Abstract：
:Immunoglobulin (Ig) light chain amyloidosis is a clonal but nonproliferative plasma cell disorder in which fragments of an Ig light chain are deposited in tissues. The clinical features depend on the organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, and peripheral/autonomic neuropathy. Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required for diagnosis. Invasive organ biopsy is not required because amyloid deposits can be found in bone marrow biopsy or subcutaneous fat aspirate in 85% of patients. N-terminal pro-brain natriuretic peptide and serum troponin T values are used to classify patients into three groups of approximately equal size; median survivals are 26.4, 10.5, and 3.5 months, respectively. All patients with a visceral amyloid syndrome require therapy to prevent deposition of amyloid in other viscera and to prevent progressive organ failure of involved sites. Stem cell transplant (SCT) is a preferred technique, but only 20% of patients are eligible. Requirements for safe SCT include mild or no cardiac involvement, troponin T value <0.06 ng/mL, age younger than 70 years, <3 organs involved, and serum creatinine value ≤1.7 mg/dL. Nontransplant candidates can be offered melphalan-dexamethasone. Pomalidomide appears to have activity, as do other combinations of chemotherapy with agents such as cyclophosphamide-thalidomide-dexamethasone, bortezomib-dexamethasone, and melphalan-prednisone-lenalidomide. Late diagnosis remains a major obstacle to initiating effective therapy when organ dysfunction is still recoverable. Recognizing the presenting syndromes is necessary for improvement in survival.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Gertz MAdoi
10.1002/ajh.21934subject
Has Abstractpub_date
2011-02-01 00:00:00pages
180-6issue
2eissn
0361-8609issn
1096-8652journal_volume
86pub_type
杂志文章,评审abstract：:Corticosteroids are essential and one of the mainstays in the treatment of acute lymphoblastic leukemia (ALL). In vitro assays show that dexamethasone(DXM) is five to six times more cytotoxic to leukemic lymphoblasts than prednisolone (PDN) [1], and the use of DXM as an alternative drug for PDN is an important issue i...
journal_title：American journal of hematology
pub_type： 信件,随机对照试验
doi：10.1002/ajh.21827
更新日期：2010-10-01 00:00:00
abstract：:Over a 36-month period, 46 consecutive Mexican mestizos with a clinical marker associated with a primary hypercoagulable state were prospectively assessed by searching for the sticky platelet syndrome (SPS), the activated protein C resistance (aPCR) phenotype, coagulation protein C activity and antigen, coagulation pr...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.20233
更新日期：2005-01-01 00:00:00
abstract：:We report on 3 female patients with immunologic thrombocytopenic purpura (ITP) for whom diagnostic procedures evidenced a chronic Hepatitis C virus (HCV) infection. In 2 cases, a transfusion performed more than 10 years ago represented the probable way of contamination. One patient received a course of steroids, which...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/(sici)1096-8652(199804)57:4<338::aid-ajh12
更新日期：1998-04-01 00:00:00
abstract：:Pentosan polysulfate is a low-molecular-weight sulfated polysaccharide used as an antithrombotic drug. We present two patients who developed thrombocytopenia and venous thrombosis during treatment with pentosan polysulfate. The relationship between pentosan polysulfate and thrombocytopenia is supported by platelet agg...
journal_title：American journal of hematology
pub_type： 杂志文章,评审
doi：10.1002/ajh.2830450312
更新日期：1994-03-01 00:00:00
abstract：:Eosinophilia associated with solid tumors is an infrequent occurrence. The pathogenesis of tumor-associated eosinophilia is not well understood. Interleukin-5 (IL-5) is a cytokine that has been implicated in the development of eosinophilia in mice and humans. However, there is little data associating IL-5 production w...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.20789
更新日期：2007-03-01 00:00:00
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. ...
