Lara Jose Perez, Santana Yaneidy, Gaddam Maneesh, Ali Asghar, Malik Sandeep, Khaja Misbahuddin
Department of Medicine, Bronx Care Health System, Affiliated with Icahn School of Medicine at Mount Sinai, 1650 Grand Concourse, Bronx, NY, 10457, USA.
Division of Pulmonary and Critical Care Medicine, Bronx Care Health System, Affiliated with Icahn School of Medicine at Mount Sinai, 1650 Grand Concourse, Bronx, NY, 10457, USA.
J Med Case Rep. 2019 Jun 23;13(1):190. doi: 10.1186/s13256-019-2097-5.
Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are two forms of thrombotic microangiopathies. They are characterized by severe thrombocytopenia, microangiopathic hemolysis, and thrombosis, leading to a systemic inflammatory response and organ failure. Plasmapheresis is used to treat thrombotic microangiopathies. A different entity known as atypical hemolytic uremic syndrome has garnered more clinical recognition because reported cases have described that it does not respond to standard plasmapheresis. Diclofenac potassium is a non-steroidal anti-inflammatory drug that is used to treat pain.
A 35-year-old Hispanic man presented to our emergency department with complaints of generalized malaise, fever, and an evanescent skin rash. During admission, he reported the use of diclofenac potassium for back pain on a daily basis for 1 week. He was noted to have peripheral eosinophilia, so he was admitted for suspected drug reaction involving eosinophilia and systemic symptoms. His initial laboratory work-up showed microangiopathic hemolytic anemia and thrombocytopenia. He also experienced a seizure, encephalopathy, and had a PLASMIC score of 7, thus raising concerns for thrombotic thrombocytopenic purpura. He underwent emergent plasmapheresis, which improved his clinical condition. The diagnosis was confirmed by assessing the levels of disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13, which was less than 3%. In addition, his skin biopsy was positive for patchy complement deposition, demonstrating complement dysregulation.
Thrombotic thrombocytopenic purpura is a rare condition that can be acquired. Our case is rare because it represents the first report of diclofenac potassium-induced thrombotic thrombocytopenic purpura with subjacent complement activation and dysregulation. Early recognition and aggressive management led to a favorable outcome.
血栓性血小板减少性紫癜和溶血性尿毒症综合征是血栓性微血管病的两种形式。它们的特征是严重血小板减少、微血管病性溶血和血栓形成，导致全身炎症反应和器官衰竭。血浆置换用于治疗血栓性微血管病。一种称为非典型溶血性尿毒症综合征的不同实体已获得更多临床认可，因为报告的病例表明它对标准血浆置换无反应。双氯芬酸钾是一种用于治疗疼痛的非甾体抗炎药。
一名35岁的西班牙裔男性因全身不适、发热和一过性皮疹就诊于我们的急诊科。入院期间，他报告每天服用双氯芬酸钾治疗背痛，持续1周。他被发现有外周嗜酸性粒细胞增多，因此因疑似药物反应伴嗜酸性粒细胞增多和全身症状而入院。他的初始实验室检查显示微血管病性溶血性贫血和血小板减少。他还经历了癫痫发作、脑病，血浆置换积分（PLASMIC score）为7分，因此引发了对血栓性血小板减少性紫癜的担忧。他接受了紧急血浆置换，临床状况得到改善。通过评估含血小板反应蛋白基序的解聚素和金属蛋白酶13（ADAMTS13）水平确诊，该水平低于3%。此外，他的皮肤活检显示补体呈斑片状沉积阳性，表明补体调节异常。
血栓性血小板减少性紫癜是一种可后天获得的罕见疾病。我们的病例很罕见，因为它是双氯芬酸钾诱导的血栓性血小板减少性紫癜伴补体激活和调节异常的首例报告。早期识别和积极治疗带来了良好的结果。
