Crystal-storing histiocytosis: a clinicopathological study of 13 cases
- PMID: 26118455
- PMCID: PMC4704983
- DOI: 10.1111/his.12768
Crystal-storing histiocytosis: a clinicopathological study of 13 cases
Abstract
Aims: Crystal-storing histiocytosis (CSH) is a rare lesion composed of histiocytes with abnormal intralysosomal accumulation of immunoglobulin (Ig) as crystals, reported in patients with plasmacytic/lymphoplasmacytic neoplasms. The aims of this study were to report the clinicopathological features of 13 patients with CSH, and to describe the proteomic composition of the crystals in three cases analysed by mass spectrometry (MS).
Methods and results: There were seven men and six women, with a median age of 60 years (range, 33-79 years). CSH was generalized in one patient (8%) and localized in 12 (92%) patients, involving various sites. CSH was associated with a low-grade B-cell lymphoma with plasmacytoid differentiation or a plasma cell neoplasm in all cases. In 10 (77%) cases, CSH represented >50% of the neoplastic infiltrate. According to immunohistochemical studies, histiocytes were positive for monotypic kappa in 5 (50%) cases, and for monotypic lambda in 4 (40%) cases; in 1 (10%) case, the results were equivocal. MS analysis of the histiocyte contents in all three tested cases showed a predominance of variable-region fragments of Ig light and/or heavy chains.
Conclusions: CSH is frequently associated with an underlying lymphoplasmacytic neoplasm. MS findings suggest that Ig alterations and/or possibly defects in the ability of histiocytes to process Ig play a role in pathogenesis.
Keywords: crystal-storing histiocytosis; immunoglobulin; lymphoplasmacytic neoplasm; mass spectrometry; proteomic analysis.
© 2015 John Wiley & Sons Ltd.
Conflict of interest statement
The authors have no conflicts of interest to declare.
Figures





References
-
- Rossi G, De Rosa N, Cavazza A, et al. Localized pleuropulmonary crystal-storing histiocytosis: 5 cases of a rare histiocytic disorder with variable clinicoradiologic features. Am J Surg Pathol. 2013;37:906–912. - PubMed
-
- Bosman C, Camassei FD, Boldrini R, et al. Solitary crystal-storing histiocytosis of the tongue in a patient with rheumatoid arthritis and polyclonal hypergammaglobulinemia. Arch Pathol Lab Med. 1998;122:920–924. - PubMed
-
- Ionescu DN, Pierson DM, Qing G, et al. Pulmonary crystal-storing histiocytoma. Arch Pathol Lab Med. 2005;129:1159–1163. - PubMed
-
- Joo M, Kwak JE, Chang SH, et al. Localized gastric crystal-storing histiocytosis. Histopathology. 2007;51:116–119. - PubMed
Publication types
MeSH terms
Substances
Grants and funding
LinkOut - more resources
Full Text Sources
Other Literature Sources