Adult T-cell leukemia with predominant skin involvement

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In February 1992, a 67-year-old man noticed erythematous tumors on his forehead and left areola mamma. The tumors then spread over his entire body. At Yokohama Sakae Mutual Aid Hospital he was diagnosed as having adult T-cell leukemia (ATL) on the basis of clinical, laboratory, and 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 designer 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 the lymph nodes. Erythematous skin tumors were evident on the forehead, back, chest, lumbar area, buttocks, and thighs (Fig. 1). The laboratory data showed no leukocytosis (5400/mm3), lymphocytosis (35.6%), or high level of serum lactate dehydrogenase (LDH) (361 mU/mL). Furthermore, there was no hypercalcemia at the time of the initial medical examination. 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. 2B). From these clinical, laboratory, and pathologic data, the patient was diagnosed as having smoldering- or lymphoma-type ATL according to the diagnostic criteria of the Lymphoma Study Group.1The patient was treated with multiagent combination chemotherapy, including an LSG4 protocol2 (VEPA-B: vincristine, cyclophosphamide, prednisone, doxorubicin, bleomycin; M-FEPA: methotrexate, vindecine, cyclophosphamide, prednisone, doxorubicin; VEPP-B: vincristine, etoposide, prednisone, procarbazine, bleomycin) and a MACOP-B protocol (methotrexate, doxorubicin, cyclophosphamide, vincristine, bleomycin, prednisone, co-trimoxazole), and electron-beam irradiation (46 Gy) from May 1992 to December 1992. The patient achieved successful remission with this protocol, but was not free of disease for long. He died on January 31, 1993, due to renal failure, with tumor invasion of the liver, lung, pancreas, myocardium, bone, bladder, prostate, and right adrenal gland.The DNA sample from the skin and the peripheral blood mononuclear cells contained HTLV-1-related sequences, but that from the inguinal lymph node yielded no such amplified sequences (Fig. 3A) by polymerase chain reaction (PCR) analysis. DNA samples from the mixture of HUT102 (HTLV-1-positive T-cell line) and Daudi (EB-transformed B-cell line) cells yielded amplified sequences 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 inguinal lymph node from the patient.

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