T-cell prolymphocytic leukemia (T-PLL) is a rare and highly aggressive T cell neoplasm with rapidly progressing clinical course. T-PLL accounts for 2% of mature lymphocytic leukemia in adults. Median overall survival with modern therapy is reported one to three years. Here we report a T-PLL patient with peritoneum involvement and progressive ascite despite anti-CD52 treatment.

A 65-year-old man with diabetes mellitus was admitted to hospital due to fatigue for a few weeks. Laboratory workup revealed that white blood cell count 469 × 103/µl (90% lymphocytes), haemoglobin of 11.4 g/dl, platelets of 104 × 103/µl. Medium sized atypical lymphoid cells with partial chromatin condensation and a visible nucleolus were observed on blood smear.

On physical examination, palpable inguinal lymph nodes, splenomegaly 3 cm below the rib margin and  a palpable lesion on the helix of left ear were noticed. Punch biyopsy of skin lesion was reported as a mature and immature T cell infiltration which are CD3 and CD10 positive and Tdt, CD34, CD20, CD99 negative. Flow cytometric study of peripheral blood sample was revealed that T-Chronic Lymphocytic Leukemia (T-CLL).

FMC protocol (fludarabine, mitoxantrone, and cyclophosphamide ) was initiated and followed by intravenous alemtuzumab at a dose of 3 mg on day 1, 10 mg on day 2 and 30 mg on day 3.  However after two months of anti-CD52 treatment, his general situation dete­riorated and pleural effusion and abdominal distension developed due to massive ascites. Small, mature lymphocytic cell infiltration was shown in ascites fluid on cytological examination. He died after six months of diagnosis.

T-PLL is a very aggressive disease with a median survival of less than 1 year. Not all patients diagnosed with T-PLL require treatment immediately.Currently, IV alemtuzumab (anti-CD52) is the accepted best avaliable treatment with very high response rates when given as first-line treatment. However, treatment is notcurative and a minority of T-PLL patients experience long-term disease-free survival.