Anrooij, Bjorn van (2012) CD30, mast cell load and the clinical presentation of mastocytosis. thesis, Medicine.
Full text available on request.Abstract
Mastocytosis is a rare disease characterized by tissue invasion of clonal mast cells, culminating into organ failure in some patients. Patients with evidence of organ dysfunction are categorized as high-grade systemic mastocytosis (SM) patients. Currently, no biochemical or immunohistochemical markers are able to identify these patients. Expression of CD30 is the first possible immunohistochemical marker of high-grade SM. The disease burden in patients with low-grade SM stems predominantly from symptoms of mediator release. Life threatening hymenoptera venom anaphylaxis (HVAn) is the most severe of these symptoms. Mediator release symptoms do not correlate with markers of mast cell load. However, in patients allergic to hymenoptera venom a higher mast cell load is a risk factor for HVAn. We retrospectively investigated the relation between mast cell load parameters and HVAn in 329 mastocytosis patients. We report that mast cell load parameter MIMA (methylimidazole acetic acid) is a significant (P= 0.005) independent predictor for HVAn in low-grade SM patients. MIMA is associated with a lower risk of HVAn (OR: 0.746). Expression of CD30 was quantified in 79 SM patients by measurement of the soluble form of CD30 (sCD30). We detected significantly higher (P< 0.000) levels of sCD30 in high-grade SM patients compared to low-grade SM patients. Serum sCD30 was strongly correlated with mast cell load. There existed no correlation between sCD30 and mediator release symptoms. However, sCD30 replaced MIMA as an independent predictor for less risk of HVAn. We hypothesize that mast cell expressed CD30 has an immune-regulatory role, suppressing HVAn.
| Item Type: | Thesis (Thesis) |
|---|---|
| Supervisor name: | Doormaal, J.J. van Interne Geneeskunde UMCG |
| Faculty: | Medical Sciences |
| Date Deposited: | 25 Jun 2020 10:49 |
| Last Modified: | 25 Jun 2020 10:49 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/1034 |
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