Antiphospholipid antibodies in haemophilia patients with severe bleeding tendency: cause, consequence or a consequential cause?
The prevalence, cause and the impact of antiphospholipid antibodies (APAs) on the clinical severity in haemophilia patients is poorly studied. We studied 72 severe seronegative (negative for HIV, HBsAg, HCV) haemophilia patients for the presence of four common APAs. Twenty-six (36.1%) were positive for any one of the APAs studied of which eight were positive only for anticardiolipin antibodies, three for beta2 glycoprotein (beta2GP1), four for prothrombin (PT) and six for anti annexin antibodies. Remaining six patients showed multi-specific antibodies. Further, clinically severe haemophilia patients (n = 37) showed higher prevalence of APAs as compared with the clinically milder group (n = 35) suggesting that these antibodies do not contribute in alleviating the clinical severity in haemophilia patients as has been observed with other inherited thrombophilia markers. The study of in vitro thrombin generation showed a higher endogenous thrombin potential (ETP) i.e. almost normal, in case of beta2GP1-positive patients as compared with patients with other types of APAs. High prevalence of APAs in clinically severe haemophilia patients may be a consequence of continuing tissue damage in the clinically severe group; as in India, clotting factor concentrates cannot be used ad lib because of financial constraints. Higher thrombin-generating potential in case of patients positive for beta2GP1 did not seem to have any impact on the clinical severity of haemophilia patients.
Shetty
S
S
National Institute of Immunohaematology (ICMR), KEM Hospital, Parel, Mumbai, India.
Vora
S
S
Kulkarni
B
B
Mota
L
L
Ghosh
K
K
D017153:Antibodies, Anticardiolipin / Q000276:immunology*; D017152:Antibodies, Antiphospholipid / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D019774:Blood Coagulation Factor Inhibitors / Q000276:immunology*; D006467:Hemophilia A / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007194:India; D008297:Male; D010949:Plasma / Q000276:immunology*; D015995:Prevalence; D012307:Risk Factors; D012720:Severity of Illness Index; D013917:Thrombin / Q000276:immunology*
D017153:Antibodies, Anticardiolipin; D017152:Antibodies, Antiphospholipid; D015415:Biomarkers; D019774:Blood Coagulation Factor Inhibitors; D013917:Thrombin