journal_title：American journal of hematology
pub_type： 杂志文章,评审
doi：10.1002/ajh.23756
更新日期：2014-08-01 00:00:00
abstract：:We report a case of T-cell chronic lymphoproliferative disorder (CLPD) that shows neither features of T-cell prolymphocytic leukemia nor disease progression for more than 34 months. Flow cytometric analyses of the lymphocytes revealed high expression of CD4 and CD25. Up-regulation of Foxp3, a master regulatory gene fo...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.20688
更新日期：2006-09-01 00:00:00
abstract：:The relationship between thrombocytopenia and the level of anticardiolipin antibodies (aCL) and/or the existence of lupus anticoagulant (LA) ware studied in 146 patients with systemic lupus erythematosus (SLE). These patients were divided into six groups: A, those LA positive with a high level of aCL (>10 U/ml) (10 ca...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/(sici)1096-8652(199805)58:1<55::aid-ajh10>
更新日期：1998-05-01 00:00:00
abstract：:Celecoxib, a specific cyclooxygenase-2 (Cox-2) inhibitor, has been shown to possess antitumor activity in a variety of cancer cells. However, the antitumor activity of celecoxib in hematopoietic tumors, especially in chronic myeloid leukemia (CML), has not been well established. This study was designed to investigate ...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.20542
更新日期：2006-04-01 00:00:00
abstract：:Twenty-two normal volunteers had approximately eight, 2-hr-long leukapheresis procedures over a 2-year period and their natural killer (NK) cell function was prospectively measured. The NK activity of the preprocedure peripheral blood (pre-PB) was found to correlate well with the NK activity of the inital leukocytes r...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.2830220203
更新日期：1986-06-01 00:00:00
abstract：:The efficacy of the three common intra- and extragenic polymorphic sites of the factor VIII and IX genes has been examined in the Indian population, with an aim to develop a strategy that would be accurate and informative, yet economical. The approach for hemophilia A carrier detection includes tests for BclI, XbaI, a...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/(sici)1096-8652(199704)54:4<271::aid-ajh2>
更新日期：1997-04-01 00:00:00
abstract：:Thrombocytopenia is a poor prognostic indicator in the myelodysplastic syndromes (MDS). Treatment options for patients with symptomatic thrombocytopenia are limited. Danazol, an attenuated androgen, may have some efficacy in increasing the platelet count of patients with MDS. We retrospectively reviewed 33 patients wi...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.10209
更新日期：2002-11-01 00:00:00
abstract：:Skin fibroblasts from patients with a variety of hematologic disorders were infected with SV40 virus in vitro in attempts to discover the reason for increased susceptibility of Fanconi anemia cells to this transforming virus. The proportion of skin fibroblasts expressing SV40 T-antigen by immunofluorescent methods was...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.2830080407
更新日期：1980-01-01 00:00:00
abstract：:Some properties of a monoclonal antibody generated against the fibrinogen component of a factor VIII preparation were investigated. The antibody bound with equal affinity in solid phase radioimmunoassays to fibrinogens isolated from both normal patients and patients with von Willebrand disease. It reacted in a sensiti...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.2830180202
更新日期：1985-02-01 00:00:00
abstract：:The soluble form of Fas (sFas) can block apoptosis induced by the Fas ligand in vitro. A recent report demonstrated that mice injected with sFas displayed autoimmune features. Therefore, an elevated serum concentration of sFas may be associated with lymphoproliferation and autoimmune diseases. We measured the serum co...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/(sici)1096-8652(199808)58:4<334::aid-ajh15
更新日期：1998-08-01 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：:Serum lactic dehydrogenase (LDH) levels are mildly elevated in beta-thalassemia major due to ineffective erythropoiesis. We reviewed the charts of 15 consecutive thalassemic children who underwent allogeneic, T-cell-depleted bone marrow transplantation (BMT) in our department during the last 3 years. Eleven patients h...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/(SICI)1096-8652(199602)51:2<166::AID-AJH13
更新日期：1996-02-01 00:00:00
abstract：:Granulocyte-macrophage colony-stimulating factor (GM-CSF) (250 microg/m2) was administered subcutaneously to 7 normal volunteers for up to 14 days to study its effects on neutrophil kinetics and function. With treatment, blood neutrophil counts rose gradually to peak at 3 1/2 times baseline by day 14. At day 5 marrow ...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/(sici)1096-8652(199801)57:1<7::aid-ajh2>3.
