Coexistence of lupus anticoagulant and acquired haemophilia in a patient with monoclonal gammopathy of unknown significance

Acquired haemophilia or factor VIII (FVIII) deficiency, caused by FVIII inhibitorantibodies, is a very rare condition that commonly results in severe haemorrhagic complications. We report a case of acquired haemophilia presenting with a left calf nontraumatichaematoma. The patient was also found to have lupus anticoagulant (LAC). The simultaneous presence of LAC and FVIII inhibitor is exceedingly rare. The differentiationbetween these two conditions is crucial, because both result in a prolongationof the activated partial thromboplastintime 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.

[1]  John Waas,et al.  Successful management of life-threatening hemorrhage in a patient with synchronous lupus anticoagulant and factor VIII inhibitor. , 2002, Journal of Vascular Surgery.

[2]  Kashyap,et al.  Acquired Haemophilia – a study of ten cases , 2000, Haemophilia : the official journal of the World Federation of Hemophilia.

[3]  M. Sahud.  Laboratory Diagnosis of Inhibitors , 2000 .

[4]  S. Schulman.  Continuous Infusion of Recombinant Factor VIIa in Hemophilic Patients with Inhibitors: Safety, Monitoring, and Cost Effectiveness , 2000, Seminars in thrombosis and hemostasis.

[5]  M. Sahud.  Factor VIII inhibitors. Laboratory diagnosis of inhibitors. , 2000, Seminars in thrombosis and hemostasis.

[6]  S. Sallah.  Inhibitors to clotting factors , 1997, Annals of Hematology.

[7]  I. Scharrer,et al.  Criteria for the Diagnosis of Lupus Anticoagulants: An Update , 1995, Thrombosis and Haemostasis.

[8]  R. Saxena,et al.  Simultaneous occurrence of factor VIIIC inhibitor and lupus anticoagulant , 1993, American journal of hematology.

[9]  E. Ascari,et al.  Study of three patients with monoclonal gammopathies and ‘lupus‐like’ anticoagulants , 1989, British journal of haematology.

[10]  K. Lechner,et al.  A Survey of 215 Non-Hemophilic Patients with Inhibitors to Factor VIII , 1981, Thrombosis and Haemostasis.

[11]  S. Shapiro,et al.  Monoclonal immunoglobulin M lambda coagulation inhibitor with phospholipid specificity. Mechanism of a lupus anticoagulant. , 1980, The Journal of clinical investigation.