P01-003 – Bleeding disorder in FMF

Excerpt

The most serious complication in Familial Mediterranean fever (FMF) is the development of amyloidosis, which usually determines the prognosis. Amyloid deposition can be systemic or organ-specific. The clinical features of amyloidosis are dependent on the organs involved, type of amyloidosis, rate of amyloid deposition and amount of amyloid fibrils. Organ dysfunction can cause life-threatening bleeding. Amyloid deposition is the main cause of abnormal bleeding but also coagulation factor deficiencies, hyperfibrinolysis, platelet dysfunction and amyloid angiopathy with increased fragility of blood vessels can be regarded as other important pathogenetic factors. Herein a case of FMF amyloidosis with splenomegaly, refractory cytopenia and bleeding disorder is presented. …