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Abstract
We describe six rare patients with B-type CLL under the age of 30 years. All were males and four showed a relatively aggressive clinical course requiring initial chemotherapy at an early stage of their disease. Four of the six patients developed prominent generalized lymphadenopathy accompanied by splenomegaly and a rapid increase in the peripheral blood lymphocyte count or a doubling time of less than 12 months and were symptomatic. Of the six cases, one still has Stage A (0) disease, 16 months after diagnosis and has not yet been treated, while another 29 year old male, initially stage C (3) at diagnosis, progressed rapidly, despite chemotherapy and died after 42 months. Of the remaining four patients all responded well to treatment initially, however one 24 year old male has progressed rapidly from stage A (2) to Stage B (2), 14 months after initial response to chlorambucil and prednisone. Two patients remain in Stage A (0), two and eight years respectively after chemotherapy; while the patient with the longest follow up is currently 39 years old in good partial remission with Stage A (0) disease ten years after initial diagnosis, having responded well to Fludarabine, following a major flare up of his disease recently. The rare phenomenon of young patients with CLL is reviewed and management options relating to new therapeutic approaches for this sub-population of young patients are discussed.
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