Crystal-Storing Histiocytosis Complicating Primary Pulmonary Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue

Abstract

Crystal-storing histiocytosis is an uncommon form of nonneoplastic histiocytic proliferation that in most patients complicates an underlying lymphoproliferative or plasma cell disorder. Lung is a common site of involvement in patients with localized disease. We present an illustrative example from a 54-year-old woman with an asymptomatic solitary lung nodule. The tumor was characterized by sheets of histiocytes with abundant cytoplasm expanded by distinctive eosinophilic inclusions. Focal necrosis was present. Aggregates of monocytoid lymphocytes and clusters of peribronchiolar plasma cells were overshadowed by the histiocytic infiltrate. Immunohistochemical stains showed CD68 staining in nonneoplastic histiocytes and CD20 staining in monocytoid lymphocytes. In situ hybridization studies showed κ light-chain restriction in plasma cells. These results, combined with the histologic findings, supported the diagnosis of crystal-storing histiocytosis complicating marginal zone lymphoma of mucosa-associated lymphoid tissue. We review the literature pertaining to pulmonary crystal-storing histiocytosis, highlighting the differential diagnosis for this rare phenomenon.