Miller Devon D, Krenzer Joseph A, Kenkre Vaishalee P, Rose William Nicholas
University of Wisconsin Hospital and Clinics, 600 Highland Ave, Madison, WI 53792, USA.
Case Rep Med. 2021 Nov 30;2021:6869342. doi: 10.1155/2021/6869342. eCollection 2021.
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy due to an acquired autoantibody to ADAMTS13 that requires a boutique treatment, urgent plasma exchange. Thus, TTP is often termed a "cannot miss" diagnosis.
We report a patient with TTP who had a history of immune thrombocytopenia (ITP), had atypical demographics for TTP, and had also met criteria for primary Sjogren's syndrome. This exceedingly rare combination presented a temptation to dismiss TTP as a diagnosis. . Our case further demonstrates the practical utility of using the PLASMIC score as a tool that can help identify patients with TTP even when the patient has statistically rare characteristics.
血栓性血小板减少性紫癜(TTP)是一种罕见的、危及生命的血栓性微血管病,由针对ADAMTS13的获得性自身抗体引起,需要特殊治疗,即紧急血浆置换。因此,TTP常被称为“不容错过”的诊断。
我们报告一例患有TTP的患者,该患者有免疫性血小板减少症(ITP)病史,TTP的人口统计学特征不典型,且符合原发性干燥综合征的标准。这种极其罕见的组合让人忍不住排除TTP的诊断。我们的病例进一步证明了使用PLASMIC评分作为一种工具的实际效用,即使患者具有统计学上罕见的特征,该工具也有助于识别TTP患者。