transfusion-dependent aplastic anemia, which responded to treatment with prednisone on two occasions. The exacerbations of aplastic anemia were associated with lymphocytic proliferation which on one occ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830110310
更新日期:1981-11-01 00:00:00
abstract::We report here a patient with de novo acute myelocytic leukemia with trilineage myelodysplasia (AML/TMDS) in whom cytogenetic analysis was normal at diagnosis and in whom Philadelphia chromosome (Ph1) with monosomy 7 emerged at the terminal stage of the disease. Reverse transcription polymerse chain reaction (RT-PCTR)...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490413
更新日期:1995-08-01 00:00:00
abstract::By virtue of their religious principles, Jehovah's Witnesses (JWs) generally object to receiving blood products, raising numerous ethical, legal, and medical challenges for providers who care for these patients, especially in the emergent setting. In this review, we discuss several areas relevant to the care of JWs, i...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24889
更新日期:2017-12-01 00:00:00
abstract::Three Indonesian patients with identical genotypes, each compound heterozygotes for Filipino beta(o)-thalassemia/HbE, expressed different clinical severities. One patient has mild disease and is transfusion independent, while the other two are severely affected and transfusion dependent. The size of the Filipino beta(...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199909)62:1<7::aid-ajh2>3.
更新日期:1999-09-01 00:00:00
abstract::Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830310105
更新日期:1989-05-01 00:00:00
abstract::The lupus anticoagulant may be accompanied by an acquired factor II deficiency and bleeding. We report on a patient with a lupus anticoagulant and factor II (Fll) deficiency responsive to Danazol. Acquired hypoprothrombinemia (FII) with the lupus anticoagulant (LA) may be accompanied by a hemorrhagic diathesis. A 64-y...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199612)53:4<272::AID-AJH14
更新日期:1996-12-01 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::The biosynthesis of the minor hemoglobin FIc, which contains acetylated gamma chains, and the major hemoglobin Fo was studied during erythroid cell differentiation and maturation in cultures of erythroid precursors isolated from five human umbilical cord blood samples. A gradual decrease in the synthesis of Hb FIc was...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120302
更新日期:1982-05-01 00:00:00
abstract::There is a high prevalence of thalassemia in the Taiwan area. Prenatal diagnosis of severe forms of thalassemia is important for the prevention of this disease. We performed prenatal diagnosis in 167 cases, of which 59 cases were diagnosed by chorionic villi biopsy, 91 cases by amniotic fluid analysis, and 17 cases by...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199706)55:2<65::aid-ajh3>3
更新日期:1997-06-01 00:00:00
abstract::FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been i...
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.25945
更新日期:2020-11-01 00:00:00
abstract::Lentiviral modification combined with ex vivo erythroid differentiation was used to stably inhibit RhAG expression, a critical component of the Rh(rhesus) membrane complex defective in the Rh(null) syndrome. The cultured red cells generated recapitulate the major alterations of native Rh(null) cells regarding antigen ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23414
更新日期:2013-05-01 00:00:00
abstract::The pharmacokinetics of factor VIII were studied in a series of 20 hemophilia-A patients undergoing surgery. Regardless of the type of operation, elimination of factor VIII was shown to be increased only in ten cases (50%) during the post-operative period. In this subgroup of patients, factor VIII half-life, measured ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830300305
更新日期:1989-03-01 00:00:00
abstract::The occurrence of factor VIII inhibitors in non-hemophilic patients is a rare event with a potentially lethal outcome. Despite its infrequent occurrence, the association of this inhibitor with multiple autoimmune diseases is well recognized. We report the case of a patient with the recently described autoimmune lympho...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200007)64:3<214::aid-ajh14>3.0.c
更新日期:2000-07-01 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::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
pub_type: 杂志文章
doi:10.1002/ajh.23307
更新日期:2012-11-01 00:00:00
abstract::The Sickle Cell Disease Clinical Research Network (SCDCRN) designed the PROACTIVE Feasibility Study (ClinicalTrials.gov NCT00951808) to determine whether elevated serum levels of secretory phospholipase A2 (sPLA2) during hospitalization for pain would permit preemptive therapy of sickle cell acute chest syndrome (ACS)...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22265
更新日期:2012-03-01 00:00:00
abstract::One of the more intriguing aspects of the spleen is the protection against certain bacterial infections afforded by its unique vascular and immune function. There have been extensive clinical surveys which indicate an incidence of overwhelming postsplenectomy infection (OPSI) above that of the disease for which the sp...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830020210
