Thrombotic microangiopathy in children

Lilian Monteiro P. Palma, Maria Helena Vaisbich-Guimarães, Meera Sridharan, Cheryl L. Tran, Sanjeev Sethi

Research output: Contribution to journalReview articlepeer-review


The syndrome of thrombotic microangiopathy (TMA) is a clinical-pathological entity characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ involvement. It comprises a spectrum of underlying etiologies that may differ in children and adults. In children, apart from ruling out shigatoxin-associated hemolytic uremic syndrome (HUS) and other infection-associated TMA like Streptococcus pneumoniae-HUS, rare inherited causes including complement-associated HUS, cobalamin defects, and mutations in diacylglycerol kinase epsilon gene must be investigated. TMA should also be considered in the setting of solid organ or hematopoietic stem cell transplantation. In this review, acquired and inherited causes of TMA are described with a focus on particularities of the main causes of TMA in children. A pragmatic approach that may help the clinician tailor evaluation and management is provided. The described approach will allow for early initiation of treatment while waiting for the definitive diagnosis of the underlying TMA.

Original languageEnglish (US)
Pages (from-to)1967-1980
Number of pages14
JournalPediatric Nephrology
Issue number9
StatePublished - Sep 2022


  • Complement
  • DGKe
  • Hemolytic uremic syndrome
  • Hypertension
  • Infection
  • Thrombotic microangiopathy
  • Transplant

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology


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