Sneddon综合征合并脾肿大及淋巴结肿大:1例报告及文献复习

Sneddon's syndrome complicated with splenomegaly and lymphadenectasis: One case report and literature review

目的 :提高临床对Sneddon综合征(Sneddon’s syndrome,SS)的认识。方法 :报道1例合并脾肿大和淋巴结肿大的SS患者的临床诊治经过,结合文献复习,重点探讨SS的临床表现、诊断及可能的发生机制。结果 :1例SS患者以反复缺血性脑血管病发作为首发表现,随后出现皮肤网状青斑,并伴有右耳听力下降、轻度认知功能下降、头痛等症状;抗磷脂抗体阴性,影像学提示脾肿大及淋巴结肿大,皮肤活检未见典型的病理学变化。给予抗血小板及改善循环治疗后,患者症状明显好转。结论 :SS合并脾肿大及淋巴结肿大罕见,其机制不明,可能与某种攻击网状内皮系统的抗体有关,这种抗体不同于抗磷脂抗体(anti-phospholipid antibody,APA),且目前的实验室检查手段无法进行检测。当SS症状不典型或为非特异症状时,易被误诊或漏诊。随着反复的脑卒中发作,患者发生严重神经功能缺损及血管性痴呆的可能性增加。因此,SS的早期识别至关重要,以便尽早做出正确诊断,并给予治疗。

Objective: To improve the understanding of Sneddon's syndrome(SS) in clinical practice.Methods: The clinical diagnosis and treatment of a patient with SS complicated with splenomegaly and lymphadenectasis were reported.The clinical manifestations,diagnosis and possible mechanism of SS were discussed based on literature review.Results: A SS patient firstly manifested recurrent ischemic cerebrovascular accidents followed by livedo racemosa of the skin,accompanying hearing impairment,mild cognition decline,headache and other symptoms.Anti-phospholipid antibody(APA) was negative.Imaging examination showed splenomegaly and lymphadenectasis.Skin biopsy found atypical pathological changes.After treatment with antiplete and circulation-improving drugs,symptoms of the patient were alleviated a lot.Conclusion: SS complicated with splenomegaly and lymphadenectasis is rare.The precise mechanism is unknown,may be related to some antibodies targeting endothelial system,which are different from anti-phospholipid antibodies(APAs) and could not be detected currently.When the symptoms of SS are atypical or unspecific,misdiagnosis or missed diagnosis is likely to happen.With recurrent attacks of stroke,the possibility of severe damage to neurologic function and vascular dementia is increased.Therefore,early recognition of SS to make correct diagnosis and treatment as soon as possible is very important.