title: Crystal-storing histiocytosis: report of a case, review of the literature (80 cases) and a proposed classification.
abstract: We report a case of crystal storing histiocytosis (CSH) of the upper lip and cheek in a 51-year-old woman and review the clinicopathologic features of 80 cases in the literature. These occurred in 41 men and 39 women with a respective mean age of 59 and 61 years (range 17-81 years). Forty-six patients (58%) had localized CSH, and, of these, 16 (35%) occurred in the head and neck, with the most common site being the eye/orbit. The remaining 34 patients (42%) had generalized CSH primarily involving bone marrow, liver, lymph nodes, spleen and/or kidney. Regardless of whether the CSH was localized or generalized, the vast majority of patients (90%) had an underlying lymphoproliferative or plasma cell disorder, especially multiple myeloma, lymphoplasmacytic lymphoma, or monoclonal gammopathy of undetermined significance. In 7 cases (8.8%), the CSH was associated with a variety of benign disorders, often with an inflammatory background, and no evidence of a clonal lymphoproliferative or plasma cell disorder. Treatment and prognosis varied according to the underlying disease. A classification of CSH based on etiology and/or associated disease and chemical composition of the crystal is proposed, rare non-immunoglobulin variants of CSH are discussed, and a differential diagnosis of other potentially confusing lesions is provided.
authors.affiliation: Department of Pathology, Memorial Sloan-Kettering Cancer Center, 1275 York Ave, New York, NY 10065, USA. dogans@mskcc.org
authors.forename: Snjezana
authors.initials: S
authors.lastname: Dogan
authors.forename: Leon
authors.initials: L
authors.lastname: Barnes
authors.forename: Wilhelmina P
authors.initials: WP
authors.lastname: Cruz-Vetrano
mesh_terms: D003460:Crystallization; D003937:Diagnosis, Differential; D005260:Female; D006644:Histiocytes / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D015614:Histiocytosis / Q000145:classification* / Q000473:pathology*; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D008046:Lip / Q000473:pathology*; D008854:Microscopy, Electron; D008875:Middle Aged
chemical_list: D007136:Immunoglobulins