Assuntos
Neoplasias/tratamento farmacológico , Compostos de Mostarda Nitrogenada/história , História do Século XX , Doença de Hodgkin/tratamento farmacológico , Doença de Hodgkin/história , Humanos , Leucemia/tratamento farmacológico , Leucemia/história , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/história , Mecloretamina/história , Mecloretamina/uso terapêutico , Neoplasias/história , Compostos de Mostarda Nitrogenada/uso terapêutico , Estados UnidosAssuntos
Anemia/diagnóstico , Anemia/classificação , Anemia/patologia , Diagnóstico Diferencial , HumanosRESUMO
The clinical course of 29 patients with idiopathic refractory sideroblastic anemia studied by us was reviewed. Four patients developed acute leukemia. We were able to find 27 out of 268 cases of idiopathic refractory sideroblastic anemia reported in the literature which terminated in acute leukemia. Nine of these were well described. The overall incidence of acute leukemia is 10%. In an attempt to identify the risk factors for the development of acute leukemia, the clinical and laboratory features of this group of thirteen well-described cases which terminated in acute leukemia were compared to the remaining 25 of our cases which did not undergo leukemic transformation. The patients who died of acute leukemia tended to have a more severe anemia, a lower reticulocyte count, and increased transfusion requirement, and thrombocytopenia. Thrombocytosis appears to be a relatively good prognostic sign.
Assuntos
Anemia Sideroblástica/complicações , Leucemia Mieloide Aguda/etiologia , Adulto , Idoso , Fosfatase Alcalina/sangue , Anemia Sideroblástica/sangue , Anemia Sideroblástica/terapia , Transfusão de Sangue , Contagem de Eritrócitos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Reticulócitos , Risco , Fatores de TempoRESUMO
Severe aplastic anemia developed in a young man with an extensive family history of leukemia, pancytopenia, and neutropenia. Megaloblastic changes became evident, and treatment with high doses of folic acid resulted in striking clinical improvement. However, red-cell folate levels remained persistently low despite high serum folate levels. A defect in cellular folate uptake was suspected, and, indeed, uptake of 5-14CH3-H4-folate by stimulated lymphocytes and by bone-marrow cells from the patient was significantly reduced (P less than 0.05 as compared to normal cells. Further characterization of folate metabolism showed that intestinal absorption of the vitamin, membrane transport of 5-14CH3-H4-folate by mature red cells, folate utilization in the conversion of deoxyuridylate to thymidylate and polyglutamate formation were all normal. At least five other family members manifest decreased uptake of 5-14CH3-H4-folate by stimulated lymphocytes. These studies suggest that a genetically induced abnormality of folate uptake contributed to this patient's severe, but reversible, aplasia.
