Crystal storing histiocytosis

Author: David Lynch, M.D. (see Authors page)

Revised: 20 November 2018, last major update November 2016

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed Search: Crystal storing histiocytosis

Related topics: Lung - nontumor - Crystal storing histiocytosis, Oral cavity - Crystal storing histiocytosis
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Cite this page: Crystal storing histiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/hematologycsh.html. Accessed December 18th, 2018.
Definition / general
  • Rare; crystalline material accumulates in cytoplasm of histiocytes; usually kappa light chain origin (Histopathology 2016;68:482)
  • Adults are affected with a wide age range; men and women affected nearly equally
  • Most commonly affects the head and neck, lung, kidney, bone marrow and lymph nodes, although nearly any site may be involved (Head Neck Pathol 2012;6:111)
  • Strongly associated with underlying plasma cell neoplasm or lymphoma with plasma cell differentiation
  • A minority of cases are associated with nonneoplastic causes such as infections and autoimmune disease
Essential features
  • Rare disease with crystalline material in cytoplasm of histiocytes
  • May affect any organ system
  • Strongly associated with underlying lymphoid or plasma cell neoplasm
Case reports
Microscopic (histologic) description
  • Cytologically benign histiocytes with abundant cytoplasm filled with many refractile eosinophilic crystals which may be needle-like or rhomboid in shape
  • Neoplastic lymphocytes and plasma cells may be present
  • Histiocytes often compose the majority of cellular elements, potentially obscuring an underlying neoplasm
Microscopic (histologic) images
Images hosted on Pathout server:
Missing Image

Bone marrow core and CD68
Contributed by David Lynch, M.D.

Cytology images

Bone marrow aspirate
Contributed by David Lynch, M.D.

Positive stains
  • Crystals are typically monoclonal, immunoreactive with kappa or lambda light chain
  • Histiocytes are positive for CD68 and CD163