Abstract:
:The 8p11 myeloproliferative syndrome (EMS) is an aggressive neoplasm associated with chromosomal translocations involving the fibroblast growth factor receptor 1 (FGFR1) tyrosine kinase gene on chromosome 8p11-12. A new case of a 9-year-old boy with leukocytosis, eosinophilia, and general lymphadenopathy is reported in this study. Bone marrow examination showed eosinophilic hyperplasia, with blast cells amounting to 6-7%. Karyotyping revealed cytogenetic abnormalities, including t(8;9)(p11.2;q3?3). Fluorescence in situ hybridization for the FGFR1 gene rearrangement yielded positive results. Lymph node biopsy confirmed the diagnosis of precursor T-lymphoblastic lymphoma. The patient responded to chemotherapy, and unmatched related bone marrow transplantation was performed. A successful outcome was obtained with complete cytogenetic remission maintained for 14 months to date. In the future, FGFR1 inhibitors might be specific and effective therapeutic targets for EMS. Similar cases from the literature are reviewed.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Lv H,Hu S,Lu J,Zhai Q,Zhai Z,Du Z,Sun Y,Li J,He H,Wang Ydoi
10.1159/000481392subject
Has Abstractpub_date
2018-01-01 00:00:00pages
176-182issue
3eissn
0001-5792issn
1421-9662pii
000481392journal_volume
139pub_type
杂志文章,评审abstract::This paper reports the bone marrow histological picture in 7 patients with hairy cell leukemia treated with recombinant interferon. Bone marrow biopsies were performed at diagnosis, after 1, 3 and 7 months from the beginning of treatment and at 12 months at treatment suspension. The main modifications observed were a ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205996
更新日期:1987-01-01 00:00:00
abstract::At our hospital, 47 out of 184 consecutive splenectomies performed over 7 recent years were carried out on patients afflicted with various hematologic diseases. The results of these 47 splenectomies were the subject of a careful retrospective analysis. The majority of the splenectomies (81%) were therapeutic. Cytopeni...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205947
更新日期:1987-01-01 00:00:00
abstract::Treatment options are limited for patients with advanced forms of myeloproliferative neoplasms (MPN) including blast-phase disease (MPN-BP). Decitabine has frequently been deployed but its efficacy and safety profile are not well described in this population. We retrospectively reviewed 42 patients treated with decita...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000506146
更新日期:2021-01-01 00:00:00
abstract::A rare case of Hodgkin's disease which initially presented with a thyroid tumor in an 18-year-old-man is reported. The tumor involved most of the thyroid gland but was well demarcated, and the border between the tumor and the remnants of the thyroid gland was relatively clear, suggesting secondary Hodgkin's disease in...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203627
更新日期:1997-01-01 00:00:00
abstract::Acute myelogenous leukemia (AML) of the inbred Wistar/Furth (W/Fu) rat is pathophysiologically similar to human AML. Subcutaneous transplantation of 1.0 X 10(6) cells of a clonal tissue culture line of W/Fu AML into 6- to 8-week-old rats produced local myeloblastomas in 8--10 days which progressed to infiltration of r...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207886
更新日期:1977-03-01 00:00:00
abstract::Factor X inhibitors are rare. The few cases documented in the literature have occurred after viral prodromes, in association with cancer, or after exposure to antibiotics. Acquired factor X deficiencies are also rare and their etiology is largely unknown. We report a new case of a factor X inhibitor and review prior c...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000342115
更新日期:2013-01-01 00:00:00
abstract::Inferferon alfa-2b (IFN) plays a major role in the current management of previously untreated patients with chronic myelogenous leukemia (CML) as well as patients with CML who have relapsed after bone marrow transplantation. Hydroxyurea (HU) is the best conventional drug for treatment of CML in the chronic phase. Ten ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203859
更新日期:1996-01-01 00:00:00
abstract::Iron overload is a constant and the more important complication in thalassemia. Serum ferritin concentration accurately reflects body iron stores. A total of 245 thalassemic patients aged 12-55 years were examined, 71 having Hb H disease and 174 beta-thalassemia/Hb E disease. The patients received minimal or no blood ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207129
