Abstract:
:Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Chaturvedi S,DeBaun MRdoi
10.1002/ajh.24235subject
Has Abstractpub_date
2016-01-01 00:00:00pages
5-14issue
1eissn
0361-8609issn
1096-8652journal_volume
91pub_type
杂志文章,评审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...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199709)56:1<59::aid-ajh12>
更新日期:1997-09-01 00:00:00
abstract::Botrocetin, a protein isolated from the venom of the snake Bothrops jararaca, induces platelet aggregation/agglutination by von Willebrand factor (vWF) binding to the membrane glycoprotein (GP) Ib, an action resembling that of ristocetin. However, some differences in the interaction between vWF and platelet GPIb induc...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830330409
更新日期:1990-04-01 00:00:00
abstract::Neonatal alloimmune thrombocytopenia is an uncommon but important cause of thrombocytopenia in infants. Because of the severity of the thrombocytopenia, some of these infants will have intracranial hemorrhage with resultant long-term disability. Obstetricians and neonatologists have recommended delivery by caesarean s...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280207
更新日期:1988-06-01 00:00:00
abstract::CD19+CD5+ lymphocytes constitute a minority of peripheral blood B cells. In view of the importance of these cells in the pathogenesis of the immunoregulation of myeloma, their incidence in another lymphoid organ was determined. CD5+ B cells were studied in 9 spleens from patients with multiple myeloma and in 10 spleen...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370306
更新日期:1991-07-01 00:00:00
abstract::We have previously shown that humic acid (well-water humic acid, HA, and synthetic humic acid, SHA) enhances cell surface expression of tissue factor (TF). Here we report that incubation of human umbilical vein endothelial cells (HUVEC) for 2 hr with HA or SHA cause a rapid rise in TF mRNA levels, as shown by Northern...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199603)51:3<200::AID-AJH4>
更新日期:1996-03-01 00:00:00
abstract::The erythrocytes of 90 pregnant women were evaluated for the presence of in vivo or in vitro oxidant damage. The reduced glutathione (P less than 0.005) and the membrane reduced sulfhydryl (P less than 0.001) concentrations were decreased in fresh erythrocytes. Following incubation with acetylphenylhydrazine, Heinz bo...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830180204
更新日期:1985-02-01 00:00:00
abstract::Although infectious complications represent a relevant cause of morbidity and mortality in patients with myelofibrosis (MF), little is known about their incidence, outcome and risk factors. We retrospectively evaluated a cohort of 507 MF patients, diagnosed between 1980 and 2014 in five Italian hematology centers, to ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24572
更新日期:2017-01-01 00:00:00
abstract::Transformation to secondary myelofibrosis (MF) occurs as part of the natural history of polycythemia vera (PPV-MF) and essential thrombocythemia (PET-MF). Although primary (PMF) and secondary MF are considered similar diseases and managed similarly, there are few studies specifically focused on the latter. The aim of ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24377
更新日期:2016-07-01 00:00:00
abstract::We analyzed 12 MLL/ENL positive ALL patients consecutively diagnosed between 1999 and 2009. The MLL/ENL fusion was identified in 4/150 (2.6%), 8/993 (0.8%), and 0/70 of pediatric, adult, and elderly patients, respectively. Eight patients had a WBC count >50 × 10(9) /L. Ten cases had an evaluable immunophenotyping. A B...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22161
更新日期:2011-12-01 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::This report reviews the diagnostic significance of immune markers, their relationship to patient outcome, and the therapeutic uses of monoclonal antibodies (MoAbs) in acute leukemia. Immunophenotyping allows for rapid and reproducible diagnosis in the majority of cases of acute leukemia. It is of particular importance...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830500307
更新日期:1995-11-01 00:00:00
abstract::Factor V G1691A (FV-Leiden) and prothrombin G20210A mutations are major inherited risk factors for venous thrombosis. Recently, it was suggested that both mutations, through stimulation of venous and placental thrombosis events, were strongly associated with recurrent idiopathic miscarriages, although other studies di...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10223
更新日期:2002-12-01 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::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::Neutrophil elastase (NE) is a serine protease stored in the primary granules of neutrophils that proteolytically cleaves multiple cytokines and cell surface proteins on release from activated neutrophils. Recent reports of mutations in the gene encoding this enzyme in some patients with neutropenic syndromes prompted ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10434
更新日期:2003-11-01 00:00:00
abstract::In a phase II trial, we evaluated chlorambucil and rituximab (CLB-R) as first-line induction treatment with or without R as maintenance for elderly chronic lymphocytic leukemia (CLL) patients. Treatment consisted of eight 28-day cycles of CLB (8 mg/m(2) /day, days 1-7) and R (day 1 of cycle 3, 375 mg/m(2) ; cycles 4-8...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ajh.23668
更新日期:2014-05-01 00:00:00
abstract::Indolent non-hodgkin lymphomas (iNHL) are a rare cause of monoclonal immunoglobulin deposits-related glomerulopathy (mIgGN). In patients with iNHL-related mIgGN, whether treatment should include either single or a combination of drug(s) to target the malignant clone and renal inflammation remains elusive. In this retr...
