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[抗磷脂综合征。肾上腺双侧出血的新病因。4例]

[Antiphospholipid syndrome. A new cause of bilateral hemorrhage of the adrenal glands. 4 cases].

作者信息

Lê T H, Wechsler B, Piette J C, Doumith R, Bellin M F, Papo T, Frenkiel N, Godeau P

机构信息

Service de Médecine interne, Groupe hospitalier Pitié-Salpêtrière, Paris.

出版信息

Presse Med. 1993 Feb 20;22(6):249-54.

PMID:8511142
Abstract

Four cases of adrenal insufficiency due to bilateral adrenal haemorrhage in patients with antiphospholipid syndrome are reported. The 1st patient had repeated episodes of thrombosis on a background of altered general condition; he was examined by computed tomography (CT) which showed enlarged and presumably tumoral adrenal glands; adrenal insufficiency was present and improved under hormone replacement therapy; the thrombotic episodes were attributed to the antiphospholipid antibodies; after a 5-year follow-up the antiphospholipid syndrome remained alone, and further examinations showed progressive adrenal atrophy. The 2nd patient had systemic lupus erythematosus with thrombocytopenia; because of abdominal pain CT was performed, showing bilateral adrenal enlargement; treatment with intravenous pulses of cyclophosphamide and high-dose immunoglobulins combined with corticosteroids failed, and splenectomy was performed disclosing an old adrenal haematoma which was evacuated. The 3rd patient had bilateral and asymmetrical adrenal hypertrophy at CT; subsequently, systemic lupus erythematosus was diagnosed with anti-prothrombinase and anticardiolipin accounting for the initial findings; follow-up examinations showed the formation of pseudocysts in the adrenals; following myocardial infarction the patient died of cerebral haemorrhage, and autopsy confirmed the presence of old, bilateral adrenal haematomas. The 4th patient had recurrent vein thrombosis associated with distal ischaemia, which prompted CT in search of a neoplasia; this examination revealed 2 large adrenal haematomas while anticardiolipin antibodies were found. In patients with antiphospholipid syndrome any functional or morphological abnormality of the adrenals should prompt a search for bilateral adrenal haemorrhage. Conversely, in all cases of adrenal insufficiency a search for antiphospholipid antibodies should be part of all aetiological investigations, and this search should be carried out prior to withdrawing corticosteroids in cases of systemic lupus erythematosus with antiphospholipid antibodies.

摘要

报告了4例抗磷脂综合征患者因双侧肾上腺出血导致肾上腺功能不全的病例。第1例患者在全身状况改变的背景下反复出现血栓形成;他接受了计算机断层扫描(CT)检查,结果显示肾上腺增大,推测为肿瘤性;存在肾上腺功能不全,在激素替代治疗下有所改善;血栓形成发作归因于抗磷脂抗体;经过5年随访,仅遗留抗磷脂综合征,进一步检查显示肾上腺进行性萎缩。第2例患者患有系统性红斑狼疮伴血小板减少症;因腹痛进行了CT检查,显示双侧肾上腺肿大;静脉注射环磷酰胺和大剂量免疫球蛋白联合皮质类固醇治疗无效,遂行脾切除术,发现一个陈旧性肾上腺血肿并予以清除。第3例患者CT显示双侧不对称肾上腺肥大;随后,诊断为系统性红斑狼疮,抗凝血酶原酶和抗心磷脂抗体解释了最初的检查结果;随访检查显示肾上腺形成假性囊肿;心肌梗死后,患者死于脑出血,尸检证实存在陈旧性双侧肾上腺血肿。第4例患者反复出现静脉血栓形成并伴有远端缺血,这促使进行CT检查以寻找肿瘤;该检查发现2个大的肾上腺血肿,同时发现了抗心磷脂抗体。在抗磷脂综合征患者中,肾上腺的任何功能或形态异常都应促使寻找双侧肾上腺出血。相反,在所有肾上腺功能不全的病例中,寻找抗磷脂抗体应作为所有病因学调查的一部分,并且在患有抗磷脂抗体的系统性红斑狼疮病例中,应在停用皮质类固醇之前进行此项检查。

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