Nephrotic syndrome and chronic renal insufficiency associated with essential cryofibrinogenemia

Abstract

Cryofibrinogenemia (CF) is defined by the presence of circulating cold insoluble complexes of fibrin, fibrinogen, fibronectin, and fibrin split products with albumin, immunoglobulins and plasma proteins that precipitate from the patient’s plasma in the cold. Cryofibrinogen clots form when the patient’s plasma is cooled to 48C and stored in the cold for up to 72 h [1,2]. Cryofibrinogen clots typically redissolve upon warming to normal body temperature down to 378C. Lesions in patients with symptomatic CF are attributed to cryoprecipitation of patient’s native fibrinogen in the small and medium sized arteries. Symptoms of CF are mainly cutaneous including purpura, ecchymosis, ulcerations, livido reticularis, ischaemic necrosis and rarely gangrene [3,4]. Cold exposed body areas like hands, feet, ears, and nose are commonly affected. While essential CF is a rare, nonfatal, often asymptomatic disorder that develops spontaneously in previously healthy persons, the secondary form occurs in patients suffering from underlying inflammatory and neoplastic disorders. The most common conditions associated with CF are malignancy, collagen vascular disease, diabetes mellitus, vasculitis and active infection. Renal lesions rarely develop in patients with essential CF [5–7]. Conversely, a high incidence of CF was identified in a cohort of French patients with IgA nephropathy [8]. CF has also been reported in patients on renal replacement therapy, either dialysis or transplantation [9,10]. This is the first reported case of chronic essential symptomatic CF in association with membrano-proliferative glomerulonephritis (MPGN). Case report