Abstract:
:The purpose of the study was to analyze clinical and/or autopsy findings at the time of death among adults with sickle cell disease (SCD) at Howard University in Washington, DC over a 25-year period. A single physician recorded circumstances of death among 141 adult SCD patients he treated and knew well from 1976 to 2001. These findings were determined by autopsy report and/or clinical assessment. In a subset of 31 patients, autopsy records were reviewed for reports of iron deposition in liver and heart and of organ pathology. One hundred and fourteen (80.9%) of the patients had SS phenotype and 66 (46.8%) were female. The mean +/- SD age at death was 36 +/- 11 years. Leading circumstances of death included pulmonary hypertension (PHT) (26.2%), sudden death (23.4%), renal failure (22.6%), infection (18.4%), thromboembolism (14.9%), cardiac diagnoses (12.0%), cirrhosis (11.3%), pneumonia or acute chest syndrome (9.9%), bleeding (7.8%), and iron overload (7.0%). When circumstances of deaths that occurred after 1991 (n = 69) were compared to those that occurred in 1991 or earlier (n = 72), PHT (36.2% vs. 16.6%; P < 0.01) was significantly more common in 1992 or later. Significant associations were found between PHT and thromboembolism and between cirrhosis and iron overload. In this proportional mortality study of adults with SCD, PHT was the leading finding at the time of death. Thromboembolism was associated with PHT, and iron overload was associated with cirrhosis.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Darbari DS,Kple-Faget P,Kwagyan J,Rana S,Gordeuk VR,Castro Odoi
10.1002/ajh.20685subject
Has Abstractpub_date
2006-11-01 00:00:00pages
858-63issue
11eissn
0361-8609issn
1096-8652journal_volume
81pub_type
杂志文章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::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
pub_type: 杂志文章,评审
doi:10.1002/ajh.21503
更新日期:2009-10-01 00:00:00
abstract::Novel agents in combination with melphalan and prednisone (MP) significantly improved progression-free survival (PFS) and overall survival (OS) in multiple myeloma (MM). Randomized trials comparing MP plus bortezomib (VMP) versus MP plus thalidomide (MPT) are lacking. Nine hundred and fifty-six elderly (>65 years) new...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23641
更新日期:2014-04-01 00:00:00
abstract::Acetylsalicylic acid (ASA) is currently recommended as an antithrombotic for patients with essential thrombocythemia (ET) who are at an increased risk of thrombotic events. However, ASA is also associated with an increased risk of bleeding in these patients as compared to the risk of bleeding in other patients treated...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199804)57:4<277::aid-ajh2>
更新日期:1998-04-01 00:00:00
abstract::Four men were diagnosed with human immunodeficiency virus infection (AIDS) and autoimmune hemolytic anemia (HIV-AIHA) during the years 1997-2000 at Cook County Hospital, Chicago. All patients presented with the acute onset of severe hemolytic anemia, fever, and splenomegaly. The direct and indirect antiglobulin tests ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10096
更新日期:2002-06-01 00:00:00
abstract::In HIV-positive patients with non-Hodgkin lymphoma (NHL), no benefit of adding rituximab to chemotherapy was seen in a randomized controlled trial (RCT). We performed a meta-analysis of prospective studies to ascertain outcomes in HIV-positive NHL patients treated with chemotherapy (chemo) versus rituximab and chemo (...
