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

Brent A. Orr, Gary L. Gallia, Ahmet Dogan, Fausto J. Rodriguez

Research output: Contribution to journalArticle

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-? restricted plasma cell dyscrasia. CNS-CSH represents a rare clinicopathologic entity with an indolent course, usually lacking systemic manifestations.

Original languageEnglish (US)
Pages (from-to)23-28
Number of pages6
JournalClinical neuropathology
Volume33
Issue number1
DOIs
StatePublished - Jan 1 2014

Keywords

  • Brain tumor
  • Crystal storing histiocytosis
  • Mass spectrometry
  • Plasma cell

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology

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