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- Title
Adenotonsillar pathology in mucopolysaccharidoses – lysosomal storage predominates in paracortical CD63+ cells.
- Authors
Murgasova, Lenka; Hulkova, Helena; Baresova, Veronika; Jurovcik, Michal; Stritesky, Jan; Jurickova, Katarina; Magner, Martin; Sikora, Jakub
- Abstract
Despite the adenoids are regularly removed in patients with mucopolysaccharidoses (MPS), the underlying tissue and cellular pathologies remain understudied. We characterized an (immuno)histopathologic and ultrastructural phenotype dominated by lysosomal storage changes in a specific subset of adenotonsillar paracortical cells in 8 MPS patients (3 MPS I, 3 MPS II, and 2 MPS IIIA). These abnormal cells were effectively detected by an antibody targeting the lysosomal membrane tetraspanin CD63. Important, CD63+ storage vacuoles in these cells lacked the monocytes/macrophages lysosomal marker CD68. Such a distinct patterning of CD63 and CD68 was not present in a patient with infantile neurovisceral variant of acid sphingomyelinase deficiency. The CD63+ storage pathology was absent in two MPS I patients who either received enzyme-replacement therapy or underwent hematopoietic stem cells transplantation prior the adenoidectomy. Our study demonstrates novel features of lysosomal storage patterning and suggests diagnostic utility of CD63 detection in adenotonsillar lymphoid tissue of MPS patients.
- Subjects
HEMATOPOIETIC stem cell transplantation; ADENOIDS; NIEMANN-Pick diseases; LYMPHOID tissue; PATHOLOGY; ULTRASTRUCTURE (Biology)
- Publication
Virchows Archiv: European Journal of Pathology, 2024, Vol 484, Issue 1, p135
- ISSN
0945-6317
- Publication type
Article
- DOI
10.1007/s00428-023-03662-y