Head and Neck Pathology

, Volume 6, Issue 1, pp 111–120

Crystal-Storing Histiocytosis: Report of a Case, Review of the Literature (80 Cases) and a Proposed Classification

  • Snjezana Dogan
  • Leon Barnes
  • Wilhelmina P. Cruz-Vetrano
North American Society of Head and Neck Pathology Companion Meeting, March 18, 2012, Vancouver, British Colombia


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.


Crystal-storing histiocytosis Intracellular crystals Immunoglobulin crystals Histiocytes 

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Snjezana Dogan
    • 1
  • Leon Barnes
    • 2
  • Wilhelmina P. Cruz-Vetrano
    • 3
  1. 1.Department of PathologyMemorial Sloan-Kettering Cancer CenterNew YorkUSA
  2. 2.Emeritus Professor of PathologyUniversity of Pittsburgh Medical Center, Presbyterian HospitalPittsburghUSA
  3. 3.Laboratory ServicesAltoona Regional Health SystemAltoonaUSA

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