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) can significantly enhance O2- production by phagocytic cells. To ascertain lymphokine production in FA patients, we measured TNF-alpha and IFN-gamma production in vivo and in vitro. TNF-alpha was detected in the plasma of 16 of 18 FA patients with concentrations ranging from 6 to 131 pg/ml (mean 31 pg/ml). TNF-alpha was detected in only one of 25 control (healthy donor) plasma, and the level was very low (7 pg/ml). IFN-gamma levels in normal and patient plasma were negligible. Spontaneous and phytohemagglutinin (PHA)-induced production of IFN-gamma and TNF-alpha by cultured peripheral blood mononuclear cells did not differ significantly between FA patients and normal controls. The significance of overproduction of TNF-alpha in vivo in the pathophysiology of FA is discussed.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Schultz JC,Shahidi NTdoi
10.1002/ajh.2830420211subject
Has Abstractpub_date
1993-02-01 00:00:00pages
196-201issue
2eissn
0361-8609issn
1096-8652journal_volume
42pub_type
杂志文章abstract:DISEASE OVERVIEW:Approximately one-fourth of primary cutaneous lymphomas are B-cell derived and are generally classified into three distinct subgroups: primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCD...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25970
更新日期:2020-08-20 00:00:00
abstract::Hypereosinophilic syndrome (HES) is a rare disorder that can manifest in various organ systems. We report the case of a 54-year-old woman with a remote history of seizure disorder who presented with early signs of right-sided heart failure. Laboratory studies showed significant eosinophilia (8 x 10(9) l(-1)). Computed...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20933
更新日期:2007-10-01 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::Regulatory T-cells (Tregs) are increased in chronic lymphocytic leukemia(CLL) and correlates with clinical and biological features of active/progressive disease. However, little is known about their ability to predict the time to first treatment (TFT). We evaluated 75 patients with Rai stage 0 CLL, in whom the absolut...
journal_title:American journal of hematology
pub_type: 信件
doi:10.1002/ajh.23170
更新日期:2012-06-01 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::We measured the plasma level of fibrinogen in 560 patients with disseminated intravascular coagulation (DIC) and evaluated its relationship with outcome and with other hemostatic markers. Forty-seven percent of patients had >200 mg/dL of plasma fibrinogen and 24% had <100 mg/dl of plasma fibrinogen, suggesting that pl...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10249
更新日期:2003-01-01 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::Achievement of a complete response has been associated with improved outcomes in patients with multiple myeloma. Recently, increasing application of minimal residual disease (MRD) assessment has shown that MRD negativity is a powerful prognostic factor for survival outcomes. We wanted to examine the impact of the poly...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.25481
更新日期:2019-07-01 00:00:00
abstract::Recombinant DNA technology and protein engineering are creating hope that we can address ongoing challenges in hemophilia care such as reducing the costs of therapy, increasing the availability to the developing world, and improving the functional properties of these proteins. Technological advances to improve the hal...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23146
更新日期:2012-05-01 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
pub_type: 杂志文章
doi:10.1002/ajh.22189
更新日期:2012-01-01 00:00:00
abstract::Cyclooxygenases (COX) are key enzymes in the conversion of arachidonic acid to prostaglandins. Several studies have shown a relation between angiogenesis and COX-2 expression. Elevated expression of cyclooxygenase-2 (COX-2), however, has not been reported in multiple myeloma (MM) in the literature. The aim of this stu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20460
更新日期:2005-11-01 00:00:00
abstract::Trimethoprim and sulfamethoxazole (Bactrim r) is a widely used antibiotic combination effective against a broad spectrum of microbial organisms. There are reports of neutropenia developing during even brief periods of oral therapy, particularly in individuals with either folate deficiency or increased folate requireme...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830230102
更新日期:1986-09-01 00:00:00
abstract::We report here a patient with de novo acute myelocytic leukemia with trilineage myelodysplasia (AML/TMDS) in whom cytogenetic analysis was normal at diagnosis and in whom Philadelphia chromosome (Ph1) with monosomy 7 emerged at the terminal stage of the disease. Reverse transcription polymerse chain reaction (RT-PCTR)...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490413
更新日期:1995-08-01 00:00:00
abstract::By using Dexter-type long-term marrow cultures (D-LTMC), it has been shown previously that hematopoietic progenitor cells (HPC) from patients with aplastic anemia (AA) have a deficient proliferation in vitro. The studies reported to date, however, have focused exclusively on granulomonocytic progenitors and no informa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199810)59:2<149::aid-ajh8>
更新日期:1998-10-01 00:00:00
abstract::To monitor successful engraftment after bone marrow transplantation, we performed Southern hybridization analysis or dot blot analysis of DNA in a set of sex-mismatched cases using a Y-chromosome-specific DNA probe (pHY10). This method was extremely sensitive and rapid for checking which cells contain the Y-chromosome...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830270108
更新日期:1988-01-01 00:00:00