更新日期:1981-01-01 00:00:00
abstract::All-trans-retinoic acid (ATRA) combined with anthracyclines is currently the standard treatment for acute promyelocytic leukemia (APL). In elderly patients the presence of comorbidities, such as cardiomyopathy or different organ failures, often represents an absolute contraindication to standard chemotherapy. In this ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000097880
更新日期:2007-01-01 00:00:00
abstract::A 69-year-old man with a rare case of lymphoma-type alpha-chain disease was admitted to the hospital with marked cervical and inguinal lymph node swelling. Lymph node biopsy showed marked infiltration of plasma cells, plasmacytoid cells and immunoblastoid cells, alone or in combination. Immunoelectrophoresis and immun...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204814
更新日期:1991-01-01 00:00:00
abstract::In the myelodysplastic syndromes (MDS) clonogenic marrow cell culture studies have demonstrated intrinsic hemopoietic stem cell and progenitor cell abnormalities consistent with these disorders representing clonal hemopathies. Abnormal responsiveness of these cells to stimulatory and inhibitory growth factors indicate...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000205911
更新日期:1987-01-01 00:00:00
abstract::Thymoma is an uncommon neoplasia derived from the epithelial cells of the thymus, which leads to immune dysregulation and is associated with a series of autoimmune diseases. However, the concurrence of these disease entities is rare, and the exact mechanisms of these diseases are still unclear. We have admitted severa...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000503376
更新日期:2020-01-01 00:00:00
abstract::We examined the in vivo effect of granulocyte colony-stimulating factor (G-CSF) on the surface expression of putative counterligands for endothelial selectins on neutrophils in healthy volunteers. G-CSF (50 microg/m2/day) was administered subcutaneously to 5 healthy volunteers for 4 days. The expression of surface ant...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040901
更新日期:1998-01-01 00:00:00
abstract::Chromosomal aberrations in a case of atypic myeloproliferative syndrome are reported. The analysis was carried out on short-term cultures of sternal bone marrow with the ASG and fluorescence method. 90% of the metaphases studied showed pseudoliploidy: one of the chromosomes 12 was missing and one submedian marker chro...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208052
更新日期:1975-01-01 00:00:00
abstract::We report on a patient with chronic myeloid leukemia (CML) with a rapidly growing left cervical tumor 5 months after the initial diagnosis of CML. This tumor was diagnosed as a very early manifestation of extramedullary myeloblastoma by a minimally invasive method. A fine needle aspirate (26-gauge needle) was obtained...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040873
更新日期:1998-01-01 00:00:00
abstract::We studied neutrophil chemotaxis and surface membrane glycoproteins in 12 patients suffering from myeloid disorders with abnormal karyotype using in vitro techniques in all 12. Chromosome studies were also carried out virtually simultaneously. We chose to study only patients showing a deficit of chemotaxis (p less tha...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205543
更新日期:1989-01-01 00:00:00
abstract::In a group of 56 patients with acute leukaemia, the relation between leukaemic cell motility and peripheral leukaemic cell count, degree of organomegaly, maturation and FAB classification was investigated. Cell motility was studied by means of directly observed motility and scoring of handmirror cells and anti-HLA-cap...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205595
更新日期:1988-01-01 00:00:00
abstract::Platelet to leukocyte adhesion phenomena (PLAP) and the phagocytosis of platelets by neutrophils from the EDTA anticoagulated blood samples are described. PLAP was transferable to a normal blood specimen by patient's plasma or serum with or without complement. Further studies revealed in vitro evidence of hereditary p...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206966
更新日期:1982-01-01 00:00:00
abstract::96 consecutive acute myelogenous leukemia (AML) patients were analyzed retrospectively with regard to the regimen used for remission induction. 35 patients received daunorubicin for 3 days, cytosine arabinoside and 6-thioguanine for 7 days. 61 were treated with the same regimen but 6-thioguanine was replaced by etopos...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205173
更新日期:1990-01-01 00:00:00
