Sandouk Zahrae, Alirhayim Zaid, Khoulani Dania, Hassan Syed
Department of Internal Medicine, Henry Ford Hospital, Detroit, Michigan, USA.
BMJ Case Rep. 2012 Nov 14;2012:bcr2012007558. doi: 10.1136/bcr-2012-007558.
A middle-aged man diagnosed with a drug reaction with eosinophilia and systemic symptom (DRESS) syndrome, secondary to phenytoin use, subsequently developed thrombotic thrombocytopaenic purpura. The patient improved with steroids and plasmapheresis. Their diagnosis can be challenging, and an early recognition and treatment are critical owing to their high mortality rates. Both diseases are thought to be of an autoimmune origin, and a potential relationship between them led to the consideration of the DRESS syndrome as an aetiology for thrombotic thrombocytopaenic purpura in this case. We concluded that two possibilities exist: some type of antibody developed during the clinical presentation of DRESS syndrome and subsequently resulted in an inhibition of a disintegrin and metalloproteinase with a thrombospondin type-1 motif, member 13 (ADAMTS13) leading to thrombotic thrombocytopaenic purpura, or perhaps this patient's autoimmune predisposition to thrombotic thrombocytopaenic purpura contributed to the drug reaction.
一名中年男子因使用苯妥英继发药物超敏反应伴嗜酸性粒细胞增多和全身症状(DRESS)综合征,随后发展为血栓性血小板减少性紫癜。患者经类固醇和血浆置换治疗后病情好转。它们的诊断可能具有挑战性,由于其高死亡率,早期识别和治疗至关重要。这两种疾病都被认为是自身免疫性起源,它们之间的潜在关系导致在本病例中将DRESS综合征视为血栓性血小板减少性紫癜的病因。我们得出结论,存在两种可能性:在DRESS综合征临床表现期间产生了某种类型的抗体,随后导致具有血小板反应蛋白1型基序的解整合素和金属蛋白酶13(ADAMTS13)受到抑制,从而导致血栓性血小板减少性紫癜;或者也许该患者对血栓性血小板减少性紫癜的自身免疫易感性导致了药物反应。