Abstract:
:Forty-one patients with polycythemia vera (PV) according to the PVSG criteria were analysed retrospectively from January 1960 to March 1996. There were 23 male and 18 female patients with a median follow-up of 66.5 months (3-431 months). Median age was 62 (range: 37-85). The median hemoglobin level at diagnosis was 18.8 g/dl. Four patients were treated by venesection alone, 20 patients received hydroxyurea and intermittent venesection, 3 were treated with radioactive phosphorus alone, and 14 had both hydroxyurea and radioactive phosphorus. During the course of illness, 14 patients (34%) developed a total of 19 thrombotic events. Of the thrombotic events, 16 were arterial and 3 were venous. Two patients had both arterial and venous thrombosis sequentially. The probability of thrombosis-free survival after treatment was 83% at 10 years and 73% at 20 years. One patient developed post-polycythemic myeloid metaplasia 24 months after diagnosis. Of 17 patients exposed to radioactive phosphorus, only 1 developed secondary acute myeloid leukemia (AML) 9 years afterwards. Of the 20 patients treated with hydroxyurea for a median duration of 63.5 months (2-130 months), there is no case of secondary malignancy. Overall survival was 83% at 10 years and 62% at 20 years. In conclusion, PV in Chinese is a relatively benign disease with a low risk of thrombosis as compared to Caucasian patients. Hydroxyurea has a very low risk of secondary leukemia and is a safe drug to use in Chinese patients with PV.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Chim CS,Kwong YL,Chan PT,Liang Rdoi
10.1002/(sici)1096-8652(199709)56:1<59::aid-ajh12>subject
Has Abstractpub_date
1997-09-01 00:00:00pages
59-62issue
1eissn
0361-8609issn
1096-8652pii
10.1002/(SICI)1096-8652(199709)56:1<59::AID-AJH12>journal_volume
56pub_type
杂志文章abstract::Vascular complications are the main cause of morbidity in diabetes mellitus. To evaluate lipoprotein and hemostatic parameters and their relationship with clinically detectable microangiopathy, we studied 58 insulin-dependent diabetes mellitus patients and 60 controls matched for age, sex, and body mass index. Thirtee...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199710)56:2<93::aid-ajh4>3
更新日期:1997-10-01 00:00:00
abstract::Immunomodulatory drugs (IMiDs) are a cornerstone in the treatment of multiple myeloma (MM), but specific markers to predict outcome are still missing. Recent work pointed to a prognostic role for IMiD target genes (e.g. CRBN). Moreover, indirect activity of IMiDs on immune cells correlated with outcome, raising the po...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24634
更新日期:2017-03-01 00:00:00
abstract::We report a patient with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) who presented with rapid enlargement of a cervical lymph node due to localized herpes simplex lymphadenitis, which was clinically indistinguishable from large cell (Richter's) transformation. The diagnosis was made by excisional...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10006
更新日期:2001-12-01 00:00:00
abstract::Iron refractory iron deficiency anemia (IRIDA) is a rare hereditary disease caused by mutations in TMPRSS6 gene encoding Matriptase-2, a negative regulator of hepcidin transcription. Up to now, 53 IRIDA patients from 35 families with different ethnic origins have been reported and 41 TMPRSS6 mutations have been identi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23929
更新日期:2015-04-01 00:00:00
abstract::Newly identified iron binding proteins isolated from rat duodenal homogenates permit better understanding of iron absorption. Mucins bind iron at acid pH to keep iron soluble and available for absorption at the more alkaline pH of the duodenum; this explains iron deficiency following prolonged achlorhydria. Integrin (...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830420114
更新日期:1993-01-01 00:00:00
abstract::AL amyloidosis, a systemic disorder characterized by widespread deposition of amyloid fibrils derived from monoclonal Ig light chains in organs and soft tissues, is typically caused by an underlying plasma cell dyscrasia. However, this disease can also be associated rarely with a B-cell lymphoproliferative disorder. I...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20635
更新日期:2006-09-01 00:00:00
abstract::We examined inter- and intraobserver reproducibility and concordance between histological diagnosis and independently collected clinical findings in a large series of patients with the major subtypes of myeloproliferative neoplasms (MPNs) and controls. Seven hematopathologists reviewed 272 bone marrow biopsies includi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23554
更新日期:2013-12-01 00:00:00
abstract::Between 1980 and 1988, 126 patients with leukemia were treated with piperazinedione and fractionated total body irradiation (TBI) followed by allogeneic bone marrow transplantation from HLA matched siblings. Sixty-one patients had acute myelogenous leukemia, 46 acute lymphoblastic leukemia, and 19 chronic myelogenous ...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.2830460205
更新日期:1994-06-01 00:00:00
abstract::We describe a 25-year-old black woman who presented with a long history of anemia requiring transfusions during childhood and adolescence. Molecular analysis revealed her to be a compound heterozygote for the sickle mutation and the approximately 22.7 kb deletion associated with hemoglobin Kenya. This patient's clinic...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830410413
更新日期:1992-12-01 00:00:00
abstract::In polycythemia vera (PV), treatment with chlorambucil and radioactive phosphorus (p32) increases the risk of leukemic transformation from 1% to 13-14%. This risk has been estimated to be 1-5.9% with hydroxyurea (HU) therapy. When compared with historical controls, the risk with use of HU does not appear to be statist...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199605)52:1<42::AID-AJH7>3
