Tips and tricks for differential diagnosis in pregnancy-associated TMAs
| . | Gestational age of onset . | Symptoms and signs . | Laboratory . | Effect of delivery . |
|---|---|---|---|---|
| Distinctive features (do help in differentiating) | ||||
| PE-SF | Do not consider if <20 wk or >72 h pp | New-onset hypertension | AST/ALT increase >>LDH increase | Resolution (<48-72 h pp) |
| Peripheral edema | ||||
| HELLP | Do not consider if <20 wk or >72 h pp | Predominant splanchnic involvement, jaundice | AST/ALT increase >>LDH increase | Resolution (<48-72 h pp) |
| Visual disturbances | ||||
| TTP | Strongly consider in late third trimester or >72 pp | Severe neurological impairment | PLT <30 000/μL | Possible worsening >48-72 h pp |
| Almost the only TMA in the first trimester | LDH increase, >>AST/ALT increase | |||
| Creatinine usually <1.1 mg/dL | ||||
| cHUS | Strongly consider in late third trimester or >72 pp | Hypertension | Creatinine usually >2 mg/dL | Possible worsening >48-72 h pp |
| Rare in the first half of pregnancy | LDH ≥8× ULN (>>AST/ALT increase) | |||
| Acute systemic vasculitis | All trimesters and postpartum | Hypertension | MAHA rare | Possible worsening >48-72 h pp |
| Acute nephritis | Creatinine usually >2 mg/dL | |||
| Small- and large-vessel thrombosis | aPL/ANA/ANCA positivity | |||
| aPTT may be increased | ||||
| Acute DIC | All trimesters and postpartum | Critical settings | MAHA rare | Possible worsening >48-72 h pp |
| Severe bleeding | PT, aPTT, D-dimer increase | |||
| Low fibrinogen | ||||
| Common features (do not help in differentiating) | ||||
| All the above TMAs | ≥20 wk to <48 h pp | Abdominal disturbances such as nausea, vomiting, abdominal pain | PLT <100 000/μL | Possible worsening <48-72 h pp |
| MAHA, Coombs negative | ||||
| Neurological disturbances such as headache, mild confusion | Creatinine >ULN <2 mg/dL | |||
| LDH > ULN <600 U/L | ||||
| AST/ALT >ULN |
| . | Gestational age of onset . | Symptoms and signs . | Laboratory . | Effect of delivery . |
|---|---|---|---|---|
| Distinctive features (do help in differentiating) | ||||
| PE-SF | Do not consider if <20 wk or >72 h pp | New-onset hypertension | AST/ALT increase >>LDH increase | Resolution (<48-72 h pp) |
| Peripheral edema | ||||
| HELLP | Do not consider if <20 wk or >72 h pp | Predominant splanchnic involvement, jaundice | AST/ALT increase >>LDH increase | Resolution (<48-72 h pp) |
| Visual disturbances | ||||
| TTP | Strongly consider in late third trimester or >72 pp | Severe neurological impairment | PLT <30 000/μL | Possible worsening >48-72 h pp |
| Almost the only TMA in the first trimester | LDH increase, >>AST/ALT increase | |||
| Creatinine usually <1.1 mg/dL | ||||
| cHUS | Strongly consider in late third trimester or >72 pp | Hypertension | Creatinine usually >2 mg/dL | Possible worsening >48-72 h pp |
| Rare in the first half of pregnancy | LDH ≥8× ULN (>>AST/ALT increase) | |||
| Acute systemic vasculitis | All trimesters and postpartum | Hypertension | MAHA rare | Possible worsening >48-72 h pp |
| Acute nephritis | Creatinine usually >2 mg/dL | |||
| Small- and large-vessel thrombosis | aPL/ANA/ANCA positivity | |||
| aPTT may be increased | ||||
| Acute DIC | All trimesters and postpartum | Critical settings | MAHA rare | Possible worsening >48-72 h pp |
| Severe bleeding | PT, aPTT, D-dimer increase | |||
| Low fibrinogen | ||||
| Common features (do not help in differentiating) | ||||
| All the above TMAs | ≥20 wk to <48 h pp | Abdominal disturbances such as nausea, vomiting, abdominal pain | PLT <100 000/μL | Possible worsening <48-72 h pp |
| MAHA, Coombs negative | ||||
| Neurological disturbances such as headache, mild confusion | Creatinine >ULN <2 mg/dL | |||
| LDH > ULN <600 U/L | ||||
| AST/ALT >ULN |
According to the American College of Obstetricians and Gynecologists, PE-SF is currently diagnosed when new-onset hypertension (≥160 mm Hg systolic or ≥110 mm Hg diastolic) occurs ≥20 wk of pregnancy, in conjunction with ≥1 severe feature (creatinine >1.1 mg/dL or 2× baseline; cerebral or visual disturbances; pulmonary edema; liver enzymes ≥2× ULN; severe or persistent abdominal pain in epigastric or right upper quadrant; platelets <100 000/μL), regardless of urine protein assessment. There is no consensus among the American College of Obstetricians and Gynecologists regarding the definition for HELLP.
pp, postpartum.