ACUTE TRANSFORMATION OF CHRONIC LARGE GRANULAR LYMPHOCYTE LEUKEMIA INTO AN AGGRESSIVE FORM ASSOCIATED WITH PREFERENTIAL ORGAN INVOLVEMENT

In most patients with large granular lymphocyte (LGL) leukemia, the di sease appears to progress slowly if at all, and no therapy is generall y required. We present a patient with CD3+ CD8+ CD16+ LGL leukemia, wh o showed a benign clinical course for more than 7 months without thera py, but subsequently developed aggressive disease. A feature of consid erable interest was the transformation into an acute or aggressive for m in this patient affected preferentially the liver and spleen, and wa s not associated with the hematologic features of the circulating leuk emic cells, as assessed by morphologic, phenotypic and molecular analy ses. The patient was treated with combination chemotherapy; this resul ted in clinical remission with persistence of the abnormal clone in th e peripheral blood. The mechanism responsible for the preferential org an involvement and the process of progression from the chronic to the acute form in this case are discussed from the viewpoint of a lymphopr oliferative disorder.