Abstract
A 69-year-old female was admitted to our hospital on February 6, 1984, because of fatigability, lymphadenopathy, progressive anemia and rash lasting for several months. Bone marrow examination, which had been performed a month before the admission, revealed a compatible finding with pure red cell aplasia. Since then, leukopenia and thrombocytopenia with bleeding tendency had developed. On admission, physical examination revealed hepatosplenomegaly, in addition to lymphadenopathy and purpura all over the body. Laboratory findings were as follows: Hb 5.2 g/dl, RBC 208×104/μl, reticulocytes 0‰, platelets 1.8×104/μl, WBC 3,600/μl and increased in polyclonal gammaglobulin. A biopsy specimen showed hypoplastic bone marrow with increased fibrous tissue. As immunological abnormalities, direct and indirect Coombs test, anti-nuclear antibody, anti-DNA antibody, anti-smooth muscle antibody and thyroid-microsome test were all positive. A diagnosis of Angioimmunoblastic lymphadenopathy was made on the basis of lymphnode biopsy findings and clinical features described above. She achieved clinical remission by administration of prednisolone and results of laboratory test including bone marrow findings were improved to near normal range. But, she was died of secondary bilialy liver cirrhosis and bronchopneumonia 10 months after in the course of tapering off the doses of prednisolone. At autopsy, there was universal depletion of lymphoid tissue with cellular reduction and thickning walls of the vessels in the lymphnodes.
