Department of Internal Medicine, Rush University Medical Center.
Department of Pediatrics, Rush University Children's Hospital, Chicago, IL.
J Pediatr Hematol Oncol. 2021 Jul 1;43(5):e736-e738. doi: 10.1097/MPH.0000000000001973.
Thrombotic microangiopathies (TMAs) are a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage. It can often be challenging to determine the underlying etiology. Our patient presented with acute pancreatitis and later developed thrombocytopenia and hemolytic anemia, along with acute renal failure. A working diagnosis of an atypical hemolytic uremic syndrome was made; however, he improved clinically and eculizumab was not started. Workup for the atypical hemolytic uremic syndrome was unrevealing. The authors propose that the pancreatitis triggered a secondary TMA, which although rare, has previously been described in the literature. This case illustrates the diagnostic and therapeutic challenges associated with TMAs.
血栓性微血管病(TMA)是一组以微血管病性溶血性贫血、血小板减少和终末器官损伤为特征的疾病。通常很难确定其根本病因。我们的患者表现为急性胰腺炎,随后出现血小板减少、溶血性贫血和急性肾衰竭。临床诊断为非典型溶血尿毒综合征;然而,他的病情有所改善,并未开始使用依库珠单抗。针对非典型溶血尿毒综合征的检查结果无异常。作者提出,胰腺炎引发了继发性 TMA,这种情况虽然罕见,但在文献中已有描述。该病例说明了 TMA 相关的诊断和治疗挑战。