Khobragade A K, Chogle A R, Ram R P, Mascarenhas Joanne, Kothari Sweta, Kawadkar Sneha, Deshpande S S, Nair Dipti, Makhija Jhoomar
Department of General Medicine, Jagjivanram Hospital, Western Railway, Mumbai.
J Assoc Physicians India. 2012 Nov;60:59-62.
Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterized by a pentad consisting of thrombocytopenic, microangiopathic hemolytic anemia, renal dysfunction, neurological signs and fever. Coexistence of thrombotic thrombocytopenic purpura and Adult Onset Still's Disease (AOSD) is extremely rare. We report a case of 18 year old girl with AOSD who developed TTP. Neuroimaging of brain demonstrated white matter edema consistent with reversible posterior leukoencephalopathy syndrome (RPLS). Complete recovery occurred with prompt anti-hypertensive treatment and high dose immunoglobulin infusions (IVIg). Plasma exchange is the standard of care and the first line treatment for patient with TTP. We used IVIg alone in our case and this showed a gratifying response. Use of IVIG before considering plasmapharesis is justifiable or not requires randomized control clinical trials. This should determine the optimal therapeutic strategies for TTP.
血栓性血小板减少性紫癜（TTP）是一种多系统疾病，其特征为血小板减少、微血管病性溶血性贫血、肾功能不全、神经体征和发热组成的五联征。血栓性血小板减少性紫癜与成人斯蒂尔病（AOSD）并存极为罕见。我们报告一例18岁患AOSD的女孩并发TTP的病例。脑部神经影像学检查显示白质水肿，符合可逆性后部白质脑病综合征（RPLS）。通过及时的抗高血压治疗和大剂量静脉注射免疫球蛋白（IVIg），患者完全康复。血浆置换是TTP患者的标准治疗方法和一线治疗手段。我们的病例仅使用了IVIg，且显示出令人满意的反应。在考虑进行血浆置换之前使用IVIG是否合理，这需要随机对照临床试验来确定。这将确定TTP的最佳治疗策略。
