crystal storing histiocytosis

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crystal storing histiocytosis2016-11-28T23:50:10+00:00

crystal storing histiocytosis

crystal storing histiocytosis image from New Medical Terms

crystal storing histiocytosiss


Definition An über rare–less than 100 cases in the world literature–complication of low-grade B cell lymphoma and monoclonal gammopathies, including myelomas, which is characterised by lysosomal storage of kappa light chain crystals

Clinical findings It may be associated with adult Fanconi syndrome, chronic tubulo-interstitial nephritis with accumulation of monoclonal kappa light chain crystals within proximal tubular cells

Differential diagnosis Benign rhabdomyoma 

Micro Sheets of macrophages and histiocytes filled with multiple needle-shaped, periodic acid-Schiff-negative refractile crystals (left image). CD68 positivity (right image) confirms macrophage/histiocytic lineage of cells.

Molecular pathology CSH is due to unique mutations in the V domains of monoclonal kappa light chains that differ from Fanconi syndrome. 

References Nephrol. Dial. Transplant. (2010) 25 (9): 2982-2990 

doi: 10.1093/ndt/gfq129

Head Neck Pathol. 2012 Mar;6(1):111-20.

doi: 10.1007/s12105-011-0326-3 (image)

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