Abstract:
:The introduction of Imatinib (IM) has significantly altered the treatment for CML, although only limited follow-up results are available. As failure of Interferon-alpha had been associated with poor prognosis and results of IM-treatment in this patient group may allow earlier estimation of long-term benefits for early chronic phase patients. Therefore we prospectively analyzed the quality and duration of remissions and the rate of BCR-ABL resistance mutations occurring in patients treated with IM, if they were intolerant or refractory to interferon. Fifty-nine patients were included and median follow up is 4.75 years. Haematologic remission rate was 92% and 62% of patients achieved at least major cytogenetic remission. There were no major differences between patients failing or being refractory to IFN. Major molecular response was observed in 67% of patients evaluable. For the entire group, median PFS was 4.3 years and OS was 82% at 4.75 years. Haematologic and complete cytogenetic remissions, but not molecular responses were correlated with improved PFS and OS. In patients with progression or inadequate response, BCR-ABL kinase domain mutations were detected in 3/28 patients. IM resulted in prolonged remission rates in a substantial proportion after IFN-failure/intolerance, with no differences in these patient groups. Quality of hematologic and cytogenetic, but not molecular response was associated with duration of remissions and survival. A low rate of resistance-mutations was detected, suggesting that alternative mechanism may play an important role in disease progression.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Hess G,Meyer RG,Schuch B,Bechthold K,El-Kholy I,Huber Cdoi
10.1002/ajh.21055subject
Has Abstractpub_date
2008-03-01 00:00:00pages
178-84issue
3eissn
0361-8609issn
1096-8652journal_volume
83pub_type
杂志文章abstract::Reverse transcription quantitative polymerase chain reaction (RTqPCR)is currently the most sensitive tool available for the routine monitoring of disease level in patients undergoing treatment for BCRABL1 associated malignancies. Considerable effort has been invested at both the local and international levels to stand...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23212
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abstract::We present a case of acquired von Willebrand syndrome (AVWS) due to a monoclonal gammopathy of undetermined significance. Initially this case was diagnosed as congenital von Willebrand disease (VWD); however, re-examination of the medical history rendered a congenital bleeding disorder unlikely. A normal plasma von Wi...
journal_title:American journal of hematology
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abstract::In light of the relationship between immune system dysregulation and multiple myeloma (MM) risk, we investigated whether genetic variation in 92 immune function genes among 77 gene regions are associated with MM susceptibility in a population-based case-control study (108 cases and 482 controls) conducted among Caucas...
journal_title:American journal of hematology
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abstract::Extramedullary meningeal hematopoiesis (EMH) represents an uncommon finding after stem-cell transplantation. We describe the case of an allogeneic bone marrow transplantation (BMT) recipient who developed EMH 1 month after radiation myelitis had been diagnosed. A 39-year-old man with multiple myeloma underwent matched...
journal_title:American journal of hematology
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abstract::Transferrin-iron uptake by peripheral blood monocytes was studied in vitro to test the hypothesis that the relative paucity of mononuclear phagocyte iron loading in hereditary hemochromatosis results from a defect in uptake of iron from transferrin. Monocytes from nine control subjects and 17 patients with hemochromat...
journal_title:American journal of hematology
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doi:10.1002/ajh.2830160405
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abstract::Therapy of Hodgkin lymphoma (HL) is designed to prolong survival and minimize toxicity. A total of 124 patients with newly diagnosed HL and adverse prognostic factors were prospectively studied between July, 1999 and August, 2005. Patients with early unfavorable and advanced disease were eligible for the study. Patien...
journal_title:American journal of hematology
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abstract::Erythrocytes from ten patients with severe liver disease displayed low methylene blue-stimulated hexose monophosphate (HMP) shunt activity and glucose recycling despite elevated total glucose consumption when compared to controls. Heinz body formation was increased and reduced glutathione concentration significantly d...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830060403
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abstract::In a phase II trial, we evaluated chlorambucil and rituximab (CLB-R) as first-line induction treatment with or without R as maintenance for elderly chronic lymphocytic leukemia (CLL) patients. Treatment consisted of eight 28-day cycles of CLB (8 mg/m(2) /day, days 1-7) and R (day 1 of cycle 3, 375 mg/m(2) ; cycles 4-8...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
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abstract::Defective fibrinolysis due to decreased tissue-type plasminogen activator (t-PA) activity is a well-established finding in patients with systemic lupus erythematosus (SLE). The possibility that this decrease in t-PA activity may be related to the presence of autoantibodies directed against t-PA, and the possible role ...
