Bone Marrow - nonneoplastic
Mucopolysaccharidosis Type VII
Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 8 October 2013, last major update December 2006
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- 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
- Alder-Reilly granules in neutrophils, monocytes, basophils and eosinophils
- Cytoplasmic inclusions surrounded by clear vacuoles in lymphocytes, plasma cells, osteoblasts, macrophages
- Lymphocytes - acid phosphatase
End of Bone Marrow - nonneoplastic > Systemic disorders > Mucopolysaccharidosis Type VII
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