Characteristics of patients with PNH and COVID-19 vaccine reactions
| Patient . | 1 . | 2 . | 3 . | 4 . | 5 . | 6 . |
|---|---|---|---|---|---|---|
| Sex | Male | Male | Female | Male | Female | Female |
| Age, y | 25 | 45 | 32 | 63 | 51 | 59 |
| Age at diagnosis, y | 24 | 24 | 23 | 31 | 43 | 46 |
| Disease history | Hemoglobinuria | Aplastic anemia, transfusion dependence, microvascular small bowel thrombosis, renal failure, smooth muscle dystonia | Aplastic anemia, transfusion dependence | Transfusion dependence, hemoglobinuria, smooth muscle dystonia | Hemoglobinuria, fatigue | Hemoglobinuria, fatigue, extravascular hemolysis with transfusion dependence on C5 inhibition |
| Complement inhibitor treatment | No | Ravulizumab | Ravulizumab* | Ravulizumab | Ravulizumab | Ravulizumab Danicopan |
| PNH clone | 11% RBC, 80% granulocytes | >99% RBC, >99% granulocytes | 39% RBC, 99% granulocytes | 99% RBC, 99% granulocytes | 77% RBC, 95% granulocytes | 76% RBC, 81% granulocytes |
| Blood type | A positive | A positive | A positive | O positive | A negative | O positive |
| COVID-19 vaccine | Pfizer-BioNTech | Pfizer-BioNTech | Moderna | Moderna | Pfizer-BioNTech | Pfizer-BioNTech |
| Vaccine dose | 2 | 1 | 2 | 2 | 2 | 2 |
| Last dose ravulizumab prior to vaccination | N/A | 4 wk | 4 wk | 4 wk (dose 1) 7 wk (dose 2) | 5 wk | 3.5 wk (dose 1) 6.5 wk (dose 2) |
| Time to symptom onset postvaccine | 5 d | Same day | Same day | Same day | 1 d | N/A |
| Duration of symptoms | 5 d | 6 d | 1 d | 6 d | 1 d | N/A |
| Hemoglobin prevaccination, g/dL | 10.9 | 11.6 | 11.3 | 11.9 | 10.9 | 11.4 |
| Hemoglobin postvaccination, g/dL | 10.4 | 8.9 | 8.4 | 10.7 (dose 1) 7.1 (dose 2) | 11.1 | 11.0 (dose 1) 10.7 (dose 2) |
| LDH prevaccination, U/L | 1127 | 211 | 255 | 305 | 207 | 263 |
| LDH postvaccination, U/L | 1633 | — | — | 312 (dose 1) 342 (dose 2) | 220 | 276 (dose 1) 258 (dose 2) |
| Total bilirubin prevaccination, mg/dL | 0.8 | 1.4 | 0.7 | 2.4 | 0.6 | 1.5 |
| Total bilirubin postvaccination, mg/dL | 2.5 | — | 2.7 | 7.1 (dose 1) 3.0 (dose 2) | — | 2.1 (dose 1) 1.2 (dose 2) |
| AST prevaccination, U/L | 70 | 18 | 19 | 26 | 17 | 32 |
| AST postvaccination, U/L | 102 | — | 25 | 24 (dose 1) 22 (dose 2) | — | 31 (dose 1) 24 (dose 2) |
| Hospitalization/emergency department | Yes | No | Yes | No | No | No |
| Thrombotic complication | Microvascular small bowel thrombosis | No | No | No | No | No |
| Transfusion required† | No | No | 2 units RBC | No | No | No |
| Vaccine reaction | Abdominal pain, fever | Fever, myalgia, headache, fatigue, hemoglobinuria | Fever, chills | Fever, fatigue, dark urine, vomiting, diarrhea | Headache, fatigue | None |
| Patient . | 1 . | 2 . | 3 . | 4 . | 5 . | 6 . |
|---|---|---|---|---|---|---|
| Sex | Male | Male | Female | Male | Female | Female |
| Age, y | 25 | 45 | 32 | 63 | 51 | 59 |
| Age at diagnosis, y | 24 | 24 | 23 | 31 | 43 | 46 |
| Disease history | Hemoglobinuria | Aplastic anemia, transfusion dependence, microvascular small bowel thrombosis, renal failure, smooth muscle dystonia | Aplastic anemia, transfusion dependence | Transfusion dependence, hemoglobinuria, smooth muscle dystonia | Hemoglobinuria, fatigue | Hemoglobinuria, fatigue, extravascular hemolysis with transfusion dependence on C5 inhibition |
| Complement inhibitor treatment | No | Ravulizumab | Ravulizumab* | Ravulizumab | Ravulizumab | Ravulizumab Danicopan |
| PNH clone | 11% RBC, 80% granulocytes | >99% RBC, >99% granulocytes | 39% RBC, 99% granulocytes | 99% RBC, 99% granulocytes | 77% RBC, 95% granulocytes | 76% RBC, 81% granulocytes |
| Blood type | A positive | A positive | A positive | O positive | A negative | O positive |
| COVID-19 vaccine | Pfizer-BioNTech | Pfizer-BioNTech | Moderna | Moderna | Pfizer-BioNTech | Pfizer-BioNTech |
| Vaccine dose | 2 | 1 | 2 | 2 | 2 | 2 |
| Last dose ravulizumab prior to vaccination | N/A | 4 wk | 4 wk | 4 wk (dose 1) 7 wk (dose 2) | 5 wk | 3.5 wk (dose 1) 6.5 wk (dose 2) |
| Time to symptom onset postvaccine | 5 d | Same day | Same day | Same day | 1 d | N/A |
| Duration of symptoms | 5 d | 6 d | 1 d | 6 d | 1 d | N/A |
| Hemoglobin prevaccination, g/dL | 10.9 | 11.6 | 11.3 | 11.9 | 10.9 | 11.4 |
| Hemoglobin postvaccination, g/dL | 10.4 | 8.9 | 8.4 | 10.7 (dose 1) 7.1 (dose 2) | 11.1 | 11.0 (dose 1) 10.7 (dose 2) |
| LDH prevaccination, U/L | 1127 | 211 | 255 | 305 | 207 | 263 |
| LDH postvaccination, U/L | 1633 | — | — | 312 (dose 1) 342 (dose 2) | 220 | 276 (dose 1) 258 (dose 2) |
| Total bilirubin prevaccination, mg/dL | 0.8 | 1.4 | 0.7 | 2.4 | 0.6 | 1.5 |
| Total bilirubin postvaccination, mg