Occurrence of angioimmunoblastic T cell lymphoma in a patient with chronicmyelomonocytic leukemia features

In a patient with recently diagnosed chronic myelomonocytic leukemia featur es, the biopsy of a peripheral lymphadenopathy seven months later revealed disorganised lymphoid tissue with a few large EBER (+) LMP1 (+) B-lymphocyt es before any treatment was given. At this time, a clonal TCR gamma rearran gement and very faint clonal IgH rearrangement were demonstrated, and the d iagnosis of angioimmunoblastic T-cell lymphoma was made. Treatment with MOP P was started, followed by Hydroxycarbamide and CHOP but the outcome was fa tal. During the evolution, there was no blastic transformation of the chron ic myelomonocytic leukemia. The T-cell lymphoma extended to abdominal lymph nodes, Waldeyer ring and bone marrow and the percentage of large LMP1 EBER (+) B-cells increased in the lymph nodes. These findings do not support a common stem cell abnormality leading to myelodysplasia in the bone marrow a nd lymphoma in peripheral lymph nodes. The lack of a clearcut light chain r estriction in the EBV infected B-cell is suggestive of a persistant EBV inf ection in polyclonal or oligoclonal activated B-cells as described in immun odepressed patients. The association of CMML features and an angioimmunobla stic T-cell lymphoma is discussed.