Central nervous system involvement of adult T-cell leukemia–lymphoma with multinucleated giant cells in the brain, skin, and kidney

Abstract

A case of central nervous system (CNS) involvement in a patient with adult T-cell leukemia-lymphoma (ATLL) with multinucleated giant cells (MNGC) is presented. A 48-year-old woman with human T-lymphotropic virus type I (HTLV-I) antibody titer had multiple focal brain symptoms and skin eruptions without lymphadenopathy, hepatosplenomegaly, or increased abnormal lymphocytes in the peripheral blood. No spastic paraparesis of the lower limbs was found. The encephalopathy was progressive, and she died 5 months later despite repeated intrathecal administration of methotrexate, cytosine arabinoside, and prednisolone and monthly systemic chemotherapy with doxorubicin, cyclophosphamide, vincristine, and prednisolone. Postmortem examinations identified unusual ATLL lesions composed of marked infiltrations of atypical mononuclear cells and bizarre MNGC with histiocytic granulomatous reactions in the leptomeninges, brain tissues along the Virchow-Robin spaces, skin, and kidney. Immunohistochemical stains confirmed the T-cell nature of such mononuclear cells and partially T-cell and partially macrophage nature of the MNGC, although no evidence of HTLV-I expression was found. ATLL presenting with CNS symptoms is rare. It was assumed that the direct cytopathic effects of HTLV-I were responsible for the formation of the MNGC after considering the similarity with MNGC in encephalopathy caused by the human immunodeficiency virus.