Department of Pathology, Saint Louis University School of Medicine, Saint Louis, Missouri.
J Clin Apher. 2020 Jan;35(1):62-65. doi: 10.1002/jca.21751. Epub 2019 Oct 25.
Thrombotic microangiopathy (TMA) associated with diabetic ketoacidosis (DKA) is a rare complication reported in the pediatric setting. We report a case of an adult patient with new-onset DM, DKA, and TMA who was treated successfully with therapeutic plasma exchange (TPE). The patient underwent five procedures and experienced quick recovery in her platelet count and a near-normalization of her LDH. Within 3 days, ADAMTS13 activity was reported at 40.7% (>66.8%). After a protracted hospital course, mostly focused on treating the patient's bilateral hemorrhagic chemosis, the patient was discharged on hospital day 30. TMA is associated with a spectrum of diseases such as TTP and sepsis but, to our knowledge, it has not been reported in the setting of DKA in an adult patient. Evidence supports that metabolic alterations associated with DKA and its treatment disrupt basal hemostatic mechanisms and promote a thrombotic state. Although ADAMTS13 activity was only moderately decreased, our patient responded rapidly to TPE, with a striking increase and stabilization of her PLT count that was durable beyond discharge. As reported recently, patients who have TMA with ADAMTS13 activity levels >10% have a range of diagnoses, presentations, and outcomes. Although the underlying microangiopathic process is incompletely understood, these patients may respond well to TPE, as was seen in this case.
糖尿病酮症酸中毒（DKA）相关血栓性微血管病（TMA）是儿科罕见的并发症。我们报告了一例成年患者新诊断为糖尿病、DKA 和 TMA，经治疗性血浆置换（TPE）成功治疗。该患者接受了五次治疗，血小板计数迅速恢复，乳酸脱氢酶（LDH）接近正常。3 天内，ADAMTS13 活性报告为 40.7%（>66.8%）。经过漫长的住院治疗，主要集中在治疗患者双侧出血性球结膜下出血，患者在住院第 30 天出院。TMA 与 TTP 和败血症等一系列疾病有关，但据我们所知，在成年 DKA 患者中尚未有报道。有证据表明，DKA 及其治疗相关的代谢改变会破坏基础止血机制并促进血栓形成状态。尽管 ADAMTS13 活性仅中度降低，但我们的患者对 TPE 反应迅速，血小板计数显著增加并稳定，出院后仍持久。最近有报道称，ADAMTS13 活性水平>10%的 TMA 患者有一系列不同的诊断、表现和结果。尽管微血管病变的潜在机制尚不完全清楚，但这些患者可能对 TPE 反应良好，正如本例所见。
