fibrin and Familial-Mediterranean-Fever

Familial Mediterranean Fever (FMF) is an inherited disease of unknown etiology characterized by recurrent inflammatory episodes. Circulating fibrin was found in patients with FMF in absence of clinical manifestation of thrombosis and was statistically less frequently observed in patients treated with colchicine. These results suggest a cellular dysfunction. Therefore, we examined the procoagulant activity (PCA) of isolated mononuclear leukocytes and purified monocytes from FMF patients (n = 20). No PCA was detectable on freshly-isolated monocytes. After several hours of culture. FMF monocytes contained more PCA than control cells and the difference was more marked after endotoxin stimulation. Data obtained with coagulation factor-deficient plasma and anti-human apoprotein III antiserum indicated that the enhanced PCA in FMF monocytes is thromboplastin-like. Lysozyme and interleukin 1 production by monocytes were similar in patients and controls. The increased monocyte PCA appears to be due to an intrinsic and selective higher responsiveness of monocytes.

Topics: Adult; Blood Coagulation; Blood Coagulation Tests; Familial Mediterranean Fever; Female; Fibrin; Humans; Male; Middle Aged; Monocytes; Platelet Count; Reference Values

Cryofibrinogenemia was found in 10 of 24 plasma samples (42%) from subjects with FMF. This precipitate was found during active disease as well as during intervals between crises. We found a higher incidence of cryofibrinogenemia in subjects with mild to moderately severe disease not being treated with colchicine (six of eight) as compared with colchicine-treated subjects who were in partial or complete clinical remission (four or 16; p less than 0.02). All cryofibrinogen precipitates contained fibrin, as assessed by electrophoretic analyses showing the presence of multimeric crosslinked forms of fibrin(ogen) linked by gamma-dimers. This finding in clinical specimens supports the hypothesis that fibrin in an obligatory component of cryofibrinogen. Fibrin was also found in HPF (two of six specimens) prepared from cryofibrinogen-negative FMF plasmas, thus showing that soluble forms of fibrin are even more prevalent in this disorder than is indicated by the frequent finding of cryofibrinogenemia.

Topics: Adolescent; Adult; Child; Colchicine; Cold Temperature; Cryoglobulins; Electrophoresis, Polyacrylamide Gel; Familial Mediterranean Fever; Fibrin; Fibrinogen; Fibrinogens, Abnormal; Humans; Racial Groups