Miesbach W, Asherson R A, Cervera R, Shoenfeld Y, Gomez Puerta J, Bucciarelli S, Espinoza G, Font J
Department of Internal Medicine III, University Hospital, Johan Wolfgang Goethe-University, Theodor-Stern-Kai 7, 60590 Frankfurt/Main, Germany.
Autoimmun Rev. 2006 Dec;6(2):94-7. doi: 10.1016/j.autrev.2006.06.012. Epub 2006 Jul 21.
The catastrophic antiphospholipid syndrome is characterised by the rapid chronological development of fulminant thrombotic complications that predominantly affect small vessels. It has been reported as frequently occurring in patients with underlying malignancies. We analysed the web site-based international registry of patients with catastrophic APS. The clinical characteristics of patients with CAPS and an underlying malignancy were evaluated. Of the 262 patients included in the CAPS registry, information on associated malignancies was available in 23 (9%) cases. Haematological malignancies were present in 6 (26%) patients. Four of the patients suffered from lung carcinoma (17%), and two patients (9%) from colon carcinoma. In most of the patients (61%), malignancy was the precipitating factor for CAPS. In 4 patients (17%), however, surgical procedures related to the carcinoma were noted as precipitating factors. In one patient CAPS occurred during allogenic stem cell transplantation after diagnosis of acute lymphoblastic leukemia (ALL). Cerebral manifestations were most common and consisted mainly of cerebral infarcts and encephalopathy. Recovery occurred in 9/23 (39%) patients. Malignancy may be an important risk factor for CAPS. 9% of patients with CAPS presented with an underlying malignancy. In most of these patients, the malignancy and/or surgical procedures were the precipitating factors for CAPS.
灾难性抗磷脂综合征的特征是暴发性血栓形成并发症迅速按时间顺序发展，主要影响小血管。据报道，该综合征在患有潜在恶性肿瘤的患者中经常发生。我们分析了基于网站的灾难性抗磷脂综合征患者国际登记处的数据。对患有灾难性抗磷脂综合征且伴有潜在恶性肿瘤的患者的临床特征进行了评估。在灾难性抗磷脂综合征登记处纳入的262例患者中，有23例（9%）提供了相关恶性肿瘤的信息。血液系统恶性肿瘤患者有6例（26%）。其中4例患者患有肺癌（17%），2例患者（9%）患有结肠癌。在大多数患者（61%）中，恶性肿瘤是灾难性抗磷脂综合征的诱发因素。然而，在4例患者（17%）中，与癌症相关的手术被认为是诱发因素。1例患者在诊断为急性淋巴细胞白血病（ALL）后进行异基因干细胞移植期间发生了灾难性抗磷脂综合征。脑部表现最为常见，主要包括脑梗死和脑病。9/23例（39%）患者病情好转。恶性肿瘤可能是灾难性抗磷脂综合征的一个重要危险因素。9%的灾难性抗磷脂综合征患者伴有潜在恶性肿瘤。在这些患者中的大多数，恶性肿瘤和/或手术是灾难性抗磷脂综合征的诱发因素。
