Abstract:
:A patient with Philadelphia chromosome (Ph) chronic myelogenous leukemia (CML), in chronic phase, was treated with recombinant gamma-interferon (r gamma-IFN) in a phase I clinical trial. Prior to treatment, analysis of in vitro agar culture parameters indicated hyporesponsiveness of granulocyte-macrophage colony-forming cells (CFU-GM) to inhibition by prostaglandin E and acidic isoferritins and diminished expression of class II major histocompatibility complex (MHC) antigens (HLA-DR). Treatment was associated with no change in bone marrow cellularity or in the percentage of Ph cells. However, in vitro cultures of bone marrow cells showed a return to normal levels of both expression of CFU-GM class II antigen and of sensitivity to inhibition by prostaglandin E and acidic isoferritins which predicted and/or confirmed clinical response. Throughout the course of interferon therapy, white blood cell counts (WBC) and the percentage of bone marrow blast cells were maintained at normal levels. Onset of aggressive-phase disease was associated with increased WBC, an increase in bone marrow blast cells, a secondary chromosomal abnormality, loss of CFU-GM sensitivity to inhibition by putative negative growth regulators, and markedly diminished MHC class II antigen expression. Following a bone marrow transplant from a matched sibling, all hematologic parameters studied were found to be normal. These findings indicate that treatment with r gamma-IFN can modulate some of the abnormal growth characteristics of CFU-GM observed in CML.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Pelus LM,Vadhan-Raj Sdoi
10.1002/ajh.2830280105subject
Has Abstractpub_date
1988-05-01 00:00:00pages
21-6issue
1eissn
0361-8609issn
1096-8652journal_volume
28pub_type
杂志文章abstract::A rare association of congenital afibrinogenemia and hereditary protein C deficiency is described in a 37-year-old female who suffered from ischemic necrosis in the left first toe. The diagnosis of afibrinogenemia was assessed by the absence of fibrinogen in clotting and immunological assays. The diagnosis of heredita...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830410111
更新日期:1992-09-01 00:00:00
abstract::A case of a patient presenting with idiopathic concurrent erythrocytic and megakaryocytic aplasia is reported. The patient's response to immunosuppressive therapy and her bone marrow pathology clearly suggest an immune mechanism. Based on the lack of suppression of erythroid colony growth, several mechanisms are postu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199601)51:1<68::AID-AJH11>
更新日期:1996-01-01 00:00:00
abstract::In patients with acute myeloid leukemia (AML), the presence of residual disease at day 14 after primary induction therapy warrants consideration of a second induction cycle. However, data to guide retreatment decisions in such patients are presently limited. Here, we retrospectively reviewed data from 176 patients wit...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24627
更新日期:2017-03-01 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::Eosinophilia associated with solid tumors is an infrequent occurrence. The pathogenesis of tumor-associated eosinophilia is not well understood. Interleukin-5 (IL-5) is a cytokine that has been implicated in the development of eosinophilia in mice and humans. However, there is little data associating IL-5 production w...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20789
更新日期:2007-03-01 00:00:00
abstract::The clinical development of lenalidomide (Revlimid™), then pomalidomide (Actimid™) as members of immunomodulatory drugs (IMiDs) for the treatment of multiple myeloma (MM), exemplifies how insight into disease biology can lead to design of effective therapeutic agents. Increased experience and understanding of IMiD's d...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23234
更新日期:2012-12-01 00:00:00
abstract::Precursor B-ALL blasts may be positive for CD20 in up to 50% of cases. There are few reports on the use of the anti CD20 monoclonal antibody, rituximab, in children with B-ALL. We report on two adult patients with precursor B-ALL who developed significant hepatic toxicity during induction chemotherapy. Single agent ri...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20675
更新日期:2006-12-01 00:00:00
abstract::The efficacy of the three common intra- and extragenic polymorphic sites of the factor VIII and IX genes has been examined in the Indian population, with an aim to develop a strategy that would be accurate and informative, yet economical. The approach for hemophilia A carrier detection includes tests for BclI, XbaI, a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199704)54:4<271::aid-ajh2>
更新日期:1997-04-01 00:00:00
abstract::Idiopathic thrombocytopenic purpura is an autoimmune disease which involves opsonization of platelets by autoantibodies directed against different surface glycoproteins, leading to their premature destruction by the reticuloendothelial system. Management of patients with refractory ITP is difficult. Recent studies hav...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20276
更新日期:2005-04-01 00:00:00
abstract::Aberrant promoter DNA methylation is a well-described mechanism of leukemogenesis within hematologic malignancies, including acute lymphoblastic leukemia (ALL). However, the importance of methylation patterns among the adolescent and young adult (AYA) ALL population has not been well established. DNA methylation of 18...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23511
