Pyruvate kinase deficiency and delayed clinical response to recombinant human erythropoietin treatment.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to R De Bock.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
With the use of a monoclonal anti-glycophorin A antibody and flow cytometric cell sorting, an erythroleukemic bone marrow sample was separated in highly purified erythroblast and myeloblast fractions. Similar karyotypic anomalies were found in both cell populations as in the unseparated bone marrow. This study confirms that acute nonlymphocytic leukemia can originate at the level of a multipotential hemopoietic stem cell.
Explore the source record for details and available documents.
With Percoll density gradients, blasts from peripheral blood and bone marrow could be separated with a significant enrichment, and very often with a high degree of purity. This allowed a study of selected cases, where the separated sample exhibited chromosome abnormalities and/or an abnormal DNA content distribution (as measured by DNA-flow cytometry). The anomalies were shown to be associated with the separated blast fraction.
The bone marrow of a patient with pancytopenia showed dyserythropoiesis, dysmegakaryocytopoiesis and 13% blasts. The patient was hypertransfused and the pancytopenia resolved completely for 1 month, while the blastic infiltration in the bone marrow remained. Three months later a frank acute lymphoblastic leukaemia developed.
In a multicenter analysis, the effect of low-dose cytosine arabinoside (Ara-C)(10 mg/ m2q 12 h subcutaneously for a minimum of 15 days) has been assessed in 13 patients with acute leukemia (10 myeloid-AML-, 3 lymphocytic-ALL-) and 7 patients with dysmyelopoietic syndromes (DMPS), conditions classified as refractory anemia with an excess of blasts ( RAEB ). Seven patients suffering from acute leukemia and 1 with DMPS in blastic transformation displayed a leukocytosis of more than 10 X 10(9)/1. Three out of 7 DMPS, 1 out of 10 AML achieved a complete remission, 1 out of 3 ALL-patients reached a partial remission twice. Seven patients showed a blast clearing in the bone marrow and peripheral blood, in another 7 instances examination of the bone marrow was not performed after therapy because of early death. The majority of patients were in their late phase of disease and refractory to conventional chemotherapy. Only 5 patients had no pretreatment at first presentation before low-dose Ara-C was initiated. At least for the DMPS-group, this therapeutic approach seems to be of some benefit.
Myelodysplastic syndromes (MDS) are acquired bone-marrow disorders, characterized by a decreased ability of the haemopoietic cell to differentiate, resulting in peripheral cytopenias. The majority of patients will die either from acute myeloid leukaemia or from infection and/or haemorrhage. Thirty-eight courses of low dose Ara C were administered in 26 MDS patients. Nineteen courses (50%) were associated with good (12) or partial (7) response. Three complete remissions were observed. The median duration of response overall was 19.5 weeks, 26 weeks for the good and 10 weeks for the partial responders. A high incidence of treatment failure was seen in patients treated after transition to AML. Major complications were observed during 14 courses and mortality was directly related to therapy in five patients. Platelet transfusions were required during 26 courses. Aggravation of peripheral-blood cytopenia during the first weeks and hypocellularity of bone-marrow aspirates at the end of therapy suggest that low-dose Ara C exerts its main activity by suppression of leukaemic growth, rather than by induction of differentiation in malignant cells. Our results in MDS patients demonstrate that low-dose Ara C can be of value in severe cytopenia and can decrease the proportion of leukaemic cells in the bone marrow, but the danger of treatment should not be underestimated.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.