Abstract:
:The hereditary persistence of fetal hemoglobin (HPFH)-6 is sporadically found in Thailand whereas the deletion-inversion type (G)gamma((A)gamma delta beta)(0)-thalassemia is described among Indians. We report a hitherto un-described case in which these two defects co-segregate. He was a 3-year-old Thai boy who had a feature of thalassemia intermedia phenotype with the following hematologic data; Hb 8.8 g/dL, Hct 29.2%, MCV 66.9 fL, MCH 20 pg, and MCHC 30.1 g/dL. Hemoglobin analysis revealed 100% Hb F with only (G)gamma-globin chain. Globin gene analyses demonstrated that he carried the HPFH-6 deletion in trans to the Indian deletion-inversion (G)gamma((A)gamma delta beta)(0)-thalassemia. Hematologic data of the patient was compared to those of the HPFH-6 heterozygote found in his father, to (G)gamma((A)gamma delta beta)(0)-thalassemia heterozygotes detected in his mother and sister, and to that of an unrelated Thai patient who was a compound heterozygote for the deletion-inversion (G)gamma((A)gamma delta beta)(0)-thalassemia and HbE.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Fucharoen S,Pengjam Y,Surapot S,Fucharoen G,Sanchaisuriya Kdoi
10.1002/ajh.10202keywords:
subject
Has Abstractpub_date
2002-10-01 00:00:00pages
109-13issue
2eissn
0361-8609issn
1096-8652journal_volume
71pub_type
杂志文章abstract::Fifty-nine HIV-1 antibody positive and 58 antibody negative hemophiliacs were evaluated over a 2 year study period to gain insight into the natural history and prognosis of HIV-1 disease in members of this risk group. Mean CD4 (Leu 3+) cell counts calculated at 6 month intervals decreased gradually in seropositive pat...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830320406
更新日期:1989-12-01 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::"PVP storage disease" is a disorder occurring in patients who have received high molecular weight polyvinylpyrrolidone (PVP), which cannot be excreted from the body. These large polymers deposit in the histiocytes and cause proliferation and infiltration of histiocytes in the reticuloendothelial system. There was usua...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199801)57:1<68::aid-ajh12>
更新日期:1998-01-01 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::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::Alpha-hemoglobin stabilizing protein (AHSP) is a potential modifier of beta-thalassemia by virtue of its ability to detoxify excess free alpha-globin. However, examination of patients with beta-thalassemia from a few geographic regions failed to identify obvious AHSP mutations. We extended these studies by analyzing A...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21041
更新日期:2008-02-01 00:00:00
abstract::Sickle cell disease (SCD) nephropathy and lower estimated glomerular filtration rate (eGFR) are risk factors for early mortality. Furthermore, rate of eGFR decline predicts progression to end-stage renal disease in many clinical settings. However, factors predicting renal function decline in SCD are poorly documented....
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25263
更新日期:2018-12-01 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
doi:10.1002/(sici)1096-8652(199705)55:1<1::aid-ajh1>3.
更新日期:1997-05-01 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::Various in vitro studies and clinical observations suggest that Fanconi's anemia (FA) patients are unable to detoxify adequately superoxide anions (O2-) released by activated phagocytes. Recent studies have shown that certain lymphokines such as tumor necrosis factor-alpha (TNF-alpha) and interferon-gamma (IFN-gamma) ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830420211
更新日期:1993-02-01 00:00:00
abstract::In order to study the influence of oxidized low-density lipoprotein (Ox-LDL) on platelet functional morphology at an early activation stage, washed human blood platelets were stimulated by 100 micrograms/ml Ox-LDL at 37 degrees C. The settling and spreading process of stimulated and unstimulated platelets on Formvar-c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490302
更新日期:1995-07-01 00:00:00
abstract::A patient with Felty's syndrome (FS) and persistent profound neutropenia developed recurrent infections and sepsis syndrome. No impairment of granulocyte-macrophage colony development was observed in vitro. Marrow morphology revealed an absence of mature neutrophil forms despite administration of granulocyte-colony st...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830430415
更新日期:1993-08-01 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::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::The Mo2 antibody recognizes a monocyte-specific cell surface antigen of unknown function. Upward modulation of Mo2 surface epitope density was demonstrated in response to 72-hr culture of monocytes with respiratory syncytial virus (RSV), but this was not seen after culture with phytohemagglutinin or other respiratory ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830250314
更新日期:1987-07-01 00:00:00
abstract::Unprecedented advances in our understanding of the pathobiology, prognostication, and therapeutic options in mantle cell lymphoma (MCL) have taken place in the last few years. Heterogeneity in the clinical course of MCL-indolent vs aggressive-is further delineated by a correlation with the mutational status of the var...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25487
