抄録
A 47-year-old Japanese woman was admitted to the hospital. On admission, splenomegaly and leukoerythroblastic anemia with poikilocysois including tear drop cells were noticed. Ph1-chromosome was negative. Bone marrow biopsy revealed hyperplasia of three blood cell lines, bizarre megakaryocytes, osteosclerosis, and slight fibrosis. A diagnosis of primary myelofibrosis was made. When she was 51 years old, splenic infarction occurred and splenectomy was performed. Marked extramedullary hematopoiesis was found in the spleen, biopsied liver and lymph nodes. Following a transient improvement of her blood picture, leukocytes with immature forms and erythroblasts increased in her blood. Many megaloblastoid cells and atypical erythroblasts with intense PAS reaction appeared. She died of disseminated fungal infection and miliary tuberculosis 1 year after the splenctomy. On autopsy, bone marrow showed leukemic hyperplasia and leukemic cells infiltrated into the liver, lymph nodes, uterus, ovaries, etc. Among leukemic cells, lysozyme- or hemoglobin-positive cells were demonstrated by immuno-histochemical staining. The terminal phase of her illness was diagnosed as erythro-leukemia. Concerning the diagnosis of leukemic transformation, the significance of erythroblastic abnormalities in course of primary myelofibrosis was discussed.
