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Aim A case report of amyloidosis with Schnitzler syndrome. Ninety-four cases of this syndrome have been reported. This is the fourth report of amyloidosis in Schnitzler syndrome. Method Gastric and duodenal biopsies of a patient were stained with haematoxylin and eosin, Congo red and kappa and lambda immunostains. Results The patient initially presented 10 years ago with ‘urticaria’ and bone pain. Since then leucocytosis, altered liver function tests and markedly raised ESR have persisted. He has a low level IgM paraprotein. One year ago, the patient developed irritable bowel symptoms, and iron deficiency anaemia was noted. He had unremarkable gastric and duodenal biopsies. Due to persistent symptoms, repeat biopsies were performed, which showed hyaline deposits in the lamina propria and around the vessels, with apple green birefringence on Congo red staining. Kappa and lambda immunostains were negative. The patient also has hepatomegaly. Amyloid A, amylpoid P, transthyretin, lysosome and fibrinogen stains are pending. Conclusion Regular follow-up in Schnitzler syndrome is necessary due to occurrence of amyloidosis and lymphoproliferative disorders. The three previous cases had AA amyloid. Distinction from AL amyloid is necessary to guide treatment. In our case the gastrointestinal amyloidosis may have caused occult blood loss. |
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