LABORATORY FINDINGS AND CLINICAL COURSES OF 33 PATIENTS WITH GRANULARLYMPHOCYTE-PROLIFERATIVE DISORDERS

The hematological and immunological findings and clinical courses of 3 3 patients (13 male, 20 female; median age at presentation, 60 years) with granular lymphocyte-proliferative disorders (GLPD) are presented. Based on the surface phenotypes of peripheral blood granular lymphocy tes (GL), the GLPD were divided into CD3+ T cell-lineage GLPD (T-GLPD) and CD3- CD16+ natural killer (NK) cell-lineage GLPD (NK-GLPD). Twent y-one patients had T-GLPD, and 12 had NK-GLPD. One patient with T-GLPD and two patients with NK-GLPD had progressive clinical courses and di ed of the disease despite receiving combination chemotherapy. Twelve p atients with T-GLPD were found to have severe anemia at presentation o r during the course of the disease; four of them fulfilled the diagnos tic criteria of pure red cell aplasia, and the others had closely rela ted conditions. Six of these 12 patients were treated with cyclophosph amide, and all responded to the treatment. In 16 patients, the clinica l course was stable, and spontaneous regression was observed in two pa tients. Since some of the patients with NK-GLPD had stable clinical co urses while some had progressive clinical courses, clinical findings i n these two groups were compared. We found, taking into consideration our cases and those reviewed in the literature, that age less than 40 years, fever, lymph node swelling, hepatosplenomegaly, and GL with CD1 6(Leu-11)-CD56+CD57- phenotype and low or absent antibody-dependent ce llular cytotoxicity seemed to be predictors of a progressive clinical course.