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.23798
更新日期:2014-10-01 00:00:00
abstract::Infantile malignant osteopetrosis (IMO) includes various genetic disorders that affect osteoclast development and/or function. Genotype-phenotype correlation studies in IMO have been hampered by the rarity and heterogeneity of the disease and by the severity of the clinical course, which often leads to death early in ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21447
更新日期:2009-08-01 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::A 64-year-old woman with mild bilateral parotid gland swelling and bilateral lower extremity purpura was admitted for evaluation of xerostomia and pancytopenia. The patient had an increased erythrocyte sedimentation rate, pancytopenia, and positive tests for antibodies to nuclear antigen, SS-A, and SS-B. Impaired cell...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830430312
更新日期:1993-07-01 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::Splenic lymphoma with villous lymphocytes (SLVL) is a relatively new entity with only a few reports published. We report seven cases of SLVL with detailed clinicopathologic and comprehensive immunophenotypic studies to further characterize this lymphoma, which is frequently confused with hairy cell leukemia and other ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830450107
更新日期:1994-01-01 00:00:00
abstract:DISEASE OVERVIEW:Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms primarily characterized by erythrocytosis and thrombocytosis, respectively. Other disease features include leukocytosis, splenomegaly, thrombohemorrhagic complications, vasomotor disturbances, pruritus, and a sma...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21946
更新日期:2011-03-01 00:00:00
abstract::"Autonomous" development of erythroid colonies in erythropoietin (EPO)-free semi-solid culture has been used as an in vitro assay for diagnosis of polycythemia vera (PV). These colonies, however, are small and poorly hemoglobinized, rendering the assay in many cases unreliable. We report here on the use of a novel ass...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199701)54:1<47::aid-ajh7>3
更新日期:1997-01-01 00:00:00
abstract::In May 2005 at the 8th International Symposium on Myelodysplastic Syndromes (MDS), a consensus meeting was held on iron overload in MDS (Seymour, Hematol Oncol Clin 2005; Suppl 1:18-25). The recommendations of the 2005 consensus meeting were discussed in the context of currently available evidence at the 9th Internati...
journal_title:American journal of hematology
pub_type: 杂志文章,实务指引
doi:10.1002/ajh.21269
更新日期:2008-11-01 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::The aim of our study was to assess the cytokine profile of sickle cell disease (SCD) patients in steady state and in vaso-occlusive crisis (VOC). VOC has a complex nature, involving interactions between sickle red blood cells (RBC), the endothelium, and leucocytes. Endothelial damage due to recurrent adhesion of sickl...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20196
更新日期:2004-12-01 00:00:00
abstract::Light chain deposition disease (LCDD) is characterized by monotypic immunoglobulin depositions which will eventually lead to loss of organ function if left untreated. While the kidney is almost always affected, the presence and degree of LCDD in other organs vary. Ten to thirty percent of LCDD patients have underlying...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24756
更新日期:2017-08-01 00:00:00
abstract::Acute leukemias of ambiguous lineage (ALAL) comprise acute undifferentiated leukemias (AUL) and mixed-phenotype acute leukemias (MPAL). In the revised fourth edition of the World Health Organization (WHO) classification provided further refinements to the diagnostic criteria for ALAL. Molecular characterization of MPA...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25771
更新日期:2020-06-01 00:00:00
abstract::Complete remission can be achieved in 60-80% of adults with diffuse aggressive non-Hodgkin's lymphoma. However, 20-40% of them will subsequently relapse. Nevertheless, formal follow-up guidelines for recurrence detection have never been advocated. We analyzed the pattern of relapse in 30 patients with intermediate- an...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10017
更新日期:2002-01-01 00:00:00