Abstract:
:Essential thrombocythemia (ET), one of the chronic myeloproliferative disorders, is a clonal disorder of multipotent stem cells. Although most patients with ET have a prolonged benign course, a minority of patients may develop a blastic crisis similar to chronic myelogenous leukemia (CML). A case of ET terminating in blastic crisis 8 years after the initial diagnosis is presented. The blast cells were cytochemically and immunophenotypically consistent with the acute myelogenous leukemia with minimal myeloid differentiation subtype of the FAB classification. From the review of the literature on blastic transformation of ET, acute leukemia with an M4 or M7 phenotype occurred more frequently. In addition, three valuable factors to predict the leukemic transformation of ET appear to be karyotypic abnormalities, such as involvement of chromosome 21, previous therapies with a mutagenic potential, and the capability of bone marrow cells to form in vitro spontaneous colonies as in CML.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Shibata K,Shimamoto Y,Suga K,Sano M,Matsuzaki M,Yamaguchi Mdoi
10.1159/000204260subject
Has Abstractpub_date
1994-01-01 00:00:00pages
84-8issue
2eissn
0001-5792issn
1421-9662journal_volume
91pub_type
杂志文章,评审abstract::Thalidomide acts on the microenvironment of myelodysplastic syndromes (MDS) by influencing cytokine networks, and growing evidence supports thalidomide's usefulness in the management of haematological malignancies, such as MDS. The European Collaboration Group on Myelofibrosis with Myeloid Metaplasia reviewed patients...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000087042
更新日期:2005-01-01 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::Hypersensitivity vasculitis occurs in response to various exogenous agents. A case of a serum sickness syndrome with cutaneous vasculitis is described in a patient given an intravenous iron-dextran infusion. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205198
更新日期:1990-01-01 00:00:00
abstract::Using labelled erythrocytes and human serum albumin, the volume of circulating erythrocytes and plasma was determined in the spleen and individual parts of the skeleton of mice under physiological conditions and 48 h after X-irradiation with a dose of 2,87 Gy. The results are significant for the interpretation of the ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207231
更新日期:1980-01-01 00:00:00
abstract::Hemochromatosis has generally been considered to be a genetic disease in which progressive iron accumulation over many years can lead to cirrhosis of the liver, hepatocellular carcinoma, diabetes, cardiomyopathy, and arthropathy. Iron depletion by phlebotomy has been the recommended therapy although a randomized trial...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000243797
更新日期:2009-01-01 00:00:00
abstract::With the widespread use of genetic diagnostic technologies, many novel mutations have been identified in hereditary spherocytosis (HS)-related genes, including SPTA1, SPTB, ANK1, SLC4A1, and EPB42. However, mutations in HS-related genes are dispersed and nonspecific in the diagnosis of some HS patients, indicating sig...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000486229
更新日期:2018-01-01 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::Pleural effusion in chronic lymphocytic leukemia (CLL) is a relatively rare phenomenon. We report a case of a pleural effusion associated with B-cell CLL but with predominantly reactive T lymphocytes in the effusion. A cell surface phenotype study showed that T lymphocytes predominated in the pleural effusion, althoug...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000203713
更新日期:1996-01-01 00:00:00
abstract::The Philadelphia chromosome-negative myeloproliferative disorders (MPDs) polycythemia vera (PV), essential thrombocytosis (ET) and primary myelofibrosis (PMF) are characterized by increased proliferation of terminally differentiated myeloid cells. Although these disorders were recognized as clonal hematopoietic stem c...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000140634
更新日期:2008-01-01 00:00:00
abstract::Recent molecular biological investigations have shown that genetic information on the short arm of chromosome 17 is frequently deleted or inactivated in acute myeloid leukaemias and a number of solid tumours. In earlier studies we have found clonal occurrence of a 17p deletion (17p-) in patients with refractory anaemi...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205033
更新日期:1990-01-01 00:00:00
abstract::Chronic pain affects 50% of adults with sickle cell disease (SCD). Although inflammation is thought to contribute to the pathogenesis of chronic pain, no studies have examined the differences in circulating cytokines between patients with SCD with and without chronic pain. We performed an observational cohort study us...