更新日期:1977-01-01 00:00:00
abstract::Everolimus is an oral antineoplastic agent that targets the raptor mammalian target of rapamycin (mTORC1). The phosphatidylinositol 3-kinase/mTOR signal transduction pathway has been demonstrated to be activated in tumor samples from patients with Hodgkin lymphoma (HL). The goal of this trial was to learn the antitumo...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21664
更新日期:2010-05-01 00:00:00
abstract::Mantle cell lymphoma (MCL) is a unique type of lymphoma with a prognosis intermediate between indolent and aggressive types. The purpose of this study was to study blood cytokine levels in newly diagnosed and relapsed MCL patients with respect to patterns of abnormalities and relationship to the MCL International Prog...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23838
更新日期:2014-12-01 00:00:00
abstract::Fibrillar granules (FGs) represent neutrophilic primary granules containing clustered filaments. We investigated neutrophils in the bone marrow obtained from 17 patients with chronic myeloproliferative disorders (CMPD) by electron microscopy. FG-positive neutrophils were seen in 15 of the 17 CMPDs with varying frequen...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470316
更新日期:1994-11-01 00:00:00
abstract::The improved microchromatographic procedure for the detection of Hb-S and/or Hb-C in cord blood at birth (Schroeder et al.: J. Lab Clin Med 86:528-532, 1975) as well as a modification thereof may also be used for the quantitative determination of Hb-F in the presence of Hb-S and/or Hb-C. However, Hb-A interferes and m...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830010308
更新日期:1976-01-01 00:00:00
abstract::Reversible posterior leukoencephalopathy syndrome (RPLS) is an uncommon but distinctive clinicoradiological entity comprising of headache, seizures, visual disturbance, and altered mental function, in association with posterior cerebral white matter edema. With appropriate management, RPLS is reversible in the majorit...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20147
更新日期:2004-09-01 00:00:00
abstract::We have previously shown that humic acid (well-water humic acid, HA, and synthetic humic acid, SHA) enhances cell surface expression of tissue factor (TF). Here we report that incubation of human umbilical vein endothelial cells (HUVEC) for 2 hr with HA or SHA cause a rapid rise in TF mRNA levels, as shown by Northern...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199603)51:3<200::AID-AJH4>
更新日期:1996-03-01 00:00:00
abstract::Achievement of a complete response has been associated with improved outcomes in patients with multiple myeloma. Recently, increasing application of minimal residual disease (MRD) assessment has shown that MRD negativity is a powerful prognostic factor for survival outcomes. We wanted to examine the impact of the poly...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.25481
更新日期:2019-07-01 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::We analyzed 12 MLL/ENL positive ALL patients consecutively diagnosed between 1999 and 2009. The MLL/ENL fusion was identified in 4/150 (2.6%), 8/993 (0.8%), and 0/70 of pediatric, adult, and elderly patients, respectively. Eight patients had a WBC count >50 × 10(9) /L. Ten cases had an evaluable immunophenotyping. A B...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22161
更新日期:2011-12-01 00:00:00
abstract::A 74-year old woman with Ph1-negative chronic myelogenous leukemia (CML) and heterozygous for glucose-6-phosphate dehydrogenase (G6PD) was studied. Both A and B types of G6PD were found in skin. In contrast, white blood cells and platelets showed only a single G6PD type A. These results provide further evidence that P...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830170113
更新日期:1984-07-01 00:00:00
abstract::Current information about clinical significance of IDH mutations in myelodysplastic syndromes (MDS), their association with other genetic alterations and the stability during disease progression is limited. In this study, IDH mutations were identified in 4.6% of 477 patients with MDS based on the FAB classification an...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23596
更新日期:2014-02-01 00:00:00
abstract::We analyzed the genetic defects of 21 unrelated patients with venous thrombosis in whom hereditary protein C deficiency was diagnosed. Eleven mutations were detected in 18 families, while no mutation was detectable in the other three families. Among these mutations, a common genetic mutation of protein C (PROC) gene r...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20043
更新日期:2004-05-01 00:00:00
abstract::Serologic diagnosis for Epstein-Barr virus (EBV) infection is problematic when patients receive exogenous immunoglobulin. We recently diagnosed primary EBV infection by detecting EBV-determined nuclear antigen (EBNA) and EBV-DNA in peripheral blood mononuclear cells (PBMC) using immunofluorescence, in situ hybridizati...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830360416
更新日期:1991-04-01 00:00:00