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 lymphoplasmacytic lymphoma is presented. Unlike previous cases, this patient presented with signs and symptoms suggestive of Weber-Christian disease. Biopsy 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 immunoglobulin (Ig)G heavy chain. Polymerase chain reaction (PCR) analysis of CDR-II* region of the immunoglobulin heavy chain locus confirmed monoclonality of the lymphoplasmacytic cells in the nodule. Electron microscopy showed polygonal-shaped amorphous crystals, characteristic of immunoglobulin in the histiocytic cells. Crystal-storing histiocytosis should be examined by immunohistochemical and DNA analysis to confirm or exclude the possibility of lymphoplasmacytic lymphoma.
Department of Diagnostic Pathology, Kanto Teishin Hospital, Tokyo, Japan.
M
M
Harada
M
M
Shimada
M
M
Fukayama
T
T
Kaneko
K
K
Kitazume
S W
SW
Weiss
D000328:Adult; D003460:Crystallization; D004247:DNA / Q000032:analysis; D003937:Diagnosis, Differential; D015321:Gene Rearrangement; D015614:Histiocytosis / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000032:analysis; D007150:Immunohistochemistry; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D010201:Panniculitis, Nodular Nonsuppurative / Q000175:diagnosis*
D007143:Immunoglobulin Heavy Chains; D004247:DNA