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 summarized. Study of the clinical consequences of these genetically determined membrane glycoprotein defects has also contributed to the understanding of the role of normal neutrophils in both host defense and host tissue damage.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Styrt Bdoi
10.1002/ajh.2830310416subject
Has Abstractpub_date
1989-08-01 00:00:00pages
288-97issue
4eissn
0361-8609issn
1096-8652journal_volume
31pub_type
杂志文章,评审abstract::A 50-year-old woman with muscular fatigue and monoclonal gammopathy of unknown significance was shown to have high levels of antibodies against the acetylcholine receptor of the skeletal muscle endplate. The specific antibody activity was exclusively associated with the monoclonal component. There were no objective si...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830290112
更新日期:1988-09-01 00:00:00
abstract::Splenectomy is a time-honoured well established approach for patients with steroid-resistant immune thrombocytopenia (ITP). However, due to the more recent availability of therapeutic options alternative to splenectomy, such as rituximab and agonists of the thrombopoietin-receptor, the choice of second-line therapy is...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23823
更新日期:2014-11-01 00:00:00
abstract::A 77-year-old man with relapsed non-Hodgkin's lymphoma, diffuse large B-cell type, was treated with naproxen, a nonsteroidal anti-inflammatory drug (NSAID), for paraneoplastic fever. A dramatic disappearance of not only the fever but also generalized lymphadenopathy was observed. Naproxen was continued, and he maintai...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200103)66:3<220::aid-ajh1048>3.0
更新日期:2001-03-01 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::Thromboembolic complications following splenectomy for hematologic diseases occur in up to 10% of patients and may range from portal vein thrombosis (PVT) to pulmonary embolism (PE) and deep vein thrombosis (DVT). Up to now there exist no recommendations for the duration and intensity of prophylactic anticoagulation, ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20018
更新日期:2004-06-01 00:00:00
abstract::Aplastic anemia is a disorder characterized by pancytopenia and bone marrow hypocellularity. There is some evidence that aplastic anemia may be due to suppression of hematopoiesis by activated T-suppressor cells. Thus, immunosuppressive agents have been used as an alternative to bone marrow transplantation for treatme...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199606)52:2<108::AID-AJH7>
更新日期:1996-06-01 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::This study concerned the gamma chain composition of Hb F and the haplotypes of 44 patients with beta-thalassemia major or intermedia and many of their relatives. Seventeen patients came from Northern (Turkish) Cyprus, 12 from the Istanbul area, and 15 from Macedonia and Bulgaria. Analysis of the A gamma T-G gamma-A ga...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830200103
更新日期:1985-09-01 00:00:00
abstract::Long-term survival following chemotherapy or autologous bone marrow transplantation in adults with relapsed/refractory non-Hodgkin's lymphoma was evaluated. English language articles published from January 1, 1988 to September 1, 1993 were obtained from a broad-based MEDLINE search retrieving 3,854 citations regarding...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830500208
更新日期:1995-10-01 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::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::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::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
pub_type: 杂志文章
doi:10.1002/ajh.2830270302
更新日期:1988-03-01 00:00:00
abstract::Intravenous immunoglobulin (IVIG) therapy is associated with rare reports of thromboembolic events and severe hyponatremia. We hypothesized that IVIG therapy may result in hyperproteinemia, increased serum viscosity, and pseudohyponatremia. We conducted a prospective observational study to evaluate the incidence of hy...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10325
更新日期:2003-06-01 00:00:00
abstract::Withdrawal: Primary prevention of sickle cell disease using preimplantation genetic testing and in vitro fertilization is cost-effective. The above article, published online on 20 August 2020 in Wiley Online Library (wileyonlinelibrary.com) as an Accepted Article, has been withdrawn by agreement between the authors, t...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25974
更新日期:2020-08-20 00:00:00
abstract::In a pilot study to reduce the duration of treatment and potential long-term toxicities, 39 patients with acute promyelocytic leukemia in remission received a single cycle of intensive consolidation therapy, followed by intermittent ATRA maintenance. Consolidation therapy required prolonged hospitalization and was ass...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20354
