A case of chronic myelomonocytic leukemia complicated by myelofibrosis and polycythemia is presented.
A 73-year-old woman was hospitalized in November 1979 for the examination of an abdominal tumor which she noticed 4 years previously. On admission her spleen and liver were palpable 12 cm and 3 cm below the costal margins, respectively. The peripheral blood showed Hb 11.7 g/d
l, RBC 402×10
4/mm
3, WBC 27,900/mm
3, and platelets 20×10
4/mm
3. WBC differential count demonstrated a marked leucoerytroblastic picture with blasts 2%, promyelocytes 4%, myelocytes 7%, metamyelocytes 8%, neutrophils 47%, eosinophils 2%, basophils 2%, monocyotoid cells 18%, lymphocytes 10% and 5 erythroblasts per 100 WBC. A bone marrow aspirate, obtained with some difficulty, revealed marked granulocytic hyperplasia with an increase in both all stages of granulocytic cells and monocytoid cells. Trephine biopsy of bone marrow showed typical change of myelofibrosis with an increase in collagen and reticulin. NAP score was high and the serum muramidase level was elevated. Cytogenetic study of bone marrow showed normal chromosome karyotype of 46 XX without Ph
1 chromosome. The circulating red cell mass was 1887 m
l by
51Cr method, giving a volume of 41.9 m
l/kg. The in vitro colony counts of CFU-C and BFU-E from both of bone marrow aspirate and circulating blood were greatly increased, while the bone marrow CFU-E colony count was markedly reduced. Because of progressive enlargement of the spleen, a short course of dibromomannitol (100 mg daily) was given, with a fall in the WBC count and a regression of the spleen size.
Thus our case was characterized by an association of myelomonocytic malignant proliforation with erythrocytosis and myelofibrosis. It is known that clinically and hematologically transitional or hybrid cases are not infrequent among chronic myeloproliferative disorders. Our case, who had proliferative changes in more than two series of blood cells, will support the concept that chronic myelomonocytic leukemia is a proliferative disorder involving all marrow cells derived from malignant proliferation of a multipotent stem cell, representing a distinct clinical entity which belongs to the so-called chronic myeloproliferative disorders.
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