Abstract:
OBJECTIVE:To assess the efficacy of intravenous immunoglobulin (IVIG), in comparison with plasma exchange (PE), in the treatment of patients with thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS). DESIGN:Prospective, nonrandomized comparative study. SETTING:Hematology department in a general hospital. PATIENTS:17 consecutive adult patients, six of them pregnant, with diagnosis of TTP/HUS. Three had a severity score at diagnosis less than or equal to 4 and were treated with IVIG and 14 had a severity score of greater than or equal to 5 and/or were pregnant and received PE. The response was evaluated after 5 days of therapy. RESULTS:Complete remission was obtained in 0/3 cases treated with IVIG and 10/14 (71%) with PE (Fisher's exact test P = 0.05). Three patients died for widespread TTP-HUS, and four had persistent disease. In three of the four resistant patients, complete remission was obtained by further PE but not by further IVIG. The overall remission rate was 76% (13/17). CONCLUSIONS:Our study does not confirm the utility of IVIG in the management of TTP-HUS, as suggested by earlier single case reports.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Finazzi G,Bellavita P,Falanga A,Viero P,Barbui Tdoi
10.1002/ajh.2830410305keywords:
subject
Has Abstractpub_date
1992-11-01 00:00:00pages
165-9issue
3eissn
0361-8609issn
1096-8652journal_volume
41pub_type
杂志文章abstract::In patients with acute myelogenous leukemia (AML), 34 out of 35 patients (97%) had cytochemically abnormal polymorphonuclear neutrophils (PMN). Some interesting regularity was noted in the appearance of cytochemical abnormalities of the PMNs. When peroxidase (PO) activity was low, both alkaline phosphatase (AP) and ch...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830150106
更新日期:1983-08-01 00:00:00
abstract::The clinical features of patients with splenic marginal zone cell lymphoma (SMZCL) have rarely been reported. In the present study, immunological abnormalities, particularly hematological abnormalities, observed in SMZCL were described. Autoimmune hemolytic anemia, immune thrombocytopenia, and appearance of lupus anti...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/(sici)1096-8652(199711)56:3<173::aid-ajh7>
更新日期:1997-11-01 00:00:00
abstract::Reduced ferrochelatase activity in erythropoietic protoporphyria (EPP) causes the accumulation of protoporphyrin IX (PPIX) leading to acute cutaneous photosensitivity and liver injury. Many EPP patients also have a mild hypochromic, microcytic anemia and iron deficiency. Iron deficiency can lead to decreased PPIX accu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25743
更新日期:2020-05-01 00:00:00
abstract::Secondary leukaemia following treatment of M3 acute promyelocytic leukaemia (APL) is a rare event. We describe a patient in remission following chemotherapy for APL who relapsed with M2 acute non-lymphoblastic leukaemia (ANLL). The original t(15;17) (q22;q21) chromosome translocation was lost and replaced by a clone c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830480108
更新日期:1995-01-01 00:00:00
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
pub_type: 杂志文章
doi:10.1002/ajh.10442
更新日期:2004-02-01 00:00:00
abstract::Little is known about the mechanisms explaining the wide variation in platelet counts (PLT) and other hematologic parameters in humans. We previously showed that the sex-based difference in hematocrit was associated with nucleotide variation in the erythropoietin receptor gene (EPOR). We sought to identify new polymor...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20095
更新日期:2004-09-01 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::Plasma cell dyscrasias are a group of clinically and biochemically diverse disorders of unknown etiology, characterized by the disproportionate proliferation of one or more clones of B cells, and the presence of a structurally and electrophoretically homogeneous (monoclonal) immunoglobulin or polypeptide subunit in se...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20736
更新日期:2006-12-01 00:00:00
abstract::This analysis, using data from the bortezomib-melphalan-prednisone (VMP) arm of the Phase III VISTA study, investigated whether increased cumulative bortezomib dose could improve overall survival (OS) in transplant-ineligible patients with previously untreated multiple myeloma. Median cumulative bortezomib dose receiv...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ajh.23933
更新日期:2015-04-01 00:00:00
abstract::Autoantibody production following allogeneic stem-cell transplantation is common and is often ascribed to the immune dysregulation associated with graft-versus-host disease. Recent data suggests that donor-memory B cells can be reactivated on exposure to antigen and result in antibody production in the recipient ident...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20350
更新日期:2005-08-01 00:00:00
abstract::Responsiveness of bone marrow erythropoietic stem cells (CFU-E and BFU-E) to recombinant human erythropoietin (rh-Ep) was examined in vitro in 23 patients with aplastic anemia and 14 with myelodysplastic syndrome (MDS) to investigate the clinical use of rh-Ep for these diseases. Bone marrow mononuclear cells were cult...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830350103
更新日期:1990-09-01 00:00:00
abstract::Cultured endothelial cells from bovine aorta were exposed to oxidized low density lipoprotein and examined by electron microscopy. The endothelial cells contracted slightly and the intercellular junctions became unclear. Some osmiophilic material increased in the cytoplasm. The oxidized low density lipoprotein appears...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490315
