Large Granular Lymphocyte Proliferative Disease - 21 Belgian Cases and Review of the Literature

We report the findings in 21 Belgian patients (12 males and 9 females, median age 61 years) with LGLPD. Symptoms at presentation included infection (n = 9), weight loss (n = 5), asthenia (n = 9). pruritus (n = 2) and arthralgia (n = 7). Four patients were asymptomatic. The main clinical findings were hepatomegaly (n = 5), splenomegaly (n = 8), lymph node enlargement ( n = 3) and arthritis (n = 5). All patients had an increased LGL count associated with anemia (n = 12), neutropenia (n = 17), often < 0,5 . 10(9)/L (n = 10) and thrombocytopenia (n = 6).Three patterns of lymphocyte surface markers were observed: CD3+CD4-8+(14 patients), CD3+CD4-8+(5 patients) and CD3+CD4+8-(1 patient). An abnormal karyotype was found in 2 patients. T-cell receptor gene was rearranged in all cases tested (9/9). Our findings are in agreement with recent data from the literature.