Adult T-cell Leukemia Presenting with Pancytopenia Followed by Diabetes Insipidus

Abstract

A case of adult T-cell leukemia (ATL) presenting with pancytopenia and comlicating diabetes insipidus was reported. A 40-year old man was admitted to our hospital because of easy fatigability in Feburary 1985. He was physically unremarkable except for cervical and inguinal lymphadenopathy and moderate hepatomegaly. Blood examination revealed Hb 5.9g/dl, platelets 20,000/mm³, and leukocytes 2,400/mm³ with 33% abnormal lymphocytes showing convoluted or lobulated nuclei. Bone marrow histologically showed significant hemopoietic depletion and scattering foci of abnormal lymphocytes without fibrosis. Lymph node biopsy showed malignant lymphoma, diffuse, medium cell type. T cell subset analysis showed cells from peripheralblood and lymph node to have helper/inducer phenotype. Anti-ATLA antibody was positive. From these findings, he was diagnosed as ATL. In late April 1985, polydipsia, polyuria (>10L/day) with a specific gravity of 1004-1008 appeared. Brain CT showed suprasellar mass. CSF cytology was positive for leukemic cells. The water-depression test and an administration of vasopressin suggested central diabestes insipidus. He died of pulmonary complication 3 months after onset of diabetes insipidus although chemotherapy and radiation therapy resulted in diminution of suprasellar mass and reduction of urine volume. Autopsy revealed necrosis of the infundibulum without leukemic infiltlation In addition, peripheral blood and lymph node mononuclear cells and serum did not suppress either normal erythroid or granuloid colony-formation.