Abstract:
:Pyruvate kinase (PK) from four patients with moderate to severe congenital non-spherocytic haemolytic anaemia was characterized by methods recommended by the ICSH. The possibility that two of the patients are true homozygotes cannot be ruled out, while the other two apparently represent double heterozygotes. All but one had levels of PK activity between 44 and 65% of normal. The variant enzymes were designed 'PK Pontos', 'PK Macedonia', 'PK Athens' and 'PK Larisa'. Multiple physicochemical as well as kinetic aberrations were detected in the above variants. Their altered kinetic behaviour is discussed in terms of the concerted transition model for allosteric enzymes and their abnormal properties are compared with other known variants, while it is also attempted to correlate them with possible mechanisms resulting in chronic haemolytic anaemia.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Tegos C,Anagnostoulis Gdoi
10.1159/000204184subject
Has Abstractpub_date
1994-01-01 00:00:00pages
91-6issue
2eissn
0001-5792issn
1421-9662journal_volume
92pub_type
杂志文章,评审abstract::Multiple myeloma (MM) is a B cell malignancy characterized by accumulation of plasma cells (PCs) in the bone marrow. Traditional methods for the detection of minimal residual disease (MRD) measure the presence of monoclonal immunoglobulin protein secreted by the malignant PCs. However, changes in the level of MRD in M...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000077565
更新日期:2004-01-01 00:00:00
abstract::Peripheral blood lymphocytes from patients with chronic lymphocytic leukemia (CLL), lymphoplasmacytoid lymphoma, centrocytic lymphoma and hairy cell leukemia were studied by scanning electron microscopy (SEM). In general, SEM revealed rather homogenous cell populations. Most lymphocytes displayed a moderately villous ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207888
更新日期:1977-03-01 00:00:00
abstract::A modified cytochemical method was developed for demonstrating magnesium-activated adenosine triphosphatase in haemic cells. The enzyme was shown in a variety of normal and leukaemic, unfixed cells. The high sensitivity of this enzyme to all fixatives tried in this study hampered further exploration and characterizati...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205983
更新日期:1987-01-01 00:00:00
abstract::Differentiation of rapidly binding coagulation factor inhibitors from antiphospholipid antibodies is a challenge for the hemostaseologic laboratory, especially with respect to the different therapeutic consequences. Several immunological and functional assays for the diagnosis of these disorders have been proposed. He...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040821
更新日期:1998-01-01 00:00:00
abstract::The detection and enumeration of sideroblasts depend critically on the method used for iron staining of bone marrow smears. Several methods proposed for semiquantitative evaluation of bone marrow hemosiderin (iron stores) were compared with respect to their suitability for detection of normal and abnormal sideroblasts...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207856
更新日期:1977-01-01 00:00:00
abstract::The unstable haemoglobin, Tb Torino (alpha43(CD1)Phe replaced by Val), has been found for the second time in a family from the Treviso region of Italy. The haemoglobin has a slightly lower oxygen affinity than normal. In both cases, the abnormal haemoglobin is associated with inclusion body anaemia but the course of t...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207942
更新日期:1976-01-01 00:00:00
abstract::Using enzyme-linked immunosorbent assay (ELISA) and confirmatory immunoprecipitation tests, sera from 640 Nigerians from Lagos and Cross River States were examined for antibodies against HIV. These comprised 570 blood donors and their family members, 56 patients with various haematological conditions and 14 patients w...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205728
更新日期:1988-01-01 00:00:00
abstract::An increased bone marrow (BM) apoptosis is one of the mechanisms responsible for the ineffective hematopoiesis of myelodysplastic syndromes (MDS). It is controversial whether the excessive apoptosis in myelodysplasia predominantly involves the subset of progenitor cells or of maturing cells. We investigated the degree...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000067275
更新日期:2003-01-01 00:00:00
abstract::Sera from 154 hemophiliacs, including 132 with hemophilia A and 22 with hemophilia B, were examined for antibodies against human T cell lymphotropic virus type III (HTLV-III) and type I by strip radioimmunoassay based on the Western blotting technique. Sixty-two patients lived in Kyushu, a known endemic area of HTLV-I...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205944
更新日期:1987-01-01 00:00:00
abstract::We have demonstrated that hemopoietic organ fibroblastoid cells can produce colony-stimulating activity (CSA) in vitro. Skin fibroblasts also produced CSA. The colonies which formed were restricted to the granulocyte/macrophage differentiation pathways. A linear relationship was obtained for granulocyte/macrophage col...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206924
更新日期:1983-01-01 00:00:00
abstract::Primary plasma cell leukemia (PCL) is a rare form of plasma cell neoplasm with a poor prognosis. Conventional melphalan-based treatments have been most disappointing. We report the case of a 62-year-old man with a primary form of PCL treated with VAD combination achieving an objective response, and who received high-d...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040952
更新日期:1999-01-01 00:00:00
abstract:BACKGROUND:The treatment of relapsed/refractory (R/R) peripheral T cell lymphoma (PTCL) is limited to a few agents. Romidepsin, a histone deacetylase inhibitor, was approved for PTCL treatment as a single agent in the R/R setting, yet with partial efficacy. Several attempts to combine romidepsin with other chemotherapy...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000498905
更新日期:2019-01-01 00:00:00
abstract::6 patients with acute leukemia are described. All of them suffered from high temperature and malaise, and showed negative urine and blood cultures. High doses of gentamicin, cephalothin and cerebenicillin failed to lower the fever. The temperature became normal after administration of chloramphenicol (CAP) 2.0 g/day. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207275