journal_title:American journal of hematology
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doi:10.1002/ajh.2830490203
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journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830170310
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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::Thrombocytopenia is a poor prognostic indicator in the myelodysplastic syndromes (MDS). Treatment options for patients with symptomatic thrombocytopenia are limited. Danazol, an attenuated androgen, may have some efficacy in increasing the platelet count of patients with MDS. We retrospectively reviewed 33 patients wi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10209
更新日期:2002-11-01 00:00:00
abstract::The requirements for factor VIII (AHF) or factor IX (PTC) of hemophilic patients undergoing dental extractions were evaluated to determine the minimum effective regimen. Sixteen patients underwent 19 operative procedures. The mean total dose of factor VIII or IX was 45.8 U/kg for 11 procedures with preoperative replac...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830160102
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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
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abstract::Reduced ferrochelatase activity in erythropoietic protoporphyria (EPP) causes the accumulation of protoporphyrin IX (PPIX) leading to acute cutaneous photosensitivity and liver injury. Many EPP patients also have a mild hypochromic, microcytic anemia and iron deficiency. Iron deficiency can lead to decreased PPIX accu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25743
更新日期:2020-05-01 00:00:00
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-formi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280105
更新日期:1988-05-01 00:00:00
abstract::Duvelisib (IPI-145), an oral, dual inhibitor of phosphoinositide-3-kinase (PI3K)-δ and -γ, was evaluated in a Phase 1 study in advanced hematologic malignancies, which included expansion cohorts in relapsed/refractory (RR) chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) and treatment-naïve (TN) CLL...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.25243
更新日期:2018-11-01 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
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abstract::Splenectomy is a time-honoured well established approach for patients with steroid-resistant immune thrombocytopenia (ITP). However, due to the more recent availability of therapeutic options alternative to splenectomy, such as rituximab and agonists of the thrombopoietin-receptor, the choice of second-line therapy is...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23823
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abstract::The activation pathways for the generation of enzymes involved in blood clotting, clot lysis, complement activation, and kinin generation are briefly reviewed. The interrelationship of the four systems is illustrated by the multiple functions of four key enzymes: Factor XIIa, kallikrein, plasmin, and C1 esterase. The ...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830040412
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abstract::Transformation to secondary myelofibrosis (MF) occurs as part of the natural history of polycythemia vera (PPV-MF) and essential thrombocythemia (PET-MF). Although primary (PMF) and secondary MF are considered similar diseases and managed similarly, there are few studies specifically focused on the latter. The aim of ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
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abstract::It has been suggested that alterations of cell cycle genes probably contribute to the pathogenesis of endemic Burkitt's lymphoma (BL) in addition to c-MYC translocation. Mutations disrupting the normal nuclear localization signal of the retinoblastoma-related gene Rb2/p130 have been documented in BL cell lines and pri...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20749
更新日期:2007-03-01 00:00:00
abstract::Most cases of immune hemolytic anemia are associated with a positive direct antiglobulin test. However, in some cases, the antiglobulin test is not sensitive enough to detect low levels of red-cell bound antibodies. This report describes a method using radiolabelled purified staphylococcal protein A which is capable o...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120405
更新日期:1982-06-01 00:00:00
abstract::We studied levels of plasma endothelin-1 (ET-1) in 25 beta-thalassemia/Hb E patients before and after blood transfusion. Baseline ET-1 levels in these patients were significantly higher than in normal controls (10.17 +/- 2.1 pg/mL vs. 8.9 +/- 2.0 pg/mL, P < 0.05). After blood transfusion, levels of plasma ET-1 tend to...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10129
更新日期:2002-07-01 00:00:00
abstract::Bacterial and protozoal infections can cause thrombocytopenia and may mimic idiopathic thrombocytopenic purpura (ITP). Brucella species and Toxoplasma are among the infectious agents with protean clinical manifestations which may induce immune thrombocytopenia. In rare cases, thrombocytopenia can be severe and may res...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10382
更新日期:2003-09-01 00:00:00
abstract::At sites of vascular injury, the platelet collagen receptor Glycoprotein Ia/IIa (GPIa/IIa) acts as an important mediator of platelet adhesion to fibrillar collagens. Two silent polymorphisms (807C/T and 873G/A) within the glycoprotein Ia gene have been implicated in increased risk of developing thrombosis and myocardi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10029
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abstract::We recently defined event-free survival at 24 months (EFS24) as a clinically relevant outcome for patients with DLBCL. Patients who fail EFS24 have very poor overall survival, while those who achieve EFS24 have a subsequent overall survival equivalent to that of the age- and sex-matched general population. Here, we de...
journal_title:American journal of hematology
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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
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abstract::Hb Knossos is a beta-chain variant (beta 27 Ser----Ala) that is unrecognizable by conventional separation methods but detectable by globin electrophoresis on urea-Triton X-acrylamide gels or by IEF. Hb Knossos is characterized by reduced synthesis and by interaction with beta-thalassemia, in which the double heterozyg...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830210202
更新日期:1986-02-01 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