P01-003 – Bleeding disorder in FMF

O Karadag,I Ertenli,N Sayinalp,T Akin Telli

doi:10.1186/1546-0096-11-s1-a7

Abstract

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.

Highlights

The most serious complication in Familial Mediterranean fever (FMF) is the development of amyloidosis, which usually determines the prognosis
The clinical features of amyloidosis are dependent on the organs involved, type of amyloidosis, rate of amyloid deposition and amount of amyloid fibrils
Amyloid deposition is the main cause of abnormal bleeding and coagulation factor deficiencies, hyperfibrinolysis, platelet dysfunction and amyloid angiopathy with increased fragility of blood vessels can be regarded as other important pathogenetic factors

Summary

Introduction

Introduction 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. Amyloid deposition is the main cause of abnormal bleeding and coagulation factor deficiencies, hyperfibrinolysis, platelet dysfunction and amyloid angiopathy with increased fragility of blood vessels can be regarded as other important pathogenetic factors.

Results

Conclusion