Introduction: T-cell prolymphocytic leukaemia (T-PLL) is a rare post-thymic T-cell malignancy with an aggressive clinical course. It has generally been resistant to alkylating chemotherapy, but some effect has been observed with the purine analog 2-deoxycoformicin with documented partial or complete response rates in up to 45% of patients. Treatment with monoclonal antibodies against CD 52 has been shown to be highly effective in T-PLL with response rates of up to 76%. This may allow for further consolidating treatment with high-dose chemotherapy with autologous stem cell support or allogeneic stem cell transplantation.
Materials and methods: Five patients treated at Rikshospitalet University Hospital are evaluated and the literature on T-PLL is reviewed.
Results and interpretation: Four of our patients were treated with alemtuzumab. Three showed complete or partial response. One patient underwent allogeneic stem cell transplantation with an HLA-identical sibling, but died on day 21 as a result of transplantation complications. The treatment is generally well tolerated; the principal management problem is immunosuppression, as shown in one patient who developed a varicella-zoster meningoencephalomyelitis as a consequence of not receiving antiviral prophylaxis. The main infusion-related adverse effects are fever and chills.