Abstract:
:Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >/=18 years seen at the Adult Sickle Cell Center at Duke University Medical Center between January 2000 and April 2005 were enrolled. Forty-three patients (21 males and 22 females) died during the study period. The median age of survival was 39 years for females (95% CI: 34-56), 40 years for males (95% CI: 34-48), and 40 years overall (95% CI: 35-48). Cardiac causes of death accounted for 25.6% (11/43 patients); pulmonary, 14.0% (six patients); other SCD related, 32.6% (14 patients); unknown, 14.0% (six patients); and others, 14.0% (six patients). Pulseless electrical activity arrest, pulmonary emboli, multiorgan failure, and stroke were the most frequent causes of death. Among the deceased patients, the most common premorbid conditions were cardiopulmonary: acute chest syndrome/pneumonia (58.1%), Pulmonary hypertension (pHTN; 41.9%), systemic HTN (25.6%), congestive heart failure (25.6%), myocardial infarction (20.9%), and arrhythmias (14.0%). Tricuspid regurgitant jet velocity was significantly higher (3.1 m/sec vs. 2.6 m/sec, P < 0.001) and hemoglobin significantly lower (8.3 g/dL vs. 9.2 g/dL, P < 0.05) in deceased patients when compared with patients who lived, respectively. With improved preventive and therapeutic advances, including hydroxyurea therapy, acute complications such as infection are no longer the leading cause of death; instead, causes of death and premorbid conditions are shifting to chronic cardiopulmonary complications. Further, arrhythmia leading to premature death is under-recognized in SCD and warrants further investigation.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Fitzhugh CD,Lauder N,Jonassaint JC,Telen MJ,Zhao X,Wright EC,Gilliam FR,De Castro LMdoi
10.1002/ajh.21569subject
Has Abstractpub_date
2010-01-01 00:00:00pages
36-40issue
1eissn
0361-8609issn
1096-8652journal_volume
85pub_type
杂志文章abstract::A quantitative bioassay for serum erythropoietin in anemic patients was established with erythroid colony-forming technique using methyl cellulose. Fetal mouse liver contains many erythropoietin-dependent erythroid colony-forming cells (CFU-E) with the concentration as well as sensitivity to erythropoietin being the h...
journal_title:American journal of hematology
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doi:10.1002/ajh.2830120210
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abstract::Platelet-rich plasma were treated with increasing concentrations of vitamin E (alpha-tocopherol). Washed platelets were exposed to oxidized low density lipoprotein (LDL) and examined by aggregometry and electron microscopy. The treatment of washed platelets by oxidized LDL induced morphological signs of activation lik...
journal_title:American journal of hematology
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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
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abstract::We report a case of refractory anemia with excess blasts (RAEB) developing in a 67-year old man with a history of polycythemia vera; results of cytogenetic and immunophenotyping studies are described. In this report the clinical, cytogenetic and hematologic features of myelodysplasia complicating polycythemia vera are...
journal_title:American journal of hematology
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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
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doi:10.1002/ajh.2830410413
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abstract::Although numerous publications have described the chromosome abnormalities in B-cell non-Hodgkin lymphoma and their significance, sparse literature exists pertaining to the chromosome abnormalities in T-cell lymphoma. We did cytogenetic analyses in 21 cases of peripheral T-cell lymphoma (PTCL). Chromosomally abnormal ...
journal_title:American journal of hematology
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doi:10.1002/ajh.2830350205
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abstract::In this study, we investigated the incidence and prognostic impact of monosomal karyotype (MK) in 405 higher-risk Myelodysplastic Syndromes (MDS) patients treated with 5-AZA. The MK was present in 66 out of 405 (16.3%) patients, most of whom had complex karyotype (CK). MK was strongly associated with CK and the cytoge...
