Bone marrow - nonneoplastic
Systemic disorders
Mucopolysaccharidosis type VII

Author: Nat Pernick, M.D. (see Authors page)

Revised: 28 June 2017, last major update December 2006

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Mucopolysaccharidosis type VII bone marrow

Cite this page: Mucopolysaccharidosis type VII. website. Accessed October 24th, 2018.
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