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. 2021 Apr 26;13(5):306. doi: 10.3390/toxins13050306

Table 3.

Discriminative features between thrombotic microangiopathies: Shiga toxin E. coli-associated hemolytic uremic syndrome (STEC-HUS), atypical hemolytic uremic syndrome (aHUS), thrombotic thrombocytopenic purpura (TTP) and hypertension-related thrombotic microangiopathy (HT-TMA).

STEC-HUS aHUS TTP HT-TMA
Cause
EHEC Deficiency or dysfunction of complement regulatory proteins Deficiency or inhibition of the vWF-cleaving protease ADAMTS13 Malignant hypertension
Clinical Features
Age Young children (<five years) and older (>60 years) adults Children and young adults Middle-aged adults Middle-aged adults
Sex ratio Non-discriminant Non-discriminant Females > males Males > females
Medical history Not specific Not specific History of auto-immune diseases History of hypertension
Blood pressure Normal/moderate hypertension Moderate/severe hypertension Normal Severe hypertension
Neurological symptoms Confusion frequent in adults Rare Frequent, focal deficit Headache
Digestive symptoms Frequent but not systematic at the time of HUS, bloody diarrhea Digestive symptoms frequent, diarrhea becomes rarely bloody Rare Rare
Biological Features
Thrombocytopenia Moderate Moderate Severe (<30 × 109/L) Inconstant to moderate
Acute kidney injury Moderate to severe Severe Absent to severe Severe
Specific Treatment
None Eculizumab Rituximab
Caplacizumab
Blood pressure control

EHEC: enterohemorrhagic Escherichia coli, vWF: von Willebrand factor, ADAMTS13: A disintegrin and metalloprotease with thrombospondin type 1 repeats.