journal_title:American journal of hematology
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abstract::We report the case of a 59-year-old woman with Durie-Salmon stage IIIB IgGkappa multiple myeloma (MM), who presented 83 days after autologous hematopoietic stem cell transplant (HSCT) with multiple subcutaneous plasmacytomas. These lesions were confined exclusively to sites where the patient had sustained local trauma...
journal_title:American journal of hematology
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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
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更新日期:2016-08-01 00:00:00
abstract:DISEASE OVERVIEW:The myelodysplastic syndromes (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older males and in individuals with prior exposure to cytotoxic th...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24102
更新日期:2015-09-01 00:00:00
abstract::A risk score based on three biological features (CD38, ZAP-70, and IGHV mutational status) was previously developed to predict progression-free survival (PFS) in untreated Binet A CLL patients. Here we perform a score validation analysis in a prospective and independent cohort of patients. Biological markers (CD38, ZA...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
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更新日期:2014-07-01 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::Bacterial and protozoal infections can cause thrombocytopenia and may mimic idiopathic thrombocytopenic purpura (ITP). Brucella species and Toxoplasma are among the infectious agents with protean clinical manifestations which may induce immune thrombocytopenia. In rare cases, thrombocytopenia can be severe and may res...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10382
更新日期:2003-09-01 00:00:00
abstract::Agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder arising from a single hematopoietic cell. Approximately 5% of reported cases of AMM have terminated in leukemic crisis; however, the precise characteristics of the leukemic cells have rarely been reported. We report a case of AMM that occurred...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830440111
更新日期:1993-09-01 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
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更新日期:2020-05-01 00:00:00
abstract::Venous thromboembolism (VTE) is a common cause of morbidity and mortality among patients with multiple myeloma (MM). The International Myeloma Working Group (IMWG) developed guidelines recommending primary thromboprophylaxis, in those identified at high-risk of VTE by the presence of risk factors. The National Compreh...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25603
更新日期:2019-11-01 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
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journal_title:American journal of hematology
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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::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::The objective of this study is to establish a method to identify patients with primary immune thrombocytopenia (ITP) utilizing administrative data from diverse data sources that would be appropriate for epidemiologic studies of ITP, regardless of patients' age and source of health care. Medical records of the Oklahoma...
journal_title:American journal of hematology
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更新日期:2012-09-01 00:00:00
abstract::The optimal treatment of advanced sporadic Burkitt lymphoma in adults is still a matter of debate. The salutary results of pediatric therapies did open the road for improving the adult outcome. Between May 1988 and March 2009, 71 consecutive patients-46 adults, 25 children-affected by Burkitt lymphoma/leukemia were tr...
journal_title:American journal of hematology
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更新日期:2012-01-01 00:00:00
abstract::The main treatment for many patients with Myelodysplastic Syndromes (MDS) remains red cell transfusion to attenuate the symptoms of chronic anemia. Fatigue can reduce a patient's health related quality of life (HRQoL), but there is little understanding of the optimal use of transfusions to improve this. A systematic r...
journal_title:American journal of hematology
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更新日期:2009-10-01 00:00:00
abstract::Patients presenting with Hodgkin's disease (HD) may show lung involvement characterized by contiguous spread from ipsilateral hilar lymph nodes. Lung consolidation or noncontiguous pulmonary involvement makes an alternative diagnosis more likely. This report describes a patient with HD in whom concurrent pulmonary bla...
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abstract::Venous thromboembolism (VTE) recurs frequently. Greater height is associated with increased risk of incident VTE, but it is unclear whether height is related to risk of VTE recurrence. Recurrent VTE is associated with substantial morbidity and mortality, thus identifying individuals at greatest risk of experiencing a ...
journal_title:American journal of hematology
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abstract::The purpose of the study was to assess consensus and interobserver agreement among an international panel of six hematopathologists regarding characterization and reproducibility of bone marrow (BM) histologic features used to diagnose early stage myeloproliferative neoplasms, in particular differentiation of so-calle...
journal_title:American journal of hematology
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更新日期:2017-10-01 00:00:00