1988 Volume 29 Issue 11 Pages 2158-2162
A 39-year-old man was admitted to Tenri Hospital, because of multiple cutaneous nodules, which were located in his face, trunk and extremities. His Hb was 16.0g/dl; Plt, 289×103/μl; WBC, 8,200/μl with normal differential counts. Blood chemistry was normal except for high serum lysozyme. There was slight increase of immature monocytes (22%) in the sternal marrow, but not in the iliac marrow. The cutaneous nodules were within 3 cm in diameter, and were composed of diffuse atypical cells in the dermis and subcutaneous tissues. These cells were diffusely stained with NaF sensitive α-naphthyl butyrate esterase and acid phosphatase, were positive for My4 and My7 antigens, but were negative for T cell-, B cell-, and interdigitating reticulum cell markers. Electron microscopy disclosed nucleus with prominent nucleoli, myeloperoxidase positive granules (<0.2μm) and well developed smooth-ER. Therefore, atypical cells of the skin were of immature ones of monocyte origin, indicating that the patient should be diagnosed as monocytic aleukemic leukemia cutis (monocytic sarcomaof the skin). Effect of anti-leukemic chemotherapy was poor, but total skin electron beam irradiation showed dramatic effect on skin tumors. However, the patient proceeded to overt acute monocytic leukemia 12 months after admission and skin tumors relapsed. He died of sepsis 5 months thereafter.