Refractory Anemias in Japan

Abstract

The hematological features and prognosis of 264 patients with refractory myelodysplastic anemias were reported. They were classified according to a uniform diagnostic criteria into 3 types; primary acquired refractory anemia (PARA, 111 cases), primary acquired sideroblastic anemia (PASA, 49 cases) and refractory anemia with excess of blasts (RAEB, 104 cases). They were ranged between 15 and 85 years of age with a median of 59 years. Male to female ratio was 1.9: 1. Peripheral cytopenias, mostly in the form of pancytopenia and bicytopenia, cellular marrow and a variety of dysplastic morphology represented major hematological findings. In addition, ineffective erythropoiesis was most prominently seen in patients with PASA. Leukemic transformation was most frequently seen in RAEB, least common in PASA. Thus, leukemia-free rate was estimated to be 60% in the whole patients; 28.9% in RAEB, 74.5% in PARA and 89.6% in PASA. The median survival from presentation was 15.8 months in RAEB, 42.6 months in PARA and 40.4 months in PASA. The blast cell count in the marrow was the most significantly variable high leukemic risk and short survival. Among the dyshematopoietic morphology, dysgranulopoietic changes, especially abnormal granules, and dysmegakaryopoietic changes, notably giant platelets and mononuclear megakaryocytes, were also related to poor prognosis. Even in the absence of leukemic transformation, certain complications such as infections and/or bleeding, resulting from persistent cytopenias were frequent causes of death. The results point to a need for a more careful management of patients in the chronic phase.