T-CELL LARGE GRANULAR LYMPHOCYTIC-LEUKEMIA AND PURE RED-CELL APLASIA IN A PATIENT WITH TYPE-I AUTOIMMUNE POLYENDOCRINOPATHY - RESPONSE TO IMMUNOSUPPRESSIVE THERAPY

Clonal proliferations of large granular lymphocytes (LGLs) of T-cell o rigin characterize T-cell LGL leukemia. This disorder has been describ ed in association with rheumatoid arthritis and other autoimmune pheno mena. The presence of endocrinologic abnormalities in patients with T- cell LGL leukemia has not been previously reported, nor has T-cell LGL leukemia been described in patients with endocrinologic abnormalities . Herein we describe a young woman with type I autoimmune polyendocrin opathy, in whom pure red cell aplasia developed in association with cl onal proliferation of LGLs. Immunosuppressive therapy with cyclophosph amide resulted in remission of pure red cell aplasia, transient improv ement in hypocalcemia, and disappearance of the LGL clone. Clonal prol iferation of LGLs may be associated with autoimmune endocrinopathies. Clinicians who are responsible for the care of such patients should be aware of this possible association.