更新日期:2013-09-01 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::Levels of many coagulation factors are low in the healthy infant and even lower in the asphyxiated premature infant. We investigated whether a brief exposure to asphyxia at the time of birth causes the activation and consumption of coagulation factors. Following delivery by caesarean section, premature lambs were asph...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280317
更新日期:1988-07-01 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::The second generation BCR/ABL kinase inhibitor nilotinib is increasingly used for the treatment of imatinib-resistant chronic myeloid leukemia (CML). So far, nilotinib is considered a well-tolerated drug with little if any side effects, although an increase in the fasting glucose level has been reported. We examined a...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.22037
更新日期:2011-07-01 00:00:00
abstract:DISEASE OVERVIEW:Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extracutaneous organs. DIAGNOSIS:The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23459
更新日期:2013-07-01 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::Severe pruritus is frequently associated with myeloproliferative and other systemic illnesses, and often fails to respond to conventional measures. We used danazol (Danocrine), a synthetic attenuated androgen, in the treatment of severe pruritus refractory to conventional therapy. Eight patients had myeloproliferative...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/(SICI)1096-8652(199602)51:2<112::AID-AJH3>
更新日期:1996-02-01 00:00:00
abstract::Lenalidomide and azacitidine are active in MDS patients, and may complement each other by targeting the bone marrow microenvironment and the malignant clone. A recent Phase I trial testing the lenalidomide and azacitidine combination yielded encouraging results; however, lenalidomide’s contribution was unclear. In thi...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.21891
更新日期:2011-01-01 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::Polycythemia vera (PV) and essential thrombocythemia (ET) are common types of myeloproliferative disorders (MPD), the prevalence of which has not been well documented in the United States. Recent breakthroughs in the molecular etiology of these disorders and the accelerated development of targeted pharmacotherapeutics...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21129
更新日期:2008-05-01 00:00:00
abstract::Chuvash polycythemia results from a homozygous 598C>T mutation in exon 3 of the von Hippel-Lindau (VHL) gene. This disrupts the normoxia pathway for degrading hypoxia inducible factor (HIF)-1alpha and HIF-2alpha causing altered expression of HIF-1 and HIF-2 inducible genes. As hypoxia induces expression of pro-inflamm...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21327
更新日期:2009-02-01 00:00:00
abstract::Iron refractory iron deficiency anemia (IRIDA) is a rare hereditary disease caused by mutations in TMPRSS6 gene encoding Matriptase-2, a negative regulator of hepcidin transcription. Up to now, 53 IRIDA patients from 35 families with different ethnic origins have been reported and 41 TMPRSS6 mutations have been identi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23929
更新日期:2015-04-01 00:00:00
abstract::Several studies provided evidence of a consistent antileukemic effect induced by cytomegalovirus (CMV) replication in acute myeloid leukemia (AML) patients receiving allogeneic hematopoietic stem cell transplantation (HSCT), however the use of antithymocyte globulin (ATG) as graft-versus-host disease prophylaxis, may ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23998
更新日期:2015-06-01 00:00:00
abstract::Community practice experience allows a nonselective care of patient using information derived from a more controlled clinical trial environment. We present our community experience with multiple myeloma patients with advanced age, long disease duration since diagnosis, advanced stage, multiple prior therapies includin...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20883
更新日期:2007-07-01 00:00:00
abstract::Cultured endothelial cells from bovine aorta were exposed to oxidized low density lipoprotein and examined by electron microscopy. The endothelial cells contracted slightly and the intercellular junctions became unclear. Some osmiophilic material increased in the cytoplasm. The oxidized low density lipoprotein appears...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490315
更新日期:1995-07-01 00:00:00
abstract::Gamma inferferon (gamma IFN), alpha tumor necrosis factor (alpha TNF), and interleukin 6 (IL-60) are cytokines produced by a wide variety of cells, including T lymphocytes and NK cells. These cytokines affect B-cell proliferation and differentiation into immunoglobulin secreting cells. In addition, gamma IFN and alpha...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490403
更新日期:1995-08-01 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::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::Because of a possible hazard from the use of radioisotopes to determine iron absorption by infants, the use of stable isotopes for this purpose has much appeal. We have applied the method of inductively coupled plasma mass spectrometry (ICP/MS) to determine the mass ratio, 58Fe/57Fe, in blood before and after oral adm...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830210307
更新日期:1986-03-01 00:00:00
abstract::Decreased von Willebrand factor (VWF)-cleaving protease activity (<5%) has been implicated in patients with congenital thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (Upshaw-Schulman syndrome) and associated with mutations within the ADAMTS13 gene. In this report, we describe longitudinal studies in a p...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20221
更新日期:2004-12-01 00:00:00