Bleeding and thrombosis in a patient with secondary antiphospholipid syndrome.
Antiphospholipid antibodies have been associated with occurrence of arterial and venous thrombotic events and fetal loss, which together constitute the antiphospholipid syndrome (APS). However, bleeding is rare in this syndrome. We report a case of systemic lupus erythematosus (SLE) with APS complicated simultaneously by thrombotic and hemorrhagic events. A 34-year-old woman was a known case of diffuse proliferative lupus nephritis associated with APS, on treatment with corticosteroids, cyclophosphamide and anticoagulants. She presented in February 2004 with severe anemia, menorrhagia, gingival bleeding and acute loss of vision in the left eye. Investigations revealed a hematoma in the psoas muscle with thrombosis of the inferior vena cava and occlusion of the retinal vein. Blood tests revealed a strongly positive lupus anticoagulant, factor XI deficiency (35%) and decrease of free protein S (44%). Factor XI inhibitor, anti-prothrombin, and anti-protein S antibodies were absent. The patient was treated with corticosteroids and six pulses of cyclophosphamide, which resulted in a rapid disappearance of bleeding, reduction of hematoma and normalization of hematological abnormalities. She was maintained on corticosteroids, azathioprine and anticoagulant agents were introduced. After a follow-up of 28 months, there was no recurrence of bleeding, the thrombosis had resolved, and there was a decrease in the levels of circulating anticoagulant as well as anticardiolipin antibodies.
Kaaroud
Hayet
H
Department of Nephrology, Charles Nicolle Hospital, Tunis, Tunisia. hayet.kaarout@rns.tn
Beji
Soumaya
S
Guermazi
Sami
S
Moussa
Fatma Ben
FB
Hamida
Fethi Ben
FB
Ezzine
Sami
S
Abdallah
Taieb Ben
TB
El Younsi
Fethi
F
Kheder
Adel
A
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications*; D001379:Azathioprine / Q000627:therapeutic use; D005260:Female; D006470:Hemorrhage / Q000150:complications* / Q000188:drug therapy*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D013927:Thrombosis / Q000150:complications* / Q000188:drug therapy*; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000925:Anticoagulants; D001379:Azathioprine