journal_title:Acta haematologica
pub_type: 新闻
doi:10.1159/000507563
更新日期:2021-01-01 00:00:00
abstract:BACKGROUND:Splanchnic vein thrombosis (SVT) is a severe complication of essential thrombocythemia (ET). No clear explanation has been given for the occurrence of thrombosis in this unusual site in patients with ET, but the existence of a specific association between unexplained SVT and the JAK2 mutation has been report...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000221962
更新日期:2009-01-01 00:00:00
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::Fas/APO-1 (CD95) is a cell surface molecule that can transduce apoptotic signals into cells. We examined the expression of Fas antigen on CD4+ and CD8+ T cells of patients who received total body irradiation (TBI) as a preparative regimen for allogeneic bone marrow transplantation. Numbers of peripheral blood lymphocy...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000046514
更新日期:2000-01-01 00:00:00
abstract::It was found that neutrophils in untreated uraemic patients as well as in subjects on regular dialysis treatment displayed higher activity of acid phosphatase, alkaline phosphatase and peroxidase. Spontaneous reduction of nitro blue tetrazolium (NBT) by granulocytes was also higher in both groups in comparison to cont...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207647
更新日期:1979-01-01 00:00:00
abstract::A 12-year-old male of Chinese ancestry had life-long hemolytic anemia attributed to alpha-thalassemia. Restriction endonuclease mapping of his DNA revealed that in reality, he had three alpha-globin loci, but he was homozygous for pyruvate kinase deficiency. The new pyruvate kinase variant carried by this patient was ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206830
更新日期:1983-01-01 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::Peripheral blood stem cells (PBSC) are widely used in the setting of dose-intensive chemotherapies in patients with multiple myeloma (MM). Although the granulocyte colony-stimulating factor (G-CSF), following chemotherapy or not, is considered the standard growth factor for mobilizing PBSC, the optimal chemotherapeuti...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000070967
更新日期:2003-01-01 00:00:00
abstract::Micromegakaryocytes (MMK) were defined morphologically by the cell area, nucleus form and cytoplasmic structure. Bone marrow smears of 7,156 patients were retrospectively analyzed. MMK were found most frequently and abundantly in acute non-lymphatic leukaemia, chronic myeloid leukaemia and pre-leukaemia. The presence ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207213
更新日期:1980-01-01 00:00:00
abstract::Neurologic complications of allogeneic hematopoietic cell transplantation (allo-HCT) include infections, cerebrovascular events, therapy-induced neurotoxicity, recurrent malignancies, and neurologic manifestations of graft-versus-host disease (GVHD). Anti-glutamic acid decarboxylase (GAD) antibody-associated cerebella...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000494423
更新日期:2019-01-01 00:00:00
abstract::Fetal hemoglobin (HbF) concentrations were measured by a radial immunodiffusion assay in 233 patients with various malignancies. In 96 of these, alpha-fetoprotein (AFP) was also measured by radioimmunoassay. The concentration of HbF exceeded 2 SDs above the normal mean in 39 of 233 patients, most notably in patients w...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207839
更新日期:1977-01-01 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::The dilution in the circulating blood of lymphocytes lablled in vitro with 3H-cytidine was examined after autotransfusion in 9 patients with Hodgkin's disease (HD) stage II A-IV B, 5 of whom were untreated; in 2 untreated patients with carcinoma, and in 1 treated patient with scleroderma. The blood transit time of exc...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208185
更新日期:1975-01-01 00:00:00
abstract::Acute megakaryoblastic leukemia is uncommon and comprises about 5% of acute nonlymphoid leukemias in the French-American-British classification. Cell lines from such leukemias are relatively rare with only about 8 reported in the literature. We established a cell line from a case of acute megakaryoblastic leukemia ari...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203557
更新日期:1997-01-01 00:00:00
abstract::An ultrastructural study of the aspirated bone marrow of a patient with congenital dyserythropoietic anemia type I is presented. Both in transmission electron microscopy (TEM) and in scanning electron microscopy (SEM) ultrastructural abnormalities of the bone marrow erythroid precursors were seen. These abnormalities ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206735
更新日期:1983-01-01 00:00:00
abstract:BACKGROUND:Despite encouraging reports concerning the declining prevalence of iron deficiency, this easily preventable disorder is still an existing problem in presumably developed regions. OBJECTIVE:To evaluate the prevalence of iron deficiency and relevant anemia in children residing in Northern Greece and to study ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000098273
更新日期:2007-01-01 00:00:00
abstract::Factor VIII (FVIII) inhibitor antibodies are produced against functional epitopes of FVIII in about 30% of severe hemophilia A patients leading to inhibition of its procoagulant activity. The Bethesda assay, the most commonly used method to measure FVIII inhibitors, based on inhibition of coagulant activity of FVIII, ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000086580
更新日期:2005-01-01 00:00:00
abstract::A rare case of acute promyelcytic leukemia (APL) is reported in a 7-year-old boy. The patient displayed the typical features of APL including impaction of the marrow with promyelocytes, marked elevation of the serum vitamin B12 and transcobalamin I levels and a hemorrhagic diathesis. The bleeding diathesis in the case...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207589
更新日期:1979-01-01 00:00:00
abstract:OBJECTIVE:The aim of this investigation was to study the effect of vitamin E treatment in oxidative stress of red and white cells of beta-thalassaemia intermedia patients. METHODS:Nine patients undergoing occasional transfusions (5 females/4 males), median age 39 years (range 15-74), were recruited for oral daily admi...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000201988
更新日期:2008-01-01 00:00:00
abstract::Contamination of autologous graft by tumor, in addition to incomplete tumor eradication, can partly explain why relapse remains the commonest cause of treatment failure after autologous stem cell transplantation (ASCT) in patients with malignant hematologic disorders. Monitoring of minimal residual disease (MRD) is no...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000088411
更新日期:2005-01-01 00:00:00