NATURAL-KILLER-CELL LYMPHOPROLIFERATIVE DISORDER OF GRANULAR LYMPHOCYTES PRESENTING AS HEMOLYTIC-ANEMIA - CASE-REPORT AND REVIEW OF THE LITERATURE

Lymphoproliferative disorders of granular lymphocytes (LDGLs) have rec ently been hypothetically defined based on their immunophenotype, func tion, and clonality. The majority (129/161) of LDGLs are of the T-cell subset, which is typically associated with neutropenia and occasional ly with anemia. The remainder (32/161) are of natural killer cell orig in. This subset is associated with neutropenia (52% of cases) and anem ia (60% of cases). The anemia most often has no immediately apparent c ause. The Coombs test was negative in all cases reported. There is onl y one reported case of an associated Coombs-negative hemolytic anemia. We report an unusual case of natural killer cell LDGL, defined by mor phology and immunophenotypic analysis, presenting as a de novo Coombs- negative hemolytic anemia. The anemia was resistant to steroid therapy . Therapeutic splenectomy resulted in a temporary resolution of the he molytic process. The splenic pathology is also described. (C) 1995 Aca demic Press, Inc.