Hematology
Other
Crystal storing histiocytosis


Topic Completed: 1 November 2016

Revised: 5 March 2019

Copyright: 2003-2019, 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: Lynch D Crystal storing histiocytosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/hematologycsh.html. Accessed September 18th, 2019.
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

Contributed by David Lynch, M.D.

Missing Image

Bone marrow core and CD68

Cytology images

Contributed by David Lynch, M.D.

Bone marrow aspirate

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