Abstract
A 53 year-old female was admitted because of fever and lumbago. The blood smear showed leucoerythroblastosis and thrombocytosis with giant platelets. Bone marrow biopsy revealed hyperplastic marrow with reticulin fibrosis. Splenectomy was performed with suspicion of splenic abscess, but removed spleen showed marked extramedullary hematopoiesis with several foci of infarction.
She suffered from high fever and painful erythema on bilateral soles which was diagnosed as erythromelalgia. She was treated with anti-platelet drugs and busulfan, but clinical and hematological effect was not ovbious. Chromosomal analysis of marrow cells revealed abnormal karyotypes mixed with normal one (46, XX/46, XX, -7, +22q-/47, XX, -7, +22q-, +mar). Although she was treated with prednisolone, melphalan, α-interferon and hydroxyurea, blastic cells increased in peripheral blood and bone marrow two years later. Chromosomal analysis revealed abnormal karyotype (46, XX, -C, +mar) in 100% of metaphases of marrow cells. She became resistant to small dose of Ara-C and died with meningeal infiltration one year later.
At autopsy, bone marrow was heavily infiltrated by leukemic cells but obvious fibrosis was not seen.
She was seemed to be a case of atypical myeloproliferative disorder which had high potentiality to develop AML.