更新日期:2005-06-01 00:00:00
abstract::Recent observations that the sickle RBC are excessively susceptible to phagocytosis by macrophages in vitro prompted me to look for evidence of in vivo erythrophagocytosis (Ep) in patients with sickle cell anemia (SS). Freshly prepared smears of unmanipulated blood of 27 patients with SS in steady state were examined ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830200406
更新日期:1985-12-01 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::Identification of genetic causes of neutropenia informs precision medicine approaches to medical management and treatment. Accurate diagnosis of genetic neutropenia disorders informs treatment options, enables risk stratification, cancer surveillance, and attention to associated medical complications. The rapidly expa...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25374
更新日期:2019-03-01 00:00:00
abstract::After the patents of biopharmaceuticals have expired, based on specific regulatory approval pathways copied products ("biosimilars" or "follow-on biologics") have been launched in the EU. This article summarizes experiences with hematopoietic medicines, namely the epoetins (two biosimilars traded under five different ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21805
更新日期:2010-10-01 00:00:00
abstract::Iron studies were compared in 434 patients from 80 hemochromatosis families classified as putative homozygotes, heterozygotes, and normal by HLA typing. There were 28 of 255 (11%) heterozygotes with an elevated serum ferritin and 22 of 255 (8.6%) with an elevated transferrin saturation. Serum ferritin (140 +/- 10.2 mi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450210
更新日期:1994-02-01 00:00:00
abstract::Primary iron overload encompasses a variety of genetic iron overload syndromes, dominated in frequency by HFE-related, or Type 1 hemochromatosis, for which French diagnostic and therapeutic guidelines have been recently proposed. Differential diagnosis of Type 1 hemochromatosis can be made from both clinical data and ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21071
更新日期:2007-12-01 00:00:00
abstract::We report on the association of Hb Dunn (alpha 6[A4]Asp----Asn) and Hb O-Arab (beta 121 [GH4]Glu----Lys) in a healthy Moroccan man. Hb Dunn had the same electrophoretic properties as Hb G-Philadelphia, but its percentage was lower. Its identification was based on sequence determination of the alpha T1 peptide. Bgl II ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830270405
更新日期:1988-04-01 00:00:00
abstract::To clarify the presentation and course of patients with chronic lymphocytic leukemia (CLL) and amyloidosis. Mayo databases were interrogated for patients who carried a diagnosis of amyloidosis and CLL evaluated at Mayo Clinic, Rochester from January 1974 to October 2012. Charts were abstracted and data analyzed. Of th...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23413
更新日期:2013-05-01 00:00:00
abstract::Among 47 patients with thrombotic thrombocytopenic purpura (TTP), 8 patients were diagnosed to have postoperative-TTP. Two patients underwent vascular surgery, 5 patients coronary artery bypass grafts, and 1 patient resection of myocardial sarcoma. Prior to surgery, all patients except one had normal hemograms and pla...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199609)53:1<11::AID-AJH3>3
更新日期:1996-09-01 00:00:00
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
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24130
更新日期:2015-11-01 00:00:00
abstract::A patient with acute nonlymphoblastic leukemia (FAB M4), showing a near tetraploid chromosome complement on his marrow cells, was treated with low dose cytosine arabinoside and achieved remission. During remission the near tetraploid marrow chromosome complement disappeared and reappeared upon relapse of leukemia. The...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830190412
更新日期:1985-08-01 00:00:00
abstract::We have characterized another subset of acute nonlymphocytic leukemia (ANLL) based on the cytogenetic and morphologic findings in a group of nine patients. Five patients had chromosomal analyses performed at the University of Chicago, two patients were studied at the All-Union Cancer Research Center in Moscow, and one...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830180409
更新日期:1985-04-01 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::Induction of carbonic anhydrase isozyme I (CA-I) by erythropoietin or hemin was investigated using erythroleukemia (K562) cells. Immunological estimation and purification of carbonic anhydrases showed that untreated K562 cells contained only carbonic anhydrase isozyme II(CA-II), while incubation of the cells with 2 un...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830380309
更新日期:1991-11-01 00:00:00