Table 1 Clinical features and treatment outcomes of four cases of aHUS
| Â | Case 1 | Case 2 | Case 3 | Case 4 |
|---|---|---|---|---|
Age | 59Â years | 57Â years | 74Â years | 67Â years |
Sex | Female | Female | Male | Female |
Underlying disease | Chronic kidney disease | Hypertension | Hypertension | Hypertension, chronic kidney disease |
Initial symptoms/clinical manifestation | Pulmonary hemorrhage Microangiopathic hemolytic anemia Thrombocytopenia Renal dysfunction | Pulmonary hemorrhage Microangiopathic hemolytic anemia Thrombocytopenia Renal dysfunction | Pulmonary hemorrhage Microangiopathic hemolytic anemia Thrombocytopenia Renal dysfunction | Microangiopathic hemolytic anemia Thrombocytopenia Liver dysfunction Renal dysfunction |
Urine occult blood | ++ | + | +++ | ++ |
WBC (× 109/L) | 12.2 | 10.2 | 14.0 | 8.5 |
Hb (g/dL) | 8.3 | 7.9 | 9.3 | 8.0 |
Plt (× 109/L) | 96 | 17 | 59 | 20 |
LDH (U/L) | 474 | 2651 | 1972 | 2663 |
Cr (mg/dL) | 2.3 | 1.8 | 4.77 | 5.57 |
ADAMTS 13 activity (%) /inhibitor (BU/ml) | 48.8%/– | 65.4%/– | 59.8%/– | 67.2%/– |
Histological findings | The swelling of endothelial cells without intravascular thrombosis in kidney | Ulcer lesions with intravascular thrombosis in intestine | not examined | not examined |
Diagnosis of aHUS | Day 16 after renal transplantation | Day 7 after admission | Day 4 after admission | Day 1 after admission |
Trigger | Renal transplantation | Influenza/acute interstitial pneumonia (AIP)/disseminated intravascular coagulation (DIC) | Influenza-mimicking symptoms/AIP/DIC | Severe fever with thrombocytopenia syndrome |
Treatment | Plasma exchange, prednisolone | Plasma exchange, prednisolone | Plasma exchange, prednisolone, eculizumab | Plasma exchange, prednisolone, eculizumab |
Treatment response | Transient response → progressive disease | Complete remission | Partial remission (CBC, LDH, renal dysfunction) Progressive disease (Lung) | Complete remission |
Side effect | – | – | – | – |
Outcome | Dead (34Â days) | Alive (768Â days) | Dead (13Â days) | Alive (235Â days) |
Genetic mutation | – | – | – | – |
Genetic variants | CFH (H402Y, E936D), THBD (A473V) | CFH (V62I, H402Y, V837I) | CFH (H402Y, E936D), THBD(A473V) | CFH (V62I, H402Y,E936D), THBD (A473V) |