ADULT T-CELL LEUKEMIA WITH PREDOMINANT SKIN INVOLVEMENT

In February 1992, a 67-year-old man noticed erythematous tumors on his forehead and left areola mamma. The tumors then spread over his entir e body. At Yokohama Sakae Mutual Aid Hospital he was diagnosed as havi ng adult T-cell leukemia (ATL) on the basis of clinical, laboratory, a nd pathologic findings.(1) He was referred to our department in April 1992. He was born in southwest Japan and had worked in Yokohama as a d esigner of electric goods. There was no family history of hematologic or other malignancies. He had suffered from tuberculosis and psychosis , but was in good general health. There was no palpable swelling of th e lymph nodes. Erythematous skin tumors were evident on the forehead, back, chest, lumbar area, buttocks, and thighs (Fig. 1). The laborator y data showed no leukocytosis (5400/mm(3)), lymphocytosis (35.6%), or high level of serum lactate dehydrogenase (LDH) (361 mU/mL). Furthermo re, there was no hypercalcemia at the time of the initial medical exam ination. Antibody against human T-cell lymphotropic virus type 1 (HTLV -1) was detected in the serum.(2) Histologic examination revealed skin involvement (Fig. 2A), but no lymph node involvement (Fig. 28). From these clinical, laboratory, and pathologic data, the patient was diagn osed as having smoldering-or lymphoma-type ATL according to the diagno stic criteria of the Lymphoma Study Group.(1) The patient was treated with multiagent combination chemotherapy, including an LSG4 protocol(2 ) (VEPA-B: vincristine, cyclophosphamide, prednisone, doxorubicin, ble omycin; M-FEPA: methotrexate, vindecine, cyclophosphamide, prednisone, doxorubicin; VEPP-B: vincristine, etoposide, prednisone, procarbazine , bleomycin) and a MACOP-B protocol (methotrexate, doxorubicin, cyclop hosphamide, vincristine, bleomycin, prednisone, co-trimoxazole), and e lectron-beam irradiation (46 Gy) from May 1992 to December 1992. The p atient achieved successful remission with this protocol, but was not f ree of disease for long. He died on January 31, 1993, due to renal fai lure, with tumor invasion of the liver, lung, pancreas, myocardium, bo ne, bladder, prostate, and right adrenal gland. The DNA sample from th e skin and the peripheral blood mononuclear cells contained HTLV-1-rel ated sequences, but that from the inguinal lymph node yielded no such amplified sequences (Fig. 3A) by polymerase chain reaction (PCR) analy sis. DNA samples from the mixture of HUT102 (HTLV-1-positive T-cell li ne) and Daudi (EB-transformed B-cell line) cells yielded amplified seq uences when the mixture contained more than 0.1% HUT102 cells (Fig. 3B ), i.e, there was less than 0.1% of HTLV-1-positive cells in the ingui nal lymph node from the patient.