abstract::Most previous studies on telomere length (TL) in chronic lymphocytic leukemia (CLL) are based on referral cohorts including a high proportion of aggressive cases. Here, the impact of TL was analyzed in a population-based cohort of newly diagnosed CLL (n = 265) and in relation to other prognostic markers. Short telomer...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.23466
更新日期:2013-08-01 00:00:00
abstract::We describe a unique case of adult T-cell leukemia/lymphoma (ATL). The patient had typical clinicohematological features as ATL, but showed a lack of antibody to human T-cell leukemia virus type-1 (HTLV-1) and was negative for HTLV-1 proviral DNA in the peripheral mononuclear cells by means of polymerase chain reactio...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(200005)64:1<64::aid-ajh11>
更新日期:2000-05-01 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...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830430211
更新日期:1993-06-01 00:00:00
abstract::Immunomodulatory drugs (IMiDs) are a cornerstone in the treatment of multiple myeloma (MM), but specific markers to predict outcome are still missing. Recent work pointed to a prognostic role for IMiD target genes (e.g. CRBN). Moreover, indirect activity of IMiDs on immune cells correlated with outcome, raising the po...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24634
更新日期:2017-03-01 00:00:00
abstract::Liquid culture of the mononuclear fraction of the blood of marmosets without any added stimulator resulted in the appearance of megakaryocytes. These cells could not be identified by light microscopy or by indirect immunofluorescence using antiplatelet antiserum. Ultrastructural identification was made using platelet ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830080108
更新日期:1980-01-01 00:00:00
abstract::Relapsed or refractory acute myeloid leukemia (R/R AML) has a poor prognosis and is best treated with salvage chemotherapy as a bridge to allogeneic stem cell transplant (alloSCT). However, the optimal salvage therapy remains unknown. Here we compared two salvage regimens; mitoxantrone, etoposide, and cytarabine (MEC)...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.25838
更新日期:2020-08-01 00:00:00
abstract::Red cell ferritin was evaluated in 101 individuals with heterozygous beta-thalassemia to determine its clinical utility as an index for iron deficiency or overload in these subjects. The mean red cell ferritin for the total population was elevated threefold and showed a significant correlation with transferrin saturat...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830300403
更新日期:1989-04-01 00:00:00
abstract::Pentosan polysulfate is a low-molecular-weight sulfated polysaccharide used as an antithrombotic drug. We present two patients who developed thrombocytopenia and venous thrombosis during treatment with pentosan polysulfate. The relationship between pentosan polysulfate and thrombocytopenia is supported by platelet agg...
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::We evaluated possible prognostic factors just before salvage therapy with vincristine, doxorubicin, and dexamethasone (VAD) for 36 patients with refractory multiple myeloma. The median duration from diagnosis to the first VAD salvage was 14 months (range 2-76 months). Among parameters that have been shown to be associ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200010)65:2<132::aid-ajh7>3.0.co
更新日期:2000-10-01 00:00:00
abstract::In acute myeloid leukemia (AML), new prognostic tools are needed to assess the risk of relapse. Hematogones (HGs) are normal B-lymphocyte precursors that increase in hematological diseases and may influence remission duration in AML. HG detection was prospectively investigated in 262 AML patients to determine its prog...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24350
更新日期:2016-06-01 00:00:00
abstract::Variation in bleeding in the perioperative period is a complex and multifactorial event associated with immediate and delayed consequences for the patient and health care resources. Little is known about the complex genetic influences on perioperative bleeding. With the discovery of multiple variations in the human ge...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21205
更新日期:2008-09-01 00:00:00
abstract::Drug metabolizing genes are involved in the detoxification of chemical carcinogens. Polymorphisms in drug-metabolizing genes affect the risk of some forms of cancer. We analyzed six polymorphisms to evaluate their association with risk for non-Hodgkin lymphoma (NHL), and to examine whether smoking modifies these assoc...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21556
更新日期:2009-12-01 00:00:00
abstract::A 36-year-old white man was found to have low erythrocyte acetylcholinesterase activity. Plasma cholinesterase activity was normal. The propositus had mild anemia and moderate elliptocytosis, but was asymptomatic. A sister, brother, father, and nephew were hematologically normal, but had slightly subnormal red cell ac...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830070403
更新日期:1979-01-01 00:00:00
abstract::Since platelets express both platelet-specific and class I HLA antigens, serum antiplatelet reactivity assessed by most platelet antibody techniques could be due to antibodies with either or both specificities. Flow cytometric analysis of sera for detection of antiplatelet antibody commonly employs a purified platelet...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830380411
更新日期:1991-12-01 00:00:00
abstract::Six patients, five with acute myeloid leukemia (AML) and one with a myelodysplastic syndrome (MDS), were found to have monosomy 7 by conventional cytogenetics at diagnosis. Repetitive DNA sequences from the heterochromatic region of human chromosomes 1 and 7 were used as probes for in situ hybridization experiments on...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830330208
更新日期:1990-02-01 00:00:00