first question | choose another case | Epharnet

J.M. is a 26 - year old, married, Caucasian male, office worker with two children.

Family history:
Father died of spontaneous pulmonary embolism at the age of forty. Mother is healthy. His sister had two years ago one episode of ileofemoral thrombosis in association with the use of oral contraceptives.

Personal history:
No prior serious illnesses. No surgery either. A couple of days ago he played football and injured his right ankle. A plaster cast was applied. After four days he began to feel pain in his right calf and the plaster cast seemed to him to be tight. He consulted his doctor again. The plaster cast was removed and a venous ultrasound revealed ileofemoral thrombosis. He was admitted to hospital. Therapy with low molecular weight heparin ( LMWH ) - Fraxiparin 0,8 ml sc bid according his body weight was started. Next day 10 mg of warfarin qd were added. On the fourth day of therapy his INR was 2,9 and Fraxiparin was withdrawn. On the same day a very severe pain in his right buttock and calf has occurred. He developed erythematous skin lesions, which became black and necrotic in the central area. Haemorrhagic bullae appeared. These lesions were characterized by a clear line of demarcation between the affected areas and their surrounding tissue.

Physical examination:
She had no dyspnea at rest, but she became short of breath while taking a seat. Body temperature was normal, she was normosthenic, blood pressure 100/60, heart rate 100/min, breathing rate 15/min, weight 55 kg. Other physical examination was completely normal, no swelling of legs. The operation wound was healed.

Laboratory results detected low levels of protein C and S. The APC ratio was increased. These results are consistent with the administration of warfarin. Therefore we underwent a laboratory thrombophilia work up with his sister which revealed protein C antigen to be 45%. Activity of protein C (measured by chromogenic assay) was decreased as well at 40%.

Laboratory tests:
Blood cell count : Leu =13.10⁹, Ery = 4.25.10¹², Hb =13.5 g/dl, Htk = 0.42, Plt 350.10⁹, antithrombin III 80%.
Resistance to activated protein C 5.1
protein C 10%
protein S 20%