Crystal-Storing Histiocytosis: Report of a Case, Review of the Literature (80 Cases) and a Proposed Classification
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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.
KeywordsCrystal-storing histiocytosis Intracellular crystals Immunoglobulin crystals Histiocytes
We thank Raymond E. Felgar, MD, PhD of the Division of Hematopathology, University of Pittsburgh Medical Center, Presbyterian Hospital for reviewing this case, Jay Arlick, DMD, oral surgeon, Clearfield Hospital, Clearfield, Pennsylvania, who removed the lesion and provided some evaluation and follow-up and Jacinthe Chenevert, MD, Division of Endocrine and Head and Neck Pathology, University of Pittsburgh Medical Center, Presbyterian Hospital for collecting some published data.
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