IgA/kappa-restricted crystal storing histiocytosis involving the central nervous system characterized by proteomic analysis.

Abstract

Crystal storing histiocytosis (CSH) is a rare disorder characterized by the accumulation of crystalline material in the cytoplasm of histiocytes. Involvement of the central nervous system (CNS) with CSH is extremely rare. Herein, we report a case of crystal storing histiocytosis involving the CNS. Using immunohistochemistry and mass spectrometry we demonstrate that the disease resulted from an IgA-κ restricted plasma cell dyscrasia. CNS-CSH represents a rare clinicopathologic entity with an indolent course, usually lacking systemic manifestations.

• Authors: Brent A Orr, Gary L Gallia, Ahmet Dogan, Fausto J Rodriguez
• Journal: Clinical neuropathology (Clin Neuropathol) 2014 Jan-Feb Vol. 33 Issue 1 Pg. 23-8 ISSN: 0722-5091 [Print] Germany
• PMID: 23924753 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural)

Chemical References

• Immunoglobulin A

Topics

• Adult
• Brain Diseases (etiology, metabolism, pathology)
• Female
• Histiocytosis (etiology, metabolism, pathology)
• Humans
• Immunoglobulin A (metabolism)
• Paraproteinemias (complications, diagnosis)