IgA/kappa-restricted crystal storing histiocytosis involving the central nervous system characterized by proteomic analysis.
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.
Brent A
BA
Orr
Gary L
GL
Gallia
Ahmet
A
Dogan
Fausto J
FJ
Rodriguez
D000328:Adult; D001927:Brain Diseases / Q000209:etiology / Q000378:metabolism / Q000473:pathology*; D005260:Female; D015614:Histiocytosis / Q000209:etiology* / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007070:Immunoglobulin A / Q000378:metabolism*; D010265:Paraproteinemias / Q000150:complications* / Q000175:diagnosis*
D007070:Immunoglobulin A