CRYSTAL-STORING HISTIOCYTOSIS ASSOCIATED WITH LYMPHOPLASMACYTIC LYMPHOMA MIMICKING WEBER-CHRISTIAN DISEASE - IMMUNOHISTOCHEMICAL, ULTRASTRUCTURAL, AND GENE-REARRANGEMENT STUDIES

A case of crystal-storing histiocytosis associated with lymphoplasmacy tic lymphoma is presented. Unlike previous cases, this patient present ed with signs and symptoms suggestive of Weber-Christian disease. Biop sy of subcutaneous nodules showed numerous deposits of crystal-storing histiocytes with lymphoplasmacytic cells, the latter exhibiting light chain restriction (lambda-chain) with a predominance of immunoglobuli n (Ig)G heavy chain. Polymerase chain reaction (PCR) analysis of CDR-I II region of the immunoglobulin heavy chain locus confirmed monoclonal ity of the lymphoplasmacytic cells in the nodule. Electron microscopy showed polygonal-shaped amorphous crys tals, characteristic of immunog lobulin in the histiocytic cells. Crystal-storing histiocytosis should be examined by immunohistochemical and DNA analysis to confirm or exc lude the possibility of lymphoplasmacytic lymphoma. HUM PATHOL 27:84-8 7. Copyright (C) 1996 by W.B. Saunders Company