更新日期：1998-01-01 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：:How often elevated serum ferritin in primary-care patients reflects increased iron stores (normally 0.8 g in men, 0.4 g in women) is not known. The Hereditary Hemochromatosis and Iron Overload Screening (HEIRS) study screened 101,168 primary-care participants (44% Caucasians, 27% African-Americans, 14% Asians/Pacific ...
journal_title：American journal of hematology
pub_type： 杂志文章,多中心研究
doi：10.1002/ajh.21179
更新日期：2008-08-01 00:00:00
abstract：:Relapsed or refractory acute myeloid leukemia (R/R AML) has a poor prognosis and is best treated with salvage chemotherapy as a bridge to allogeneic stem cell transplant (alloSCT). However, the optimal salvage therapy remains unknown. Here we compared two salvage regimens; mitoxantrone, etoposide, and cytarabine (MEC)...
journal_title：American journal of hematology
pub_type： 杂志文章,多中心研究
doi：10.1002/ajh.25838
更新日期：2020-08-01 00:00:00
abstract：:Solid-phase enzyme immunoassays using recombinant gag and env proteins were developed to study humoral immune responses to HIV infection in a cohort of 105 hemophiliac patients. Thirteen patients with ARC or AIDS and 92 asymptomatic patients were studied. A cross-sectional study showed a wide range of antibody respons...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.2830360108
更新日期：1991-01-01 00:00:00
abstract：:The increased adhesiveness of sickle erythrocytes (SS RBC) to endothelial cells has been confirmed in a static system utilizing fresh umbilical vein endothelium. Adherence of SS RBC to the endothelium was as great in the presence of calcium-containing buffer as when incubated in plasma. SS RBC suspended in autologous ...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.2830340110
更新日期：1990-05-01 00:00:00
abstract：:In 2010, the Food and Drug Administration (FDA) added a black box warning to anti-D immune globulin (Rho(D) immune globulin, anti-D) for immune thrombocytopenia (ITP) to warn of the complications related to severe hemolysis. The objective of this retrospective medical record review was to examine recent trends in anti...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.22261
更新日期：2012-03-01 00:00:00
abstract：:Nucleotide sequence analysis of the 5' beta-globin gene flanking region has been carried out for numerous homozygous beta-thalassemia patients with different mutations and of various ethnic backgrounds. Four different rearrangements were found associated with numerous beta-thalassemia mutations. The (AT)X(T)Y repeat m...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.2830450108
更新日期：1994-01-01 00:00:00
abstract：:Ticlopidine-induced aplastic anemia (TIAA) is considered very uncommon. We present two new cases, and we review 55 additional cases from the literature. The first case concerns a 70-year-old man who developed severe aplastic anemia 7 weeks after treatment with 500 mg of ticlopidine daily. The patient sustained a sever...
journal_title：American journal of hematology
pub_type： 杂志文章,meta分析,评审
doi：10.1002/ajh.10150
更新日期：2002-09-01 00:00:00
abstract：:Cobalamin (vitamin B12) deficiency is a common cause of megaloblastic anemia in Western populations. Laboratory evaluation of megaloblastic anemia frequently includes the assessment of patient cobalamin and folate status. Current total serum cobalamin measurements are performed in the clinical laboratory with competit...
journal_title：American journal of hematology
pub_type： 杂志文章,评审
doi：10.1002/ajh.23421
更新日期：2013-06-01 00:00:00
abstract：:Compared to age-matched controls, cancer patients have increased risk of bleeding when treated with anticoagulation. However, there are little data regarding bleeding as it relates to anticoagulant choice and other risk factors. We evaluated the six-month incidence of bleeding among patients treated with anticoagulati...
journal_title：American journal of hematology
pub_type： 杂志文章
doi：10.1002/ajh.25494
更新日期：2019-07-01 00:00:00
abstract：:Fourteen patients, aged 65-85 years, with refractory (11) or relapsing (3) multiple myeloma were treated with a "protracted-sequential" protocol comprising vincristine 1-2 mg or vindesine 3 mg/M(2) (max. 5 mg) IVI over 4 hr on D1, prednisolone 40-50 mg PO D1-14, and melphalan 2-4 mg PO or cyclophosphamide 50-100 mg PO...
journal_title：American journal of hematology
pub_type： 临床试验,杂志文章
doi：10.1002/1096-8652(200009)65:1<81::aid-ajh15>3.0.co
更新日期：2000-09-01 00:00:00
abstract：:Cobalamin derivatives serve as coenzymes for the body's two cobalamin-dependent enzymes--adenosylcobalamin-dependent methylmalonyl CoA mutase, and methylcobalamin-dependent methionine methyltransferase. This essay reviews, in brief form and in personal terms, the history, beginning in the mid-1950s, of how these enzym...
journal_title：American journal of hematology
pub_type： 杂志文章,评审
doi：10.1002/ajh.2830340203
更新日期：1990-06-01 00:00:00
