Abstract:
:Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal stem-cell disorder in which blood cells lack complement inhibiting membrane proteins, and become susceptible to complement-mediated injury, leading to chronic intravascular hemolysis and pancytopenia. Glucocorticoids have been a mainstay of therapy. For patients refractory to glucocorticoids and requiring blood transfusions, an alternative therapy is needed. We studied danazol therapy in 5 patients refractory to other treatments. Four of the 5 benefited, showing rise in hematocrit and eventual cessation of transfusion requirements. Remissions lasted > or =2 years in 3 and 10 years in 1 patient. Danazol was well-tolerated without serious side effects. Danazol appears to be a good alternative treatment in PNH.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Harrington WJ Sr,Kolodny L,Horstman LL,Jy W,Ahn YSdoi
10.1002/(sici)1096-8652(199702)54:2<149::aid-ajh9>subject
Has Abstractpub_date
1997-02-01 00:00:00pages
149-54issue
2eissn
0361-8609issn
1096-8652pii
10.1002/(SICI)1096-8652(199702)54:2<149::AID-AJH9>journal_volume
54pub_type
杂志文章,评审abstract::Eight patients with severe chronic autoimmune thrombocytopenic purpura (AITP) refractory to high-dose intravenous immunoglobulin (IVIgG) and/or oral prednisone were treated with one to three infusions of high-dose methylprednisolone (HDMP) (15 mg/kg/day). The mean platelet count before treatment was 12 +/- 10 x 10(9)/...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830480416
更新日期:1995-04-01 00:00:00
abstract::In order to study the influence of oxidized low-density lipoprotein (Ox-LDL) on platelet functional morphology at an early activation stage, washed human blood platelets were stimulated by 100 micrograms/ml Ox-LDL at 37 degrees C. The settling and spreading process of stimulated and unstimulated platelets on Formvar-c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490302
更新日期:1995-07-01 00:00:00
abstract::Chronic transfusion therapy is the treatment of choice for preventing stroke recurrence in children with sickle cell disease (SCD). The majority of children affected by this devastating complication live in the developing world where access to regular blood transfusions may be impractical. Since 2000, in the absence o...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.22142
更新日期:2011-10-01 00:00:00
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
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830370110
更新日期:1991-05-01 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::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::Ticlopidine-induced aplastic anemia (TIAA) is considered very uncommon. We present two new cases, and we review 55 additional cases from the literature. The first case concerns a 70-year-old man who developed severe aplastic anemia 7 weeks after treatment with 500 mg of ticlopidine daily. The patient sustained a sever...
journal_title:American journal of hematology
pub_type: 杂志文章,meta分析,评审
doi:10.1002/ajh.10150
更新日期:2002-09-01 00:00:00
abstract::This study was designed to explore the value of markers of bone turnover, macrophage inflammatory protein-1alpha (MIP-1alpha), and osteopontin (OPN) in the diagnosis of myeloma bone disease. Twenty-five patients with newly diagnosed and untreated multiple myeloma (MM), and 22 age-, sex-, and bone mineral density-match...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20794
更新日期:2007-03-01 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::Various in vitro studies and clinical observations suggest that Fanconi's anemia (FA) patients are unable to detoxify adequately superoxide anions (O2-) released by activated phagocytes. Recent studies have shown that certain lymphokines such as tumor necrosis factor-alpha (TNF-alpha) and interferon-gamma (IFN-gamma) ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830420211
更新日期:1993-02-01 00:00:00
abstract::We studied the growth of erythroid burst-forming units (BFU-E) and erythroid colony forming units (CFU-E) from bone marrow and blood in six patients with erythroleukemia. Five patients grew CFU-E, while BFU-E were found in the marrow of two and in the peripheral blood of only one patient. In all cases with colony grow...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830050403
更新日期:1978-01-01 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
pub_type: 杂志文章
doi:10.1002/ajh.23226
更新日期:2012-09-01 00:00:00
abstract:OBJECTIVE:To assess the efficacy of intravenous immunoglobulin (IVIG), in comparison with plasma exchange (PE), in the treatment of patients with thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS). DESIGN:Prospective, nonrandomized comparative study. SETTING:Hematology department in a general hos...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830410305
更新日期:1992-11-01 00:00:00
abstract::Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830310105
更新日期:1989-05-01 00:00:00
abstract::We have generated a murine hybridoma that secretes a monoclonal antibody (mAb) that is highly specific for hemoglobin C (HbC) [alpha 2 beta 2 6(A3)Glu----Lys] and shows no cross reactivity with HbA, HbA2, HbF, HbS, HbE, or Hb O-Arab. Using this antibody, we developed a simple and rapid enzyme linked immunosorbent assa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830330308
更新日期:1990-03-01 00:00:00
abstract::Therapy-related acute myeloid leukemia (t-AML) arises as a late complication following antecedent solid tumors or hematologic diseases and their associated treatments. There are limited data regarding risk factors and outcomes following allogeneic hematopoietic cell transplantation (HCT) for t-AML following a prior so...
