Severe bleeding, cryofibrinogenaemia (CF) and rheumatoid arthritis (RA)

BLANCO AN; GROSSO SH; BASTOS L; SPERONI J; SÁNCHEZ-LUCEROS A; ALBERTO MF; AIXALÁ M; MESCHENGIESER SS; LAZZARI MA

Boston, USA

Congreso; XXII Congress of the International Society on Thrombosis and Haemostasis; 2009

International Society on Thrombosis and Haemostasis

CF is essential or secondary, associated with collagen vascular diseases among others and mainly thrombotic disorders. CF is often asymptomatic; but spontaneous bleeding may occur. CF was suspected in a 61 year old man with compartmental syndrome in the left upper limb and bleeding after surgical decompression, treated with FFP, red-cells and Vit.K. He had RA and no personal or familiar history of bleeding. Considering therapeutic implications, we tried to demonstrate the presence of CFg. Laboratory (day 60): Normal hemogram and liver profile; ESR 45mm; (-) RPC, RF (latex), HCV and cryoglobulins; polyclonal hypergammaglobulinemia and (+) circulant immunocomplexes (0.25UDO). Abnormal PT (45%), APTT (48sec), TT (35/19sec) and fibrinogen (Fg) (Clauss’ method) 56mg/dL, all corrected with normal plasma (N), abnormal ELT (55min), FDP 40µg/mL and DD 1750ng/mL. After 2h at 37ºC a precipitate was observed; being more evident after 72h at 4ºC. Fg in the supernatant lowered according to temperature (25ºC:55; 15ºC:41; 4ºC:27%) and incubation time (1h:27; 2h:18; 24h:15; 72h:10%). Plasma protein electrophoresis (E) showed a very low band with Fg’s mobility. Precipitate’s E showed bands with Fg’s and gammaglobulins’ mobility. Familiar studies were normal. Mixtures(1:3) of N with propositus’ (P) supernatant or serum were incubated at 4ºC for 72h. CFg was observed in all mixtures and low Fg levels in the supernatants. CFg formation was followed through the absorbance at 490nm; results were expressed as a ratio (R=P/N). All R were >1; increasing values according to incubation time (0; 15min; and 1; 2; 24; 48 and 72h) were observed for supernatant (1.41-2.30) and serum (1.46-2.15); similar to P’ plasma sample behaviour (1.72-2.95). Conclusion: A singular form of secondary CF (severe disease and major bleeding symptoms) is described. Identification of the substance responsible for the appearance of CFg is still pending.