CUTANEOUS INVOLVEMENT IN PRELYMPHOMATOUS ANGIOIMMUNOBLASTIC LYMPHADENOPATHY

We describe prelymphomatous angioimmunoblastic lymphadenopathy with cu taneous involvement in a 73-year-old female patient. A maculopapular s kin eruption was the first sign of the disease. Skin histology showed extensive perivascular and periadnexal mixed lymphoid infiltrates incl uding centroblasts and immunoblasts with a high proliferative index an d with focal erythrocyte extravasation. Lymph node histology confirmed the diagnosis, showing nearly complete effacement of the follicular a rchitecture, a mixed lymphoid infiltrate, and numerous high endothelia l venules in an expanded T-cell zone. Immunohistochemistry, however, d emonstrated preservation of at least some follicular structures. T-cel l receptor gene rearrangement analysis revealed oligoclonal patterns i n both lymph node and skin specimens. In contrast, immunoglobulin heav y-chain gene rearrangement analysis revealed a polyclonal pattern. Acc ordingly, the disease was classified as a prelymphomatous stage of ang ioimmunoblastic lymphadenopathy with dysproteinemia (AILD) with specif ic involvement of both lymph node and skin. The patient was treated wi th high-dose corticosteroids, and long-lasting remission was induced. In contrast to our case, most reported cases of AILD show a monoclonal T-cell pattern indicating AILD-type lymphoma. Therefore we discuss th e concept of prelymphomatous AILD developing into AILD-type lymphoma. Persistence of some antigenic stimulus may induce the proliferation of a monoclonal population of lymphoid cells from a polyclonal backgroun d in a multistep fashion. Proper treatment of AILD at an early, prelym phomatous stage may protract or inhibit development of full-blown, fat al AILD-type lymphoma.