Clinical and biologic characteristics of 11 patients with HELLP syndrome
| Patient . | Age, y*† . | GA, w . | BP, mmHg . | Plts, G . | ALAT, U/L . | LDH, U/L . | SCr, μM . | Outcome . | Peculiar features . |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 24 (0) | 34 | 160/110 | 39 | 85 | 890 | 220 | ESRD | P1 with a CFH mutation had a history of unexplained chronic renal failure (CrCl: 0.7 mL/sec) when she developed HELLP syndrome |
| Subsequently, she experienced 2 early (1 mo) losses of renal transplants due, on at least one occasion, to widespread intrarenal thrombosis, a feature highly suggestive of TMA | |||||||||
| 2 | 21 (0) | 28 | 180/120 | 78 | 188 | 855 | 93 | CR | Uneventful second pregnancy, while on aspirin |
| 3 | 28 (3) | 38 | 230/110 | 37 | 218 | 850 | 384 | CR | |
| 4 | 41 (2) | 38 | 150/95 | 130 | 365 | 750 | 247 | CRF | Liver hematoma |
| 5 | 31 (0) | 27 | 170/100 | <100 | 85 | 830 | 115 | CRF | Atypical HUS at the age of 28 years requiring HD and PE |
| Mildly decreased plasma C3 level (75 and 82 mg/L; normal 85-193 mg/L) on 2 consecutive assays. | |||||||||
| Paternal grandfather with unexplained ESRD | |||||||||
| 6 | 24 (0) | 40 | 160/95 | 31 | 328 | 710 | NA | CR | Mild thrombocytopenia (110 × 109/L) and undetectable haptoglobin were noted during 2 subsequent pregnancies (while on aspirin) |
| 7 | 32 (0) | 24 | 160/100 | 30 | 92 | 945 | NA | CR | Liver hematoma |
| 8 | 30 (2) | 28 | 230/120 | 35 | 71 | 3400 | HD | CR | Underwent PE |
| 9 | 36 (1) | 37 | 180/95 | 16 | 580 | 6500 | 500 | CRF | Underwent PE |
| 10 | 33 (0) | 34 | 180/110 | 100 | 304 | 863 | 107 | CR | |
| 11 | 31 (3) | 31 | 180/110 | 26 | 875 | 1000 | HD | CR |
| Patient . | Age, y*† . | GA, w . | BP, mmHg . | Plts, G . | ALAT, U/L . | LDH, U/L . | SCr, μM . | Outcome . | Peculiar features . |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 24 (0) | 34 | 160/110 | 39 | 85 | 890 | 220 | ESRD | P1 with a CFH mutation had a history of unexplained chronic renal failure (CrCl: 0.7 mL/sec) when she developed HELLP syndrome |
| Subsequently, she experienced 2 early (1 mo) losses of renal transplants due, on at least one occasion, to widespread intrarenal thrombosis, a feature highly suggestive of TMA | |||||||||
| 2 | 21 (0) | 28 | 180/120 | 78 | 188 | 855 | 93 | CR | Uneventful second pregnancy, while on aspirin |
| 3 | 28 (3) | 38 | 230/110 | 37 | 218 | 850 | 384 | CR | |
| 4 | 41 (2) | 38 | 150/95 | 130 | 365 | 750 | 247 | CRF | Liver hematoma |
| 5 | 31 (0) | 27 | 170/100 | <100 | 85 | 830 | 115 | CRF | Atypical HUS at the age of 28 years requiring HD and PE |
| Mildly decreased plasma C3 level (75 and 82 mg/L; normal 85-193 mg/L) on 2 consecutive assays. | |||||||||
| Paternal grandfather with unexplained ESRD | |||||||||
| 6 | 24 (0) | 40 | 160/95 | 31 | 328 | 710 | NA | CR | Mild thrombocytopenia (110 × 109/L) and undetectable haptoglobin were noted during 2 subsequent pregnancies (while on aspirin) |
| 7 | 32 (0) | 24 | 160/100 | 30 | 92 | 945 | NA | CR | Liver hematoma |
| 8 | 30 (2) | 28 | 230/120 | 35 | 71 | 3400 | HD | CR | Underwent PE |
| 9 | 36 (1) | 37 | 180/95 | 16 | 580 | 6500 | 500 | CRF | Underwent PE |
| 10 | 33 (0) | 34 | 180/110 | 100 | 304 | 863 | 107 | CR | |
| 11 | 31 (3) | 31 | 180/110 | 26 | 875 | 1000 | HD | CR |
Patients with identified mutations in CFH, CFI, and MCP genes are shown in bold; patients with features of an abnormal control of the complement alternative pathway and no identified mutations in CFH, CFI, and MCP genes are shown in italics.
y indicates year; GA, gestational age in weeks; BP, blood pressure; Plts, platelet count; ALAT, alanine aminotransferase; LDH, lactate dehydrogenase; SCr, serum creatinine; ESRD, end-stage renal disease; CrCl, creatinine clearance estimated using the MDRD formula; CR, complete remission; CRF, chronic renal failure; HUS, hemolytic uremic syndrome; HD, hemodialysis; PE, plasma exchange; and NA, not available.
Age at the onset of the HELLP syndrome.
The number of previous pregnancies is indicated in parentheses. Death of the newborn occurred in cases 2, 5, and 7.