更新日期:1995-07-01 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::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
abstract::Substantial evidence exists indicating T cell abnormalities in chronic lymphocytic leukemia (CLL). There is also evidence that the T cell is an important source of burst promoting activity (BPA) for the peripheral blood (PB) erythroid burst forming unit (BFU-e). We studied the BPA of T cells and response of BFU-e in n...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830160207
更新日期:1984-02-01 00:00:00
abstract::A case of red cell adenosine deaminase (ADA) overproduction associated with hereditary hemolytic anemia is reported here. This appears to be the second report. Proband is a 38-year-old Japanese male who had hemoglobin, 15.8 g/100 ml; reticulocyte count, 4.5%; serum indirect bilirubin, 4.9 mg/100 ml; 51Cr-labeled red c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830050205
更新日期:1978-01-01 00:00:00
abstract::We report a family with a dominantly transmitted syndrome resembling Fanconi's anemia and spanning two generations. This syndrome was characterized by an ill-defined hematologic stem cell disorder, immune dysfunction, poor dentition, hyperpigmented skin, warts, and multiple second trimester spontaneous abortions and i...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830320402
更新日期:1989-12-01 00:00:00
abstract::A variability in DNA content detected was found with image cytometry, in immature bone marrow cells from 13 healthy donors (median age 31 yr). The mean coefficient of variation (C.V.) of the DNA content was found to be significantly (P = 0.0002) higher in immature blasts and promyelocytes than in mature granulocytes, ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830430412
更新日期:1993-08-01 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::Several syndromes characterized by striking eosinophilia may be complicated by thrombosis. The experiments described indicate that, paradoxically, eosinophils and certain of their constituents inhibit the activation of Hageman factor (HF, factor XII). In earlier studies, suspensions of mixed types of granulocytes, oth...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830420127
更新日期:1993-01-01 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::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::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
doi:10.1002/ajh.20783
更新日期:2007-04-01 00:00:00
abstract::During 1971-1988, 194 adults with de novo acute myeloid leukemia (AML) received initial therapy at the University of Minnesota with an anthracycline-based regimen. Seventy-two of the 194 required further chemotherapy and received a second cycle of the same or similar therapy; 63 of these 72 were evaluable. For each ma...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470305
更新日期:1994-11-01 00:00:00
abstract::Progressive multifocal leukoencephalopathy (PML), a demyelinating infectious disease caused by JC virus (JCV), occurs almost exclusively in immunocompromised patients usually with malignant diseases. We report here a Japanese female with follicular lymphoma who subsequently developed PML. In addition to JCV, human her...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.1108
更新日期:2001-07-01 00:00:00
abstract::Argatroban is a direct thrombin inhibitor approved for the treatment of heparin-induced thrombocytopenia (HIT) type II. Argatroban is predominantly metabolized in the liver. It is widely believed that no dosage adjustment is required in patients with renal insufficiency, making it a preferred agent in patients on rena...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21072
更新日期:2008-03-01 00:00:00
abstract::Leukemic cells from the peripheral blood of 52 patients with acute and chronic leukemias were incubated with 12-0-tetradecanoyl phorbol ester (TPA). Thirty-one cases of lymphocytic leukemia (18 cases of acute lymphoblastic and 13 cases of chronic lymphocytic leukemia), 13 cases of acute nonlymphoblastic (myelo or myel...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830130303
更新日期:1982-11-01 00:00:00
abstract::Hydroxyurea (HU) is a widely used cytotoxic agent that is known to induce fetal hemoglobin (HbF) production and is presently used to ameliorate the severity of pain episodes in patients with sickle cell anemia (HbSS). Previously we have shown that HU inhibits growth of burst forming unit-erythroid (BFU-E) colonies in ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200011)65:3<227::aid-ajh9>3.0.co
更新日期:2000-11-01 00:00:00
abstract::A rare beta-thalassemia mutation at the splicing junction [namely, G-->C in intervening sequence (IVS) I-1] was found in a Japanese family. The proband and his mother were heterozygous for the mutation. Analysis of mRNA extracted from the reticulocyte-rich fraction obtained from the proband's mother revealed that the ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10244
更新日期:2003-01-01 00:00:00
abstract::Six patients with thrombotic microangiopathy associated with drug therapy had serial analyses of von Willebrand factor (vWF) multimeric patterns in their EDTA-plasma samples by sodium dodecyl sulfate-1% agarose gel electrophoresis and autoradiography. In the plasma of five patients (one with chronic myelogenous leukem...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830420306
更新日期:1993-03-01 00:00:00