Abstract:
:The objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myeloid metaplasia (AMM). We searched the literature (Medline, 1970-1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or transfusion requirements in patients with AMM. We employed formal decision analysis to determine the relative value of medical and surgical treatment of advanced AMM. Results of data synthesis showed that splenectomy in AMM is associated with an operative mortality of 13.4% (95% confidence intervals (CI): 9.5-17.2%), an early morbidity of 45.3% (CI: 39.6-51.1%), and a late morbidity of 16.3% (CI: 9.9-22.5%). Almost all patients with portal hypertension and painful splenomegaly, but only about half of those with thrombopenia and anemia were reported to have experienced relief in their symptoms or signs after splenectomy. We found no evidence that splenectomy affects survival in AMM. We concluded that splenectomy in advanced AMM is a palliative procedure that carries a substantial risk. It may be considered for symptomatic patients after they have been informed about the operative mortality, morbidity, and chances of palliation. Decisions about treatment of advanced AMM should be guided predominantly by the patient's preferences.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Benbassat J,Gilon D,Penchas Sdoi
10.1002/ajh.2830330210subject
Has Abstractpub_date
1990-02-01 00:00:00pages
128-35issue
2eissn
0361-8609issn
1096-8652journal_volume
33pub_type
杂志文章,评审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 f...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10202
更新日期:2002-10-01 00:00:00
abstract::Autologous 51Cr-platelet kinetic studies were performed in splenectomized mongrel dogs. Mean survival time of PRP-platelets was 5.4 +/- 1.5 (SD) days (n = 6). The curves, though slightly curvilinear, showed mostly a linear type of decay, denoting that platelet removal from the circulation is mainly determined by aging...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830170407
更新日期:1984-01-01 00:00:00
abstract::An adolescent male patient developed functional asplenia in the course of graft versus host disease (GVHD) after successful allogeneic bone marrow transplantation (BMT) for aplastic anemia. Coincident with the onset of the asplenia, amelioration of the ongoing GVHD was observed. Unexpectedly, after 34 months of functi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120112
更新日期:1982-02-01 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::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::Pure red cell aplasia (PRCA) sometimes accompanies thymoma. Herein, we report a PRCA patient with thymoma with a clonal disorder of T cells. A 55-year-old man presented with anemia and anterior mediastinum tumor. The laboratory study revealed hemoglobin 8.2 g/dl; leukocytes 15.8 x 10(9)/L with 76.5% neutrophils, 20.0%...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199704)54:4<324::aid-ajh12
更新日期:1997-04-01 00:00:00
abstract::Autoimmune hemolytic anemia is thought to be mediated via auto-antibodies produced by lymphoid B cells. This may be an idiopathic process or secondary to an underlying infection or lymphoproliferative disorder. Conventional treatment comprises immunosuppression with corticosteroids and, in some cases, splenectomy. A p...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20220
更新日期:2005-02-01 00:00:00
abstract:DISEASE OVERVIEW:Chronic neutrophilic leukemia (CNL) is a rare, often aggressive myeloproliferative neoplasm (MPN) defined by persistent mature neutrophilic leukocytosis, bone marrow granulocyte hyperplasia, and frequent hepatosplenomegaly. The seminal discovery of oncogenic driver mutations in colony-stimulating facto...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25688
更新日期:2020-02-01 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::"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::The role of reduced-intensity conditioning (RIC) in adult acute lymphoblastic leukemia (ALL) remains unclear because of the small sample size, short follow-up duration, various regimens for conditioning and graft-versus-host disease (GVHD) prophylaxis, and the heterogeneity of selection criteria for transplantation. W...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.23465
更新日期:2013-08-01 00:00:00
abstract::In thalassemia, fetal hemoglobin (HbF) augmentation with hydroxycarbamide (also known as hydroxyurea) is not always successful. The expected parallel effects on red cell (RBC) membrane deformability, cell hydration, and membrane phospholipid organization, all important for extending RBC life span and increasing Hb, ha...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21266
更新日期:2008-11-01 00:00:00
abstract::Although large vessel thrombi are occasionally reported in patients with homozygous sickle cell disease, the role of intravascular coagulation in typical pain crises is controversial. Therefore, we studied 24 sickle cell patients during and between episodes of pain crisis, using several sensitive tests of hemostasis. ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830230403
更新日期:1986-12-01 00:00:00
abstract::The objective of this case-matched study was to compare the efficacy and toxicity of the addition of clarithromycin (Biaxin) to lenalidomide/low-dose dexamethasone (BiRd) vs. lenalidomide/low-dose dexamethasone (Rd) for newly diagnosed myeloma. Data from 72 patients treated at the New York Presbyterian Hospital-Cornel...