journal_title:American journal of hematology
pub_type: 杂志文章,meta分析
doi:10.1002/ajh.22275
更新日期:2012-03-01 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::While evidence for measurable residual disease (MRD) is a harbinger of inferior outcome in acute myeloid leukemia (AML) patients referred for allogeneic stem cell transplantation (allo-SCT), the exact clinical trajectory of specific patient subsets in this clinical setting is undefined. Using a recently published prog...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25905
更新日期:2020-06-12 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::A 1750 base pair cDNA to human terminal deoxynucleotidyl transferase (TdT) has been cloned. This cDNA detects a dominant 2200 base pair messenger RNA species in normal and leukemic cells synthesizing the enzyme. A quantitative dot blot assay was utilized to survey a number of clinical samples from patients with TdT po...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830250305
更新日期:1987-07-01 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::We describe two patients with mild hemophilia A (MHA) who developed high titer inhibitor (HTI) following intensive recombinant factor VIII (rFVIII) concentrate replacement for surgery and trauma. Intranasal desmopressin was instituted shortly following immunosuppressive therapy (IST) and activated prothrombin complex ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.1176
更新日期:2001-11-01 00:00:00
abstract::A variability in DNA content detected was found with image cytometry, in immature bone marrow cells from 13 healthy donors (median age 31 yr). The mean coefficient of variation (C.V.) of the DNA content was found to be significantly (P = 0.0002) higher in immature blasts and promyelocytes than in mature granulocytes, ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830430412
更新日期:1993-08-01 00:00:00
abstract::The serum free light chain (FLC) assay quantitates free immunoglobulin kappa and lambda light chains, which has prognostic value in plasma cell dyscrasias. However, there is limited data on serum FLC in lymphoid malignancies. We analyzed the association of pretreatment FLC with event-free survival (EFS) and overall su...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.22168
更新日期:2011-12-01 00:00:00
abstract::A 75-year-old female patient with agranulocytosis caused by ticlopidine is reported. She took the drug at 200 mg/day for 30 days to prevent recurrence of cerebral infarction. The leukocyte count at the nadir was 500/microliters on the 34th day since she started to take the drug. Complete recovery of her peripheral leu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370405
更新日期:1991-08-01 00:00:00
abstract::Recent advances in massively parallel sequencing technologies have provided a detailed picture of the mutational landscape in CLL and underscored the vast degree of interpatient and intratumor heterogeneities. These studies have led to the characterization of novel putative driver genes and recurrently affected biolog...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24301
更新日期:2016-05-01 00:00:00
abstract::A case of idiopathic myelofibrosis (IMF) presenting with hypercalcemia and hypercalcitriolemia is reported. It is proposed that ectopic production of the active vitamin D metabolite related to ongoing clonal expansion in the bone marrow accounts for the hypercalcemic state. Consistently low levels of circulating type ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830390316
更新日期:1992-03-01 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::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::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::Familial Pseudohyperkalemia (FP) is a dominant red cell trait characterized by increased serum [K(+)] in whole blood stored at or below room temperature, without additional hematological abnormalities. Functional gene mapping and sequencing analysis of the candidate genes within the 2q35-q36 critical interval identifi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23357
更新日期:2013-01-01 00:00:00
abstract::A single-tube osmotic fragility test has been proposed for thalassemia screening with a range of different concentrations of saline having been employed. We have compared the sensitivity and specificity of 0.32%, 0.34%, and 0.36% buffered saline, and on the basis of our findings, recommend the use of 0.36% saline. Thi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20387
更新日期:2005-07-01 00:00:00
abstract::A 67-year-old woman was admitted to our hospital because of lymphadenopathy and lymphocytosis. Monoclonal integration of HTLV-I provirus DNA was detected, and a diagnosis of adult T-cell leukemia (ATL) was made. Flow cytometry revealed that the ATL cells expressed CD20 as well as T-cell-associated antigens, and expres...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200101)66:1<39::AID-AJH1005>3.0.
更新日期:2001-01-01 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::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::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::The effect of several iron chelators on iron uptake and release by mouse peritoneal macrophages has been investigated. The 1,2-dimethyl (L1) and 1-ethyl-2-methyl (L1NEt) derivatives of 3-hydroxypyrid-4-one markedly enhanced iron mobilisation from macrophages pulsed with 59Fe-transferrin-antitransferrin immune complexe...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830340106
更新日期:1990-05-01 00:00:00
abstract::Red blood cell (RBC) autoantibodies are a relatively uncommon cause of anemia. However, autoimmune hemolytic anemia (AIHA) must be considered in the differential diagnosis of hemolytic anemias, especially if the patient has a concomitant lymphoproliferative disorder, autoimmune disease, or viral or mycoplasmal infecti...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.10062
更新日期:2002-04-01 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::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