Abstract
Acquired haemophilia or factor VIII (FVIII) deficiency, caused by FVIII inhibitor antibodies, is a very rare condition that commonly results in severe haemorrhagic complications. We report a case of acquired haemophilia presenting with multiple bluish patches affecting face, neck, upper & lower limbs, history of gum bleeding and left knee haemarthrosis. The patient was found to have acquired FVIII inhibitor and lupus anticoagulant (LAC). The simultaneous presence of LAC and FVIII inhibitor is exceedingly rare. The differentiation between these two conditions is crucial, because both result in a prolongation of the activated partial thromboplastin time test, which does not correct when mixed with the plasma of a normal control; however, the clinical manifestations range from thrombosis in the presence of LAC to massive haemorrhage with FVIII inhibitors.
References
Gawryl MS, Hoyer LW (1982) Inactivation of factor VIII coagulant activity by two different types of human antibodies. Blood 60:1103–1109
Saxena R, Mishra DK, Kashyap R, Choudhry VP, Mahapatra M, Bhargava M (2000) Acquired haemophilia––a study of ten cases. Haemophilia 6:78–83
Sahud MA (2000) Factor VIII inhibitors. Laboratory diagnosis of inhibitors. Semin Thromb Hemost 26:195–203
Saxena R, Dhot PS, Saraya AK, Singh H, Malhotra OP (1993) Simultaneous occurrence of factor VIIIC inhibitor and lupus anticoagulant. Am J Hematol 42:232–233
Brings HA, Waas JK, McCrae KR, Baele HR, Goldstone J (2002) Successful management of life-threatening hemorrhage in a patient with synchronous lupus anticoagulant and factor VIII inhibitor. J Vasc Surg 36:853–855
Jennings I, Kitchen S, Woods TA et al (1997) Potentially clinically important inaccuracies in testing for the lupus anticoagulant:an analysis of results from three surveys of the UK National External Quality Assessment Scheme (NEQAS) for blood coagulation. Thromb Haemost 77:934–937
Sahud MA, Pratt KP, Zhukov O, Qu K (2007) Thompson ar. ELISA system for detection of immune responses to FVIII: a study of 246 samples and correlation with the Bethesda assay. Haemophilia 13:317–322
Sallah S (1997) Inhibitors to clotting factors. Ann Hematol 75:1–7
Green D, Lechner K (1981) A survey of 215 non-hemophilic patients with inhibitors to factor VIII. Thromb Haemost 45:200–203
Sallah S, Wan JY (2001) Inhibitors against factor VIII in patients with cancer. Analysis of 41 patients. Cancer 91:1067–1074
Kasper CK, Pool JG (1975) Letter: measurement of mild factor VIII inhibitors in Bethesda units. Thromb Diath Haemorrh 34:875–876
Schulman S (2000) Continuous infusion of recombinant factor VIIa in haemophilic patients with inhibitors: safety, monitoring, and cost effectiveness. Semin Thromb Haemost 26(4):421–424
Roberts HR, Monroe DM, White GC (2004) The use of recombinant factor VIIa in the treatment of bleeding disorders. Blood 104:3858–3864. doi:10.1182/blood-2004-06-2223.PMid:15328151
Franchini M, Tagliaferri A, Mannucci PM (2007) The management of haemophilia in elderly patients. Clin Interv Aging 2:361–368
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Gupta, D., Chatterjee, T., Sharma, A. et al. Rare Case of Acquired Haemophilia and Lupus Anticoagulant. Indian J Hematol Blood Transfus 30, 197–200 (2014). https://doi.org/10.1007/s12288-012-0204-5
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12288-012-0204-5