Bone marrow - nonneoplastic
Systemic disorders
Mucopolysaccharidosis type VII



Topic Completed: 1 December 2006

Revised: 10 January 2019

Copyright: (c) 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Mucopolysaccharidosis type VII bone marrow


Nat Pernick, M.D.
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Cite this page: Pernick N Mucopolysaccharidosis type VII. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/bonemarrowmps7.html. Accessed December 5th, 2019.
Definition / general
  • Very rare lysosomal storage disease due to deficiency of beta glucuronidase, which degrades glycosaminoglycans
  • Causes progressive accumulation of glycosaminoglycans and subsequent lysosomal distension in multiple tissues
Microscopic (histologic) description
  • Alder-Reilly granules in neutrophils, monocytes, basophils and eosinophils
  • Cytoplasmic inclusions surrounded by clear vacuoles in lymphocytes, plasma cells, osteoblasts, macrophages
Positive stains
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