更新日期:2019-06-01 00:00:00
abstract::Several studies reported improved outcomes of adolescents and young adults (AYA) with acute lymphoblastic leukemia (ALL) treated with pediatric-based ALL regimens. This prompted the prospective investigation of a pediatric Augmented Berlin-Frankfurt-Münster (ABFM) regimen, and its comparison with hyper-fractionated cy...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24419
更新日期:2016-08-01 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::Variation in bleeding in the perioperative period is a complex and multifactorial event associated with immediate and delayed consequences for the patient and health care resources. Little is known about the complex genetic influences on perioperative bleeding. With the discovery of multiple variations in the human ge...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21205
更新日期:2008-09-01 00:00:00
abstract::Imatinib is currently the standard treatment for chronic myeloid leukemia(CML). Previous studies have shown that imatinib affects bone metabolism in CML patients. However, these effects are not well-studied prospectively. The authors studied bone-mineral density (BMD) and bone metabolism in 17 CML patients and matched...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.23155
更新日期:2012-05-01 00:00:00
abstract::A 63-year-old white man with myasthenia gravis and red cell aplasia was found to have hepatic iron overload. The differential diagnosis included hereditary hemochromatosis and secondary iron overload related to red cell aplasia. Family studies of siblings, including HLA typing, initially suggested a diagnosis of secon...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450314
更新日期:1994-03-01 00:00:00
abstract::The fat embolism syndrome is an important complication of patients with sickle cell hemoglobinopathies because of severe morbidity and mortality. Our recent experience with three cases that survived with intensive supportive care and prompt use of transfusion stimulates this review. A high index of suspicion, prompt u...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20348
更新日期:2005-05-01 00:00:00
abstract::The trial CICL670AUS04 was a single-arm, open-label study of the cardiac efficacy of 18 months of deferasirox monotherapy [1]. Cardiac response in this study was related to the degree of liver siderosis. Patients with mild to moderate liver siderosis improved their cardiac T2* while more severely siderotic patients di...
journal_title:American journal of hematology
pub_type: 临床试验,信件
doi:10.1002/ajh.21830
更新日期:2010-10-01 00:00:00
abstract::A minority of super-utilizing adults with sickle cell disease (SCD) account for a disproportionate number of emergency department (ED) and hospital admissions. We performed a retrospective cohort study comparing the rate of admission before and after the opening of a clinic for adults with SCD. Unique to this clinic w...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23912
更新日期:2015-03-01 00:00:00
abstract::This report describes studies investigating the use of a collagen binding assay to improve the laboratory monitoring of desmopressin (DDAVP) therapy in patients with von Willebrand's disease (vWD). We evaluated the response of seven patients with vWD (four type I, three type IIA) to DDAVP, administered using a standar...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450303
更新日期:1994-03-01 00:00:00
abstract::Chronic lymphocytic leukemia (CLL) is rarely complicated by chylothorax: we present a 93-year-old woman with CLL who developed recurrent pleural effusions that were ultimately found to be chylous in nature. Despite eight repeated thoracenteses, she continued to experience re-accumulation of fluid, and therefore, video...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10142
更新日期:2002-07-01 00:00:00
abstract::The treatment of corticosteroid- and/or splenectomy-refractory immune thrombocytopenic purpura (ITP) includes vinca alkaloids, immunosuppressives, Danazol, intravenous gammaglobulin, and alpha-interferon. However, these treatments have often been associated with toxic side effects. Brox et al. (Br J Haematol 70:341-34...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830430110
更新日期:1993-05-01 00:00:00
abstract::We have developed a rapid approach to detect the two most common alpha-thalassemia-2 (alpha-thal-2) determinants by the polymerase chain reaction (PCR) technique, which takes a few hours to complete. Specific oligonucleotides selectively amplify appropriate segments of the chromosome with the deletion and the normal c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460309
更新日期:1994-07-01 00:00:00
abstract::In this study, we analyzed a thiotepa-based conditioning regimen for allogeneic stem cell transplantation in adults with acute lymphoblastic leukemia, using the EBMT database. A total of 323 patients were identified. The median age was 43 years. Disease status at transplant was first complete remission (CR1) in 48.9%,...
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.24567
更新日期:2017-01-01 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