Catastrophic antiphospholipid syndrome and Kikuchi-Fujimoto disease:: the first case reported
Catastrophic antiphospholipid syndrome and Kikuchi-Fujimoto disease:: the first case reported
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DOI：
10.1191/0961203305lu2232cr
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发表时间：
2005-01-01
期刊：
影响因子：
2.6
通讯作者：
San Jaun, JA
中科院分区：
文献类型：
--
作者：
de Larrañaga, GF;Remondino, GI;San Jaun, JA
报告1例菊内藤本病(KFD)和灾难性抗磷脂综合征(CAPS)的诊断病例。他表现为长期发热、淋巴结病、关节痛、体重减轻、肝脾肿大和巨细胞病毒IgM阳性。当他接受抗结核药物的经验性治疗时，他突然出现了全身炎症反应综合征、多器官衰竭和远端坏死。怀疑有严重脓毒症时，给予抗生素、皮质激素和重组人活化蛋白C(Xgris(R))治疗。详尽的实验室搜索结果是否定的。淋巴结组织病理学检查最早发现恶性淋巴瘤，但后来确诊为KFD。一个月后，实验室检测显示存在抗磷脂抗体(APL)。病人在住院两个月后出院。本病例表现为一种由明确的上限引起的KFD。巨细胞病毒可能参与了CAPS和KFD的发生发展。考虑到病情的严重性，我们认为XIGRIS(R)显著改善了与CAPS相关的凝血状态的改变。
The case of a man with diagnosis of Kikuchi-Fujimoto disease (KFD) and catastrophic antiphospholipid syndrome (CAPS) is reported. He presented prolonged fever, lymphadenopathies, arthralgia, weight loss, hepatosplenomegaly and positive IgM for cytomegalovirus. While he was empirically treated with tuberculostatic drugs, he suddenly developed systemic inflammatory response syndrome, multiple organ failure and distal necrosis. On suspicion of severe sepsis, antibiotics, corticoids and recombinant human activated protein C (XIGRIS (R)) were administrated. Exhaustive laboratory searching was negative. Histopathologic examinations of lymph node first disclosed malignant lymphoma but later KFD was confirmed. One month later, laboratory tests showed the presence of antiphospholipid antibodies (aPL). The patient was discharged after two months of hospitalization. This case exhibits a KFD complicated by definite CAPS. Cytomegalovirus could be involved in the development of both CAPS and KFD. Because of the severity of the case, we believe that XIGRIS (R) noticeable improved the altered coagulation profile associated with CAPS.