1986 Volume 27 Issue 1 Pages 68-74
A 39-year-old female was admitted because of exertional dyspnea. Hepatosplenomegaly was not observed. Laboratory investigation disclosed marked pancytopenia with a few blast cells (29%) in the peripheral blood smear. Peroxidase activity was negative in the blast cells, however, some of them contained small number of basophilic granules. There were no tear drop cells or nucleated erythrocytes. Repeated bone marrow aspirations yielded dry taps. A bone marrow biopsy showed marked myelofibrosis and an increased number of atypical megakaryocytes. Cytogenetic examination of circulating mononuclear cells revealed a 46, XX, t (11q+; 17q-) karyotype. No evidence of extramedullary hemopoiesis was obtained by a ferrokinetic examination. From these findings, a diagnosis of acute myelofibrosis was made.
After 3 months from admission, white blood cells gradually increased in number exceeding 20,000/mm3 with 80% blast cells. Antileukemic agents including small dose Ara-C were inefective, however, it was noticeable that a lot of basophils began to appear in the circulation after the chemotherapy. On 8th month of admission, the white blood cell count was 60,000/mm3 with 70% basophils and 15% blast cells. These cells were compatible with basophils on several cytochemical stainings including metachromasia with toluidine blue. There was no hiatus in the maturation of basophils. The patient died of pneumonia and hemothorax. An autopsy showed widespread leukemic infiltration of bone marrow, liver, spleen and other organs.
The patient was the first reported case of acute myelofibrosis terminated in basophilic leukemia. The nature of acute myelofibrosis was discussed in relation to megakaryoblastic leukemia and myelodysplastic syndrome.
