Pelletier S, Landi B, Piette J C, Ekert P, Coutellier A, Desmoulins C, Fadlallah J P, Herson S, Valla D
Service de Médecine Interne I, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
J Hepatol. 1994 Jul;21(1):76-80. doi: 10.1016/s0168-8278(94)80140-1.
In a prospective study of 22 patients with non-tumorous Budd-Chiari syndrome, four were found to have the antiphospholipid syndrome with no other cause of hepatic vein thrombosis. All four patients were young women. The antiphospholipid syndrome was secondary to systemic lupus in one case, to a "lupus-like disease" in another, and apparently primary in the remaining two cases. Two patients died. The other two are in good health on chronic oral anticoagulation. In our experience, the antiphospholipid syndrome is a frequent cause, after myeloproliferative disorders, of non-tumorous Budd-Chiari syndrome. In such patients, long-term anticoagulation may prevent recurrence or extension of thrombosis.
在一项对22例非肿瘤性布加综合征患者的前瞻性研究中，发现4例患有抗磷脂综合征，且无其他肝静脉血栓形成原因。所有4例患者均为年轻女性。抗磷脂综合征在1例中继发于系统性红斑狼疮，在另1例中继发于“狼疮样疾病”，其余2例显然为原发性。2例患者死亡。另外2例在接受慢性口服抗凝治疗后健康状况良好。根据我们的经验，抗磷脂综合征是继骨髓增殖性疾病之后非肿瘤性布加综合征的常见病因。对于此类患者，长期抗凝治疗可能预防血栓形成的复发或扩展。
