Subacute thrombophelebitis on deep and superficial branches of femoral and poplyteal veins in sickle cell anemia with protein C-protein S deficiency: A case report

The main clinical manifestation of sickle cell disease is vessel obstruction. Activation of both blood coagulation system and platelets in sickle cell disease and also protein C-protein S deficiency increase risk of thrombosis in these patients. We present a case of 16 years-old girl with homozygous sickle cell anemia. She was referred with generalized and extremity pain to Shafa hospital clinic. Gradually during two months her pain became worse and she suffered from severe right leg edema. Color doppler sonography revealed a sub-acute thrombophelebtis on right leg with deep and superficial branches of femoral and poplyteal veins with severe decreasing blood flow. She was put on intravenous heparin and maintenance oral warfarin. After three months of treatment with warfarin, obstruction was completely resolved and treatment was continued for further five months. During the course of treatment, protein C-protein S were less than normal range. This may be one of the risk factor to increasing thrombosis in our patient. Protien C-protien S were measured in patient's centrifuged serum by full automatic coagulometer ACL 8000 and specific kits.In sickle cell diseased-patient who present with extremity pain and edema, due to vein thrombosis, measurement of protein C-protein S levels and other diagnostic measures, in addition to other medical interventions, appropriate anticoagulant therapy with IV heparin and oral warfarin are needed.