[Catastrophic antiphospholipid syndrome. Report of two cases].
The antiphospholopid syndrome has been associated with thromboembolic events, thrombocytopenia and fetal loss. Some patients with the antiphospholipid syndrome may develop an acutely catastrophic syndrome characterized by multiple vascular occlusions which often results in death. Most patients dye as a result of a combination of cardiac and pulmonary failure. Although trigger factors are present in a minority, in the majority the condition develops quite suddenly. Precipitating factors include infections and trauma (surgical). Treatment of the condition, once recognized, needs to be heroic. Plasmapheresis seems to be useful in several cases who had not responded to conventional therapy (heparin, steroids, immunosuppressive) directed against immunologically mediated intravascular thrombosis. We report here two cases of fatal catastrophic antiphospholipid syndrome and highlight the need for vigilance in the management of patients with antiphospholipid syndrome.
Ben Hadj Slama
Foued
F
Laboratoire d'immunologie, Faculté de Médecine de Sousse.
Mahjoub
Touhami
T
Fejji
Samir
S
Maaroufi
Amel
A
Trabelsi
Amel
A
Amri
Fethi
F
Laouani
Chadia
C
D000208:Acute Disease; D000328:Adult; D016736:Antiphospholipid Syndrome / Q000473:pathology* / Q000628:therapy; D002648:Child; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007239:Infections / Q000150:complications; D008297:Male; D010956:Plasmapheresis; D011379:Prognosis; D012307:Risk Factors; D013927:Thrombosis / Q000209:etiology*