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

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

doi:10.5414/np300645

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.

Highlights

We describe a case of immune Crystal storing histiocytosis (CSH) involving the central nervous system in a 38-year-old woman with seizures
We used immunohistochemistry and mass spectrometry to determine that the CSH in this case was associated with an IgA-κ restricted plasma cell proliferation
Crystal storing histiocytosis (CSH) is a rare disorder defined by the accumulation of crystalline material in the cytoplasm of histiocytes

Summary

Introduction

Crystal storing histiocytosis (CSH) is a rare disorder defined by the accumulation of crystalline material in the cytoplasm of histiocytes. We describe a case of immune CSH involving the central nervous system in a 38-year-old woman with seizures. We used immunohistochemistry and mass spectrometry to determine that the CSH in this case was associated with an IgA-κ restricted plasma cell proliferation.

Results

Conclusion