1981 Volume 22 Issue 10 Pages 1572-1580
A 52-year-old man who had marked pallor showed severe anemia with nearly absent reticulocyte and distinctive bone marrow erythroid hypoplasia in association with normal WBC and platelet counts. No thymoma was found on chest X-ray. A clinical diagnosis of pure red cell aplasia (PRCA) was made, although a possibility of preleukemic state was suspected hematologically, based on such abnormal findings as presence of cytogenetic abnormal clone, 46, XY, t (8q-; 20q+) and ringed sideroblast in the bone marrow and appearance of rare blastoid cell and megakaryocyte in the circulating blood. Administration of piridoxin, prednisolone, cyclophosphamide and methenolone showed no effective response on his erythropoiesis except for a return of bone marrow M/E ratio toward the normal range.
After 15 months from admission, following administration of folic acid, myeloblast and erythroblast began to appear in the circulation and gradually increased along with bone marrow erythroid hyperplasia with megaloblastoid change and excess myeloblast (8.6%), indicating erythroleukemic conversion. Five months later, hematological examination revealed blastic marrow, leukocytosis over 3×104 cells with 53% myeloblast and persisting same abnormal karyotype. He died of adrenocortico-insufficiency. An autopsy revealed erythroleukemic infiltration in the bone marrow, spleen, liver and kidney. The relation between PRCA and preleukemic state was discussed.