更新日期:1996-05-01 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::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::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::A modified protein-bound cobalamin absorption test was used to study dietary cobalamin absorption in healthy adults of different age groups and patients with isolated low serum concentrations of cobalamin. Dietary cobalamin absorption was significantly reduced in healthy adults aged 55-75 years compared with young adu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830390202
更新日期:1992-02-01 00:00:00
abstract::Hydroxyurea is a drug that is used to treat some patients with sickle cell disease. We have measured the deformability of sickle erythrocytes incubated in hydroxyurea in vitro and found that hydroxyurea acts to decrease the deformability of these cells. The deformability of normal erythrocytes was not significantly af...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.1098
更新日期:2001-07-01 00:00:00
abstract::Initially described in 1948 by Hertert thromboelastography (TEG) provides a real-time assessment of viscoelastic clot strength in whole blood. Rotational thromboelastometry (ROTEM) evolved from TEG technology and both devices generate output by transducing changes in the viscoelastic strength of a small sample of clot...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23599
更新日期:2014-02-01 00:00:00
abstract::Erythrocytes from ten patients with severe liver disease displayed low methylene blue-stimulated hexose monophosphate (HMP) shunt activity and glucose recycling despite elevated total glucose consumption when compared to controls. Heinz body formation was increased and reduced glutathione concentration significantly d...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830060403
更新日期:1979-01-01 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::Because of the T-cell abnormalities observed in Hodgkin's disease and the growing number of Hodgkin's disease cases observed in association with the acquired immunodeficiency syndrome (AIDS), concern has been expressed that a retrovirus may be the primary cause of Hodgkin's disease. We examined plasma specimens from 1...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830270104
更新日期:1988-01-01 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::Human T-cell leukemia virus (HTLV-I) is known to be associated with certain hematologic malignancies, and a related virus, HTLV-III/LAV, might be the cause of AIDS. Some persons with AIDS have had evidence of HTLV-I infection. Unrelated to these findings, it has been suggested that HTLV-I is transmitted via blood prod...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830200205
更新日期:1985-10-01 00:00:00
abstract::The relative importance of the resolution level of HLA typing has not been fully defined for related donor transplantation. To address this question, we retrospectively evaluated patients who underwent a first related hematopoietic stem cell transplantation (HSCT) from 2000 to 2011 from an HLA high-resolution matched ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24028
更新日期:2015-07-01 00:00:00
abstract::BCR-ABL1-negative myeloproliferative neoplasms (MPNs) are clonal stem cell disorders defined by proliferation of one or more myeloid lineages, and carry an increased risk of vascular events and progression to myelofibrosis and leukemia. Portal hypertension (pHTN) occurs in 7-18% of MPN patients via both thrombotic and...
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.24798
更新日期:2017-09-01 00:00:00
abstract::Children with sickle cell anemia (SCA) and a primary overt stroke are at high risk of recurrent (secondary) stroke. Chronic transfusion therapy dramatically reduces but does not eliminate this high risk, and inevitably results in transfusion-related hemosiderosis. We previously reported the use of hydroxyurea/phleboto...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21986
更新日期:2011-04-01 00:00:00
abstract::Over 300,000 infants are born annually with sickle cell anemia (SCA) in sub-Saharan Africa, and >50% die young from infection or anemia, usually without diagnosis of SCA. Early identification by newborn screening (NBS), followed by simple interventions dramatically reduced the mortality of SCA in the United States, bu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23578
更新日期:2013-12-01 00:00:00
abstract::An infectious episode is the presenting manifestation of hairy cell leukemia (HCL) in approximately 30% of cases. Most often this is bacterial and only rare cases of opportunistic fungal infection are described. We report a patient who presented with sporotrichal involvement of multiple cutaneous sites and lymph nodes...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460214
更新日期:1994-06-01 00:00:00
abstract::Leukemic cells of 43 patients with acute promyelocytic leukemia (M3) were investigated morphologically and cytochemically to determine the percentage of aberrant enzymes and whether or not the presence impacts on the clinical outcome. Twelve patients (27.9%) showed alpha-naphthyl acetate esterase (ANAE) activity in th...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830290306
更新日期:1988-11-01 00:00:00
abstract::Ten previously untreated elderly patients with hypocellular acute leukemia received a low dose of cytosine arabinoside (Ara-C), 10 mg/m2 injected subcutaneously every 12 hours for 14-28 days. Six patients achieved a complete remission (CR) for periods ranging for 6-23 months (median 8.5 months) and the others had a pa...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.2830390111
更新日期:1992-01-01 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::An HTLV-I-seronegative case of adult T-cell leukemia (ATL) carrying the HTLV-I genome is reported. Screening serological tests were negative and Western blot analysis revealed only a faint band for HTLV-I p24. Polymerase chain reaction (PCR) disclosed the presence of HTLV-I gag, pol, env, pX, and LTR sequences in the ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199610)53:2<133::AID-AJH13
更新日期:1996-10-01 00:00:00