An Autopsy Case of Smoldeing Adult Tcell Leukemia/Lymphoma with Intestinal Perforation and Hemophagocytic Histiocytosis

An autopsy case with atypical adult T cell leukemia lymphoma (ATL) was reported. A 24-year-old male who had skin lesions and respiratory distress as premonitory symptoms from his childhood. Skin lesions appeared in the form of vasculitis. Infiltration of ATL cells were not recognizable in the skin and peripheral blood. On his admission, before four months of his death, ATLA (antibody to ATL virus associated antigen) was detected to be positive in his serum and simultenously also in the serum of his mother whose native place south-west region of Japan (Kagawa prefecture). After admission, acute abdominal symptoms occurred and roentgenography revealed multiple intestinal ulcers. He was treated with surgical operations three times, however, massive alimentary bleeding has continued and died on July, 1, 1987.The infiltration of atypical lymphoid cell of T cell character (Leu 1 positive) was observed in the ulcerated walls of intestine surgically resected. Autopsy revealed systemic swelling of lymph nodes of their cell components being in agreement with picture of pleomorphic type of ATL Invasion of ATL cells were found in various organs, i.e. liver, kidneys, spleen, gastrointestinal tract, adrenals and bone marrow. Moreover, remarkable haemophagocytic cells appeared in the lymph nodes, spleen and liver. Lymphoid cells had pleomorphic contour and large, irregular shaped, convoluted nuclei. Immunocytochemically, these cells were positive for Leu 1 and faintly positive for OKIa. On the other hand, reaction for MT-1, MB-1, immunoglobulins and histiocytic markers were negative. Phagocytic histiocytes positively reacted for lysozyme, α1-antitrypsin, and CEA, but negatively for S100 protein. No pulmonary infection was recognized. From clinical and anatomical evidences the patient developed typical ATL having duration of more than ten years with insidious course but without leukemic manifestation. This picture corresponded to smoldering ATL proposed by Takatsuki and Yamaguchi.