Thrombotic Microangiopathy. Focus on Atypical Hemolytic Uremic Syndrome

C. John Sperati; Alison R. Moliterno

doi:10.1016/j.hoc.2015.02.002

Thrombotic Microangiopathy. Focus on Atypical Hemolytic Uremic Syndrome

C. John Sperati, Alison R. Moliterno

School of Medicine

Research output: Contribution to journal › Review article › peer-review

19 Scopus citations

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 language	English (US)
Pages (from-to)	541-559
Number of pages	19
Journal	Hematology/Oncology Clinics of North America
Volume	29
Issue number	3
DOIs	https://doi.org/10.1016/j.hoc.2015.02.002
State	Published - Jun 1 2015

Keywords

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

ASJC Scopus subject areas

Hematology
Oncology

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10.1016/j.hoc.2015.02.002

Cite this

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title = "Thrombotic Microangiopathy. Focus on Atypical Hemolytic Uremic Syndrome",

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.",

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KW - Thrombotic thrombocytopenic purpura

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Thrombotic Microangiopathy. Focus on Atypical Hemolytic Uremic Syndrome

Abstract

Keywords

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