Table 1.

Clinical and laboratory features of pregnancy-associated microangiopathies

Preeclampsia/HELLPTTPHUSAFLP
Elevated blood pressure +++ ++ (50% of cases) 
Neurological symptoms +/++ (headache) +++ (numbness, weakness, aphasia, mental status) 
Abdominal symptoms + (RUQ pain) ++ (unspecific/diffuse) +++ (unspecific/diffuse) 
Fever − −/+ −/+ − 
Easy bruising − −/+ − − 
Thrombocytopenia +/++ (>50 × 109/L) +++ (<20 × 109/L) + (<100 × 109/L) 
Renal impairment (elevated creatinine; > ∼2 mg/dL) +/++ +/++ +++ ++/+++ 
Hepatic dysfunction and inflammation (AST/ALT) −/+ −/+ +++ (and bilirubin) 
Coagulopathy −/+ − − +++ 
LDH +/+++ +/++ +++ 
Microangiopathic hemolytic anemia +/+++ +/++ 
Hypoglycemia − − − 
ADAMTS13 activity Normal <10%* >20%-30% >30% 
Preeclampsia/HELLPTTPHUSAFLP
Elevated blood pressure +++ ++ (50% of cases) 
Neurological symptoms +/++ (headache) +++ (numbness, weakness, aphasia, mental status) 
Abdominal symptoms + (RUQ pain) ++ (unspecific/diffuse) +++ (unspecific/diffuse) 
Fever − −/+ −/+ − 
Easy bruising − −/+ − − 
Thrombocytopenia +/++ (>50 × 109/L) +++ (<20 × 109/L) + (<100 × 109/L) 
Renal impairment (elevated creatinine; > ∼2 mg/dL) +/++ +/++ +++ ++/+++ 
Hepatic dysfunction and inflammation (AST/ALT) −/+ −/+ +++ (and bilirubin) 
Coagulopathy −/+ − − +++ 
LDH +/+++ +/++ +++ 
Microangiopathic hemolytic anemia +/+++ +/++ 
Hypoglycemia − − − 
ADAMTS13 activity Normal <10%* >20%-30% >30% 

Estimated prevalence of clinical signs and symptoms and laboratory features in women with TMAs during pregnancy. Reference ranges in healthy pregnancy must be taken into consideration.

+, prevalence; −, not usually present; ALT, alanine aminotransferase; AST, aspartate transaminase; LDH, lactate dehydrogenase; RUQ, right upper quadrant.

*

Some investigators require that the ADAMTS13 activity level in plasma be below 10% of normal to make the diagnosis of TTP, whereas others use this solely as providing confirmation of a clinical diagnosis.

ADAMTS13 activity is generally above 30% of normal in patients with a clinical diagnosis of aHUS, but there are no guidelines that exclude this diagnosis based on activity levels per se.

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