更新日期:1980-01-01 00:00:00
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 i...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000481392
更新日期:2018-01-01 00:00:00
abstract::The maturation process of leukemic myelomonocytoid cells derived from peripheral blood of two typical cases of chronic myelomonocytic leukemia was investigated in vitro. In both cases, liquid culture of the leukemic cells in the presence or absence of 12-o-tetradecanoyl phorbol-13-acetate yielded either monocyte-macro...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206382
更新日期:1984-01-01 00:00:00
abstract::To test whether anti-idiotypic immunoregulation of factor VIII(FVIII)-inhibiting antibodies could be feasible in hemophiliacs, we assayed the minimal number and range of immunogenic, functional FVIII epitopes to which a series of murine anti-FVIII monoclonal antibodies (MAb) were directed. Rabbit anti-idiotypic sera t...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205171
更新日期:1990-01-01 00:00:00
abstract::Residues 124 and 125 of the beta-chain of human haemoglobin are prolyl-prolyl. In the delta-chain, one of these positions is occupied by a glutaminyl, and there has been uncertainty as to which of the two residues is the prolyl and which the glutaminyl. The sequence has now been established to be delta124 Pro - 125 Gl...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207923
更新日期:1976-01-01 00:00:00
abstract::We describe two kindreds of Arab ancestry characterized by multiple cases of acute lymphoblastic leukemia. Consanguinity and intermarriages were prevalent in the two families. Age, mode of presentation, characteristics of the leukemic cells, response to treatment and prognosis were remarkably similar among the patient...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204222
更新日期:1994-01-01 00:00:00
abstract::Aspergillus vegetative endocarditis developing in a patient with acute lymphocytic leukemia during the phase of hematological remission has led to a fatal outcome, complicated with severe hemolytic anemia with red cell fragmentation. Systemic aspergillosis may involve heart valves with underlying disorders, but seldom...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206021
更新日期:1986-01-01 00:00:00
abstract::Chronic graft-versus-host disease (GVHD) is a severe complication of allogeneic stem cell transplantation, with a substantial impact on the quality of life and survival, still lacking with regard to an optimal therapeutic strategy. Corticosteroids are considered the standard of care for first-line treatment of chronic...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000345836
更新日期:2013-01-01 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::In a previous study, we evaluated efficacy of repeated antilymphocyte globulin (ALG) treatment for patients with severe aplastic anemia not responding to an initial ALG treatment or relapsing after initial response to ALG. We now searched in the same cohort of patients for differences between patients who responded to...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000041005
更新日期:2000-01-01 00:00:00
abstract::We describe a patient with recurrence of Hodgkin's disease and severe liver disease of unknown origin in whom autoimmune neutropenia developed. Because of possible seronegative viral hepatitis he was treated with high-dose intravenous immunoglobulin instead of steroids. He responded with a prompt but transitory increa...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203968
更新日期:1995-01-01 00:00:00
abstract:BACKGROUND/AIMS:The aim of this work was to investigate the efficacy and predictive factors of CLAG treatment in refractory or relapsed (R/R) acute myeloid leukemia (AML) patients. METHODS:Sixty-seven R/R AML patients were enrolled in this prospective cohort study and treated by a CLAG regimen: 5 mg/m2/day cladribine ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000493250
更新日期:2019-01-01 00:00:00
abstract::The most common single genetic disorder and a major public health issue in Greece and other Mediterranean countries is beta-thalassemia. Current therapeutic approaches for homozygous beta-thalassemia entail blood transfusions and iron chelation therapy with deferoxamine or deferiprone for preventing tissue hemosideros...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000077551
更新日期:2004-01-01 00:00:00
abstract::The frequency of chronic lymphocytic leukemia (CLL) varies a great deal from one population to another. We have undertaken to compare the aspects of CLL in Maghreb and Europe through two series of comparable importance, studied during the same period and under similar conditions in Algiers and Amiens. This comparison ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206896
更新日期:1983-01-01 00:00:00
abstract::Natural killer (NK) cell activity against K562 cell targets and the distribution of T cell subpopulations were investigated in the peripheral blood of 25 patients affected by beta thalassemia major, 18 clinically healthy heterozygotes, and 25 age-matched normal subjects. It was found that thalassemia major patients di...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206598
更新日期:1984-01-01 00:00:00
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 ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000204260
更新日期:1994-01-01 00:00:00
abstract::Treatment of acute lymphoblastic leukemia is unsatisfactory in adults due to disease and patient-related factors and probably because adult chemotherapy regimens are weaker than pediatric protocols. Worries about inadequacy of adult regimens urged many hematologists, including us, to reconsider their routine treatment...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1159/000351172
更新日期:2013-01-01 00:00:00
abstract::During a screening for hemoglobinopathies, we found a carrier of the Sardinian δβ-thalassemia condition. The proband's hematology and hemoglobin (Hb) profile agreed with those of the other carriers previously identified during our diagnostic program except for the fetal Hb (HbF) composition, which consisted of both α2...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000447942
更新日期:2016-01-01 00:00:00