Korean J Med.  2019 Feb;94(1):83-88. 10.3904/kjm.2019.94.1.83.

Differential Diagnosis and Treatment of Thrombotic Microangiopathy Syndrome

Affiliations
  • 1Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea. alertjun@hanmail.net

Abstract

Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.

Keyword

Thrombotic microangiopathy; Thrombotic thrombocytopenic purpura; Hemolytic uremic syndrome; Plasma exchange; Eculizumab

MeSH Terms

Acute Kidney Injury
Anemia, Hemolytic
Comorbidity
Diagnosis
Diagnosis, Differential*
Disseminated Intravascular Coagulation
Enterohemorrhagic Escherichia coli
Hemolytic-Uremic Syndrome
Humans
Hypertension, Malignant
Mortality
Plasma Exchange
Plasmapheresis
Polymerase Chain Reaction
Purpura, Thrombotic Thrombocytopenic
Thrombocytopenia
Thrombospondins
Thrombotic Microangiopathies*
Thrombospondins
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