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A patient with a severe glottic stenosis and saddle nose.
Acta Clin Belg. 2017 Apr;72(2):130-132
Authors: Sabato V, Vanderveken OM, Van den Wyngaert T, Van Laer C, Ebo D
Abstract
We present the case of a 39-year-old man with a severe glottis stenosis. The saddle nose, images of laryngotracheal stenosis and the (FDG) positron-emission tomography/computed tomography lead to a final diagnosis of relapsing polychondritis. In the patient a coexistent myelodysplastic syndrome was diagnosed. Moreover, the elevated total IgG4 exceeding 135 ml/dl requested additional immunochemistry for detection of IgG4-bearing plasma cells in the biopsies. The patient underwent an allogenic stem cell transplantation and died on day 40 after the transplantation because of an acute steroid-resistent graft vs host.
PMID: 27075792 [PubMed - indexed for MEDLINE]
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