Hematological considerations on mucopolysaccharidoes
Popa, G.; Pencea, V.; Niculescu, M.; Gavrilescu, M.; Iordächescu, L.; Moscovici, R.
Revista Medico-Chirurgicala a Societatii de Medici Si Naturalisti Din Iasi 83(4): 645-649
1979
ISSN/ISBN: 0048-7848 PMID: 162077 Document Number: 4198
The authors report on three children with a clinical, biologic and hematologic syndrome of mucopolysaccharidosis, each one presenting a different clinical type of the disease: (1) Hunter's syndrome; (2) Morquit syndrome; (3) Maroteaux-Lamy syndrome. The diagnostic importance of the X-ray examination and determination of urinary mucopolysaccharides is emphasized. Hematological studies revealed the presence of accumulations of metachromatic granules in the medullary and peripheral cells of all the cellular lines that metabolize mucopolysaccharides, confirming the hypothesis according to which the hematologic anomalies observed in these syndromes reflect general constitutional disorders, namely the metabolism of mucopolysaccharides.
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