Increased Procoagulant Response of Monocytes from Patients with Familial Mediterranean Fever

Summary

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.

Keywords

Monocytes - Procoagulant activity - Familial Mediterranean Fever - Interleukin 1

• References

• 1 Eliakim M, Levy M, Ehrenfeld M. Reccurent polyserositis. Familial Mediterranean Fever. Periodic disease. Elsevier Amsterdam 1981; 1-203
• 2 Zemer D, Pras M, Sohar E, Modan M, Cabili S, Gafni J. Colchicine in the prevention and treatment of the amyloidosis of Familial Mediterranean Fever. N Engl J Med 1986; 314: 1001-1005
• 3 Dinarello CA, Chusid MJ, Fauci AS, Gallin JI, Dale DC, Wolff SM. Effect of prophylactic colchicine therapy on leukocyte function in patients with familial Mediterranean fever. Arthritis Rheum 1976; 19: 618-622
• 4 Schlesinger M, Ilfeld D, Handzel ZT, Altman Y, Kuperman O, Levin S, Bibi C, Netzer L, Trainin N. Effect of colchicine on immunore-gulatory abnormalities in Familial Mediterranean Fever. Clin Exp Immunol 1983; 54: 73-79
• 5 Matzner Y, Brzezinski A. C₅a - inhibitor deficiency in peritoneal fluids from patients with Familial Mediterranean Fever. N Engl J Med 1984; 311: 287-290
• 6 Shamir H, Pras M, Sohar E, Gafni J. Cryofibrinogen in Familial Mediterranean Fever. Arch Intern Med 1974; 134: 125-126
• 7 Mosesson MW, Wautier JL, Amrani DL, Dervichian M, Cattan D. Evidence for circulating fibrin in Familial Mediterranean Fever. J Lab Clin Med 1982; 99: 559-567
• 8 Bar-Eli M, Gallily R, Levy M, Eliakim M. Monocyte function in Familial Mediterranean Fever. Am J Med Sci 1977; 274: 265-270
• 9 Aisen PS, Haines KA, Given W, Abramson SB, Pras M, Serhan C, Hamberg M, Samuelsson B, Weissmann G. Circulating hydroxy fatty acids in Familial Mediterranean Fever. Proc Natl Acad Sci 1985; 82: 1232-1236
• 10 Bevilacqua MP, Pober JS, Majeau GR, Cotran RS, Gimbrone MA. Interleukin 1 (IL-1) induces biosynthesis and cell surface expression of procoagulant activity in human vascular endothelial cells. J Exp Med 1984; 160: 618-623
• 11 Li CY, Lam KW, Yam LT. Esterases in human leukocytes. J Histochem Cytochem 1973; 21: 1-12
• 12 Bevilacqua MP, Amrani D, Mosesson MW, Bianco C. Receptors for cold - insoluble globulin (plasma fibronectin) on human monocytes. J Exp Med 1983; 153: 42-60
• 13 Markwell MA K, Haas SM, Bieber LL, Tolbert NE. A modification of the Lowry procedure to simplify protein determination in membrane and lipoprotein samples. Anal Biochem 1978; 87: 206-210
• 14 Helin HJ, Fox RI, Edgington TS. The instructor cell for the human procoagulant monocyte response to bacterial lipopolysaccharide is a Leu-3a⁺T cell by fluorescence - activated cell sorting. J Immunol 1983; 131: 749-752
• 15 Meltzer MS, Oppenheim JJ. Bidirectional amplification of mac-rophage-lymphocyte interactions: enhanced lymphocyte activation factor production by activated adherent mouse peritoneal cells. J Immunol 1977; 118: 77-82
• 16 Osterud B, Flaegstad T. Increased tissue thromboplastin activity in monocytes of patients with meningococcal infection: related to an unfavourable prognosis. Thromb Haemostas 1983; 49: 5-7
• 17 Edwards RL, Rickies FR, Cronlund M. Abnormalities of blood coagulation in patients with cancer. J Lab Clin Med 1981; 98: 917-928
• 18 Edwards RL, Rickies FR. Delayed hypersensitivity in man: effects of systemic anticoagulation. Science 1978; 200: 541-543
• 19 Lyberg T, Prydz H, Baklien K, Hoyeraal HM. Effect of immune complex-containing sera from patients with rheumatic diseases on thromboplastin activity of monocytes. Thromb Res 1982; 25: 193-202