A Case of Atypical Thrombotic Microangiopathy.
- Author:
Ji Young OH
1
;
Se Jin PARK
;
Ki Hwan KIM
;
Beom Jin LIM
;
Hyeon Joo JEONG
;
Jung Hye KI
;
Kee Hyuck KIM
;
Jae Il SHIN
Author Information
1. Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine, Korea. shinji@yuhs.ac
- Publication Type:Case Report
- Keywords:
Fever;
Anemia without hemolysis;
Thrombocytopenia;
Atypical thrombotic microangiopathy (TMA);
Renal biopsy
- MeSH:
Acute Kidney Injury;
Adolescent;
Anemia;
Biopsy;
Brain;
Complement System Proteins;
Creatinine;
Diagnosis;
Edema;
Female;
Fever;
Hemolysis;
Hemolytic-Uremic Syndrome;
Hospitalization;
Humans;
Hypertension, Renal;
Leukoencephalopathies;
Magnetic Resonance Imaging;
Nausea;
Proteinuria;
Purpura, Thrombotic Thrombocytopenic;
Resin Cements;
Seizures;
Thrombocytopenia;
Thrombotic Microangiopathies*
- From:Journal of the Korean Society of Pediatric Nephrology
2013;17(2):149-153
- CountryRepublic of Korea
- Language:English
-
Abstract:
We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.
