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 identified. One hundred and fifty-one patients with F/P+ MN-eo (143 males; mean age at diagnosis 49 years; mean annual incidence: 0.18 case per million population) were included in this retrospective nationwide study involving all French laboratories who perform the search of F/P fusion gene (study period: 2003-2019). The main organs involved included the spleen (44%), skin (32%), lungs (30%), heart (19%) and central nervous system (9%). Serum vitamin B12 and tryptase levels were elevated in 74/79 (94%) and 45/57 (79%) patients, respectively, and none of the 31 patients initially treated with corticosteroids achieved complete hematologic remission. All 148 (98%) IM-treated patients achieved complete hematologic and molecular (when tested, n = 84) responses. Forty-six patients eventually discontinued IM, among whom 20 (57%) relapsed. In multivariate analysis, time to IM initiation (continuous HR: 1,01 [0.99-1,03]; P = .05) and duration of IM treatment (continuous HR: 0,97 [0,95-0,99]; P = .004) were independent factors of relapse after discontinuation of IM. After a mean follow-up of 80 (56) months, the 1, 5- and 10-year overall survival rates in IM-treated patients were 99%, 95% and 84% respectively. In F/P+ MN-eo, prompt initiation of IM and longer treatment durations may prevent relapses after discontinuation of IM.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Rohmer J,Couteau-Chardon A,Trichereau J,Panel K,Gesquiere C,Ben Abdelali R,Bidet A,Bladé JS,Cayuela JM,Cony-Makhoul P,Cottin V,Delabesse E,Ebbo M,Fain O,Flandrin P,Galicier L,Godon C,Grardel N,Guffroy A,Hamidou M,doi
10.1002/ajh.25945subject
Has Abstractpub_date
2020-11-01 00:00:00pages
1314-1323issue
11eissn
0361-8609issn
1096-8652journal_volume
95pub_type
临床试验,杂志文章,多中心研究abstract::Leukemic cells of 43 patients with acute promyelocytic leukemia (M3) were investigated morphologically and cytochemically to determine the percentage of aberrant enzymes and whether or not the presence impacts on the clinical outcome. Twelve patients (27.9%) showed alpha-naphthyl acetate esterase (ANAE) activity in th...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830290306
更新日期:1988-11-01 00:00:00
abstract::Recent advances in massively parallel sequencing technologies have provided a detailed picture of the mutational landscape in CLL and underscored the vast degree of interpatient and intratumor heterogeneities. These studies have led to the characterization of novel putative driver genes and recurrently affected biolog...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24301
更新日期:2016-05-01 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::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::Corticosteroids are essential and one of the mainstays in the treatment of acute lymphoblastic leukemia (ALL). In vitro assays show that dexamethasone(DXM) is five to six times more cytotoxic to leukemic lymphoblasts than prednisolone (PDN) [1], and the use of DXM as an alternative drug for PDN is an important issue i...
journal_title:American journal of hematology
pub_type: 信件,随机对照试验
doi:10.1002/ajh.21827
更新日期:2010-10-01 00:00:00
abstract::The roles of fibrinogen and fibronectin were contasted in the responses of human platelets to Staphylococcus aureus and collagen. Congenital afibrinogenemic (CA) platelets and washed normal platelets had delayed aggregation due to a prolonged latent phase in response to contact with the bacteria when fibrinogen was ab...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830090106
更新日期:1980-01-01 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::The expression of mature B-cell markers and T markers was determined in lymphocytes isolated from the peripheral blood (PBL) of 20 healthy and 51 patients with non-Hodgkin malignant lymphoma (NHL). The disease was classified as newly diagnosed, in remission, or being treated with chemotherapy and of low-, intermediate...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830260203
更新日期:1987-10-01 00:00:00
abstract::In this study we describe the time-dependent effects of a high dose (750 micrograms/ml/24 hr) continuous infusion of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) on monocyte number, cytokine release, and superoxide anion production. Blood was taken from patients prior to rhGM-CSF infus...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830430410
更新日期:1993-08-01 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 improved microchromatographic procedure for the detection of Hb-S and/or Hb-C in cord blood at birth (Schroeder et al.: J. Lab Clin Med 86:528-532, 1975) as well as a modification thereof may also be used for the quantitative determination of Hb-F in the presence of Hb-S and/or Hb-C. However, Hb-A interferes and m...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830010308
更新日期:1976-01-01 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::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:DISEASE OVERVIEW:Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by stem cell-derived clonal myeloproliferation that is often but not always accompanied by JAK2, CALR, or MPL mutations; additional disease features include bone marrow stromal reaction including reticulin fibrosis, abnorm...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25230
更新日期:2018-12-01 00:00:00
abstract::Central nervous system (CNS) thrombotic events are a well-known complication of acute lymphoblastic leukemia (ALL) induction therapy, especially with treatments including l-asparaginase (l-ASP). Data on risk factors and clinical evolution is still lacking in adult patients. We report on the clinical evolution of 22 CN...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24130
更新日期:2015-11-01 00:00:00
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
更新日期:1979-01-01 00:00:00
abstract::A family is described in which venous thromboembolic disease is associated with reduced plasma protein C activity and normal levels of protein C antigen. Immunoelectrophoretic analysis of protein C antigen gave an abnormal pattern in all affected members, suggesting that the disorder is related to the presence of a st...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830290410
更新日期:1988-12-01 00:00:00
abstract::During 1971-1988, 194 adults with de novo acute myeloid leukemia (AML) received initial therapy at the University of Minnesota with an anthracycline-based regimen. Seventy-two of the 194 required further chemotherapy and received a second cycle of the same or similar therapy; 63 of these 72 were evaluable. For each ma...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470305
更新日期:1994-11-01 00:00:00
abstract::Langerhans cell histiocytosis (LCH) is an enigmatic histiocytic proliferative disorder of unknown etiology that affects children primarily. We have investigated the possibility that viruses are etiological or that they have a "triggering effect" in LCH. Sensitive in situ hybridization and polymerase chain reaction (PC...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830470104
更新日期:1994-09-01 00:00:00
abstract::The Sicilian type of (delta beta) (0)-thalassemia characterized by a approximately 13 kb deletion, was present in a Turkish boy who is a homozygote and in his heterozygous parents who are first cousins. The father with approximately 21% Hb F had five alpha-globin genes (alpha alpha/alpha alpha alpha) and the mother wi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830340316
更新日期:1990-07-01 00:00:00
abstract::Sickle cell (SS) disease is a complex of various genetic conditions. In some, homozygosity for the beta S gene may be present alone or in combination with the heterozygous or homozygous alpha-thalassemia-2 condition. Such combinations might ameliorate the clinical and hematological condition of the patient. The same m...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830060210
更新日期:1979-01-01 00:00:00
abstract::A patient is described who had blastic transformation of Ph1 negative chronic myelogenous leukemia (Ph1 - CML). Characterization of the leukemic cells revealed a population with a lymphoid stem cell phenotype (cALL-, TdT+, Ia+, cIgM-). This particular phenotype may be responsible for the refractoriness to vincristine ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830180211
更新日期:1985-02-01 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::The role of peripheral platelet destruction as a reversible etiology of thrombocytopenia in chronic lymphocytic leukemia (CLL) was evaluated in nine patients with CLL and refractory thrombocytopenia who underwent splenectomy. The patients' ages ranged from 54 to 74 years. Progressive thrombocytopenia refractory to ant...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830150306
更新日期:1983-11-01 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::To elucidate the precise mechanisms of molecular and cellular regulation of hemopoiesis, it is necessary to develop a chemically defined culture assay for purified hemopoietic progenitors. To approach this long-term goal, we attempted to develop a serum-free culture system for enriched human progenitors that permits e...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830280404
更新日期:1988-08-01 00:00:00
abstract::A new density system for the separation of human red blood cells by density-gradient centrifugation is described. The gradient medium is made with colloidal silica particles coated with polyvinylpyrrolidone suspended in aqueous solution of meglamine diatrizoate. By this method, more than 10 red-cell fractions can be s...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830080307
更新日期:1980-01-01 00:00:00
abstract::We analyzed the genetic defects of 21 unrelated patients with venous thrombosis in whom hereditary protein C deficiency was diagnosed. Eleven mutations were detected in 18 families, while no mutation was detectable in the other three families. Among these mutations, a common genetic mutation of protein C (PROC) gene r...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20043
更新日期:2004-05-01 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::Intravenous anti-D immune globulin (anti-D IGIV) is indicated for the treatment of immune thrombocytopenic purpura (ITP) in nonsplenectomized patients who are Rh(D)-positive. Recent reports have described episodes of intravascular hemolysis (IVH) and acute renal failure (ARF) after anti-D IGIV. We report the first adu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10432
更新日期:2003-12-01 00:00:00