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.25395
更新日期:2019-04-01 00:00:00
abstract::Thrombocytopenia developing in the course of chemotherapy for malignant disease is usually attributed to drug-induced marrow suppression and/or marrow replacement by tumor. We describe two patients who developed severe thrombocytopenia and hemorrhagic symptoms while being treated with oxaliplatin, 5-fluorouracil, and ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20516
更新日期:2006-03-01 00:00:00
abstract::Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 x 10(9)/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor th...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.10255
更新日期:2003-02-01 00:00:00
abstract::Chronic myeloid leukemia (CML) is considered to be a pleuripotential stem cell disorder with the capacity to differentiate into myeloid, erythroid, megakaryocytic, and lymphoid cell lines. Consequently, blast crisis (BC) involving each of the above lineages has been well described. Among lymphoblastic crises, differen...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830360204
更新日期:1991-02-01 00:00:00
abstract::We studied 15 patients with essential thrombocythemia (ET) before treatment and after normalization of platelet count by anagrelide. Significantly increased plasma levels of PDGF, TGFbeta, and bFGF were found. Patients with mild reticulin fibrosis in bone marrow had higher PDGF levels. During treatment, plasma TGFbeta...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.10091
更新日期:2002-06-01 00:00:00
abstract::We have studied 91 patients with SS genotype, 44 children and 47 adults. Excluding the Cameroon and atypical haplotypes, the distribution in the children's sample exhibited 43% Benin, 38% Bantu, and 3% Senegal. In adults, the sample exhibited 46% Benin, 30% Bantu, and 9% Senegal (chi 2: 13.511, 2 df, P = 0.001). When ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490212
更新日期:1995-06-01 00:00:00
abstract::Sickle cell disease is a single amino acid molecular disorder of hemoglobin leading to its pathological polymerization, red cell rigidity that causes poor microvascular blood flow, with consequent tissue ischemia and infarction. The manifestations of this disease are protean.Among them, leg ulcers represent a particul...
journal_title:American journal of hematology
pub_type:
doi:10.1002/ajh.21838
更新日期:2010-10-01 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
doi:10.1002/ajh.23729
更新日期:2014-07-01 00:00:00
abstract::Hb Saint Nazaire [beta 103 (G5) Phe-->Ile] was found in four apparently unrelated French families. The five patients carrying this hemoglobin have been detected because of a moderate erythrocytosis. The structural abnormality of Hb Saint Nazaire concerns the same residue as in Hb Heathrow [beta 103 (G5) Phe-->Leu). A ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830440105
更新日期:1993-09-01 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::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
pub_type: 杂志文章
doi:10.1002/ajh.2830350205
更新日期:1990-10-01 00:00:00
abstract::Cutaneous lymphoma is a disease characterized with massive skin infiltration of lymphoid malignant cells. They commonly express some T-cell markers, such as CD2, CD3, CD4, and CD7, and thus termed as CTCL (cutaneous T cell lymphoma). Here, we present a case with CD56/N-CAM-positive cutaneous lymphoma, which appears ly...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470406
更新日期:1994-12-01 00:00:00
abstract::Over a 36-month period, 46 consecutive Mexican mestizos with a clinical marker associated with a primary hypercoagulable state were prospectively assessed by searching for the sticky platelet syndrome (SPS), the activated protein C resistance (aPCR) phenotype, coagulation protein C activity and antigen, coagulation pr...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20233
更新日期:2005-01-01 00:00:00
abstract::To investigate the mechanisms that modulate granulocyte-macrophage colony-stimulating activity (GM-CSA) and burst promoting activity (BPA) elaboration, we studied human peripheral blood-derived monocyte-macrophage (M0) and T-lymphocyte (TL) interaction. Coincubation of live M0 with autologous TL at a 1:3 ratio in the ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120412
更新日期:1982-06-01 00:00:00
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