Autoimmune Thrombocytopenic Purpura Complicating Chronic Lymphocytic Leukemia: Analysis of 60 Patients.

Autoimmune thrombocytopenic purpura (AITP) represents the autoimmune condition most frequently associated with chronic lymphocytic leukemia (CLL) after autoimmune haemolytic anemia. However, the main characteristics and outcome of AITP in the course of CLL, as well as the impact of this complication in the natural history of the tumor remain unknown.

We identified 60 consecutive patients with CLL who developed AITP, representing 3,5% of CLL patients diagnosed in the three participating centers between 1995 and 2004. To be included in this study patients had to experience at least one episode of AITP, which was defined as the occurrence of acute and severe thrombocytopenia in the presence of normal or augmented number of megakariocytes in the bone marrow, without extensive lymphoid marrow infiltration, splenomegaly or recent cytotoxic treatment. A complete response (CR) to AITP treatment was defined by a platelet count > or = 150×10(9)/L, and a partial response (PR) by a platelet count > 50×10(9)/L or by an increase of at least twofold the initial level. Remaining patients were considered as no responders (NR).

Median age of our 60 patients was 65 years (range 48–83) and 40 were males. At CLL presentation RAI stage was 0 to 2 in 88%, time to CLL treatment was 13,8 months (range 0–120), while first line treatment for CLL consisted of Chlorambucile alone (Chl) in 73% of patients with 18% of patients that received no treatment for their malignancy. Median overall survival was 57 months. AITP occurred concomitantly to CLL diagnosis in 13 patients (22%), while median time to AITP for remaining 47 patients was 30 months (range 2–147). The median platelet count at AITP diagnosis was 23 × 10(9)/L(range, 1–81). Twenty-five patients (42%) presented with moderate bleeding signs at AITP diagnosis, while 4 patients (7%) experienced severe hemorrhagic episodes, requiring hospitalization and blood transfusions. Fifty-two of the 60 patients (87%) received at least one treatment for AITP: 32 patients received i.v.Ig alone or in combination with steroids, leading to a short-term NR in 66% (CR 19%, PR 15%); nine patients underwent splenectomy and 7 (78%) experienced a durable CR; patients who were treated with chemotherapy (Chl, COP, CVP) +/− steroids had at least a PR in 73% of cases. With a median follow-up from AITP onset of 35 months, 17 of the 52 treated patients are still NR (33%) and 13 of them are on treatment.

In our series of patients with CLL and AITP we observed an unexpectedly short survival regardless of a large prevalence of low RAI stages at diagnosis. Treatment of AITP with i.v. Ig +/− steroids leaded to a low rate of short-term responses, while splenectomy and chemotherapy seemed sufficiently adequate therapeutic approaches.