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.21777
更新日期:2010-09-01 00:00:00
abstract::Fetal hemoglobin (HbF) inhibits the polymerization of sickle hemoglobin, modulating the clinical features of sickle cell anemia (SCA). Hydroxyurea (HU) therapy can increase the HbF level, although its production can be influenced by genetic determinants. Twenty-two Brazilian SCA patients were evaluated over 5 years be...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20293
更新日期:2005-03-01 00:00:00
abstract::The incidence of drug-induced neutropenia has not changed in the western hemisphere over the last 30 years. Yet, the drug panorama has changed considerably. This implies that host factors may play an intriguing role for this idiosyncratic reaction. The knowledge as to mechanisms for the reaction has advanced with emer...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21433
更新日期:2009-07-01 00:00:00
abstract::Variable survival outcomes are seen following treatment for aggressive non-Hodgkin lymphoma (NHL). This study examined whether outcomes for aggressive B-cell NHL are associated with single nucleotide polymorphisms (SNPs) in oxidative stress-related genes, which can alter drug metabolism and immune responses. Genotypes...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23709
更新日期:2014-06-01 00:00:00
abstract::Patients initially diagnosed with type 1 von Willebrand disease (VWD) have been reclassified as type 2 after a more exhaustive analysis in several studies. Our study's objectives were (1) to reanalyze patients that were previously diagnosed as type 1 to achieve a more accurate diagnosis and (2) to compare the von Will...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20470
更新日期:2005-11-01 00:00:00
abstract::FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been i...
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.25945
更新日期:2020-11-01 00:00:00
abstract::In addition to transferrin, ferritin can also function as a source of iron for heme synthesis (Gelvin D, et al. Blood 1996;88:3200-3207; Meyron-Holtz EG, et al. Blood 1999;94:3205-3211). In the present study we investigate the effect of external ferritin on the proliferation and hemoglobinization of human erythroid pr...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10355
更新日期:2003-07-01 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::Autologous blood doping refers to the illegal re-transfusion of any quantities of blood or blood components with blood donor and recipient being the same person. The re-transfusion of stored erythrocyte concentrates is particularly attractive to high-performance athletes as this practice improves their oxygen capacity...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.26078
更新日期:2020-12-16 00:00:00
abstract::The binding of anti-human platelet glycoprotein (GP) IIbIIIa monoclonal antibodies to human umbilical vein endothelial cells (HUVE) was studied. Scatchard analysis using 125I-anti-platelet GPIIb-IIIa monoclonal antibody showed that the maximum binding capacity (B max) was 8 X 10(4)/cell and Kd was 40.2 nM. The binding...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830250113
更新日期:1987-05-01 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::Evidence suggest that even patients aged 70 or above benefit from specific AML therapy. The fundamental decision in AML then becomes whether to recommend standard or investigational treatment. This decision must rest on the likely outcome of standard treatment. Hence we review factors that predict treatment related mo...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24439
更新日期:2016-08-01 00:00:00
abstract::The recent progress in sensitive KIT D816V mutation analysis suggests that mutation analysis of peripheral blood (PB) represents a promising diagnostic test in mastocytosis. However, there is a need for systematic assessment of the analytical sensitivity and specificity of the approach in order to establish its value ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23672
更新日期:2014-05-01 00:00:00
abstract::Lysosomes (granules) of rabbit PMN leukocytes were extracted with either HCl or H2SO4, and the extracts were chromatographed over Sephadex to separate protein constituents. Some of the low molecular weight cationic proteins homogeneous on SDS PAGE (8% and 12.5% gels) were characterized by electrophoretic mobility in a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830070309
更新日期:1979-01-01 00:00:00
abstract::We present two patients with human immunodeficiency virus-1 (HIV-1) infection in whom T-cell non-Hodgkin lymphoma developed, based on pathologic diagnosis, immunophenotyping, and T-cell receptor gene rearrangement. Both cases were positive for human immunodeficiency virus-1 by enzyme-linked immunosorbent assay and imm...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830310307
更新日期:1989-07-01 00:00:00
abstract::Granulocyte macrophage-colony-stimulating factor (GM-CSF) has shown promise as a means of alleviating leukopenia associated with a wide variety of disorders. It is currently undergoing evaluation as an adjunct to bone marrow transplantation but its use in patients with metabolic disorders, such as Hurler's syndrome (H...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830410411
更新日期:1992-12-01 00:00:00
abstract::Current information about clinical significance of IDH mutations in myelodysplastic syndromes (MDS), their association with other genetic alterations and the stability during disease progression is limited. In this study, IDH mutations were identified in 4.6% of 477 patients with MDS based on the FAB classification an...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23596
更新日期:2014-02-01 00:00:00