abstract::Premature neonates require blood transfusions, and biological parents may wish to be directed donors. Biological mothers pose a potential danger because their plasma may contain antibodies that will react with blood cell antigens inherited by the infant from the father. We studied 25 healthy, pregnant women at the tim...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204889
更新日期:1991-01-01 00:00:00
abstract::We report a patient with myelodysplastic syndrome (MDS), refractory anaemia with excess blasts in transformation, in whom complete remission (CR) was achieved with the administration of granulocyte colony-stimulating factor (G-CSF). The 76-year-old patient was admitted to our hospital with a fever and a productive cou...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000057637
更新日期:2002-01-01 00:00:00
abstract::Agranulocytosis developed in a 20-year-old Greek patient with beta-thalassaemia major, 11 weeks after commencing chelation with the oral iron chelator 1,2-dimethyl-3-hydroxypyrid-4-one (L1) and 6 weeks after receiving the drug at a total daily dose of 105 mg/kg. The patient presented with generalised weakness, low-gra...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000204494
更新日期:1993-01-01 00:00:00
abstract::To assess the effect of prophylactic treatment with antithymocyte globulin (ATG) on graft-versus-host disease (GvHD) in myeloablative transplant patients, we performed a meta-analysis of randomized and cohort studies. Medline, Embase, the Cochrane Controlled Trial Register and the Science Citation Index were searched ...
journal_title:Acta haematologica
pub_type: 杂志文章,meta分析,评审
doi:10.1159/000343604
更新日期:2013-01-01 00:00:00
abstract::A 16-year-old Japanese woman with acute myelogenous leukemia (AML) and t(6;9) received peripheral blood stem cell transplantation (PBSCT). Although chromosomal studies just prior to and following PBSCT showed a normal karyotype, reverse transcription-polymerase chain reaction (RT-PCR) detected the mRNA derived from th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203996
更新日期:1995-01-01 00:00:00
abstract::12 thalassaemic patients from Northern Sardinia showing the beta + phenotype were examined by isoelectric focusing and high-performance liquid chromatography techniques for the determination of the variant A gamma T globin chain of the foetal haemoglobin. Two patients (16.7%) were homozygotes for the A gamma T gene va...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206057
更新日期:1986-01-01 00:00:00
abstract::Two family members (daughter and mother) with a bleeding disorder showed prolonged bleeding time and activated partial thromboplastin time associated with decreased plasma levels of factor VIII procoagulant activity, factor VIII-related antigen, and factor VIII-ristocetin cofactor activity. The ristocetin-induced plat...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206579
更新日期:1984-01-01 00:00:00
abstract::In pregnant mice. 55Fe-labeled iron-dextran (Imferon) is transferred across the placenta. It was detected in the bone marrow, liver, spleen and peripheral blood of the pregnant animal, as well as in the embryonic liver erythroid precursors and peripheral blood. Uptake by liver and peripheral blood cells of pregnant an...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208195
更新日期:1975-01-01 00:00:00
abstract::The severe endothelial dysfunction in children with acute lymphoblastic leukemia (ALL) can result from the disease itself, from treatment, or from other conditions (e.g. sepsis). The aim of this study was to determine the levels of markers of endothelial activation in children with ALL and to assess their potential pr...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000322120
更新日期:2011-01-01 00:00:00
abstract::A 27-year-old male developed massive generalized lymphadenopathy with chronic myelomonocytic leukemia (CMML) presenting as extramedullary blast crisis mimicking a lymphocytic lymphoma. On presentation, a consistent chromosomal abnormality involving chromosomes 8 and 13, i.e. 46,XY,t(8;13) (q11;p11), was present in lym...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204687
更新日期:1992-01-01 00:00:00
abstract::350 patients with idiopathic thrombocytopenic purpura (ITP) aged 2/12-15 years (mean 6.3 +/- 2.7) were followed up during the period January 1st, 1975 to March 31, 1992. They constituted 40% of cases with hemorrhagic diathesis attending the Hematology/Oncology Clinic, Children's Hospital, Ain Shams University (relativ...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000204392
更新日期:1993-01-01 00:00:00