Thrombotic Microangiopathy. Focus on Atypical Hemolytic Uremic Syndrome

Research output: Contribution to journalArticle

Abstract

Thrombotic microangiopathies (TMA) such as atypical hemolytic uremic syndrome (aHUS) have evolved from rare, fulminant childhood afflictions to uncommon diseases with acute and chronic phases involving both children and adults. Breakthroughs in complement and coagulation regulation have allowed redefinition of specific entities despite substantial phenotypic mimicry. Reconciliation of phenotypes and delivery of life saving therapies require a multidisciplinary team of experts. The purpose of this review is to describe advances in the molecular pathophysiology of aHUS and to share the 2014 experience of the multidisciplinary Johns Hopkins TMA Registry in applying diagnostic assays, reporting disease associations, and genetic testing.

Original languageEnglish (US)
Pages (from-to)541-559
Number of pages19
JournalHematology/Oncology Clinics of North America
Volume29
Issue number3
DOIs
StatePublished - Jun 1 2015

Fingerprint

Thrombotic Microangiopathies
Genetic Testing
Acute Disease
Registries
Chronic Disease
Phenotype
Atypical Hemolytic Uremic Syndrome
Therapeutics

Keywords

  • Atypical hemolytic uremic syndrome
  • Hemolytic uremic syndrome
  • Thrombotic microangiopathy
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Oncology
  • Hematology

Cite this

Thrombotic Microangiopathy. Focus on Atypical Hemolytic Uremic Syndrome. / Sperati, C John; Moliterno, Alison R.

In: Hematology/Oncology Clinics of North America, Vol. 29, No. 3, 01.06.2015, p. 541-559.

Research output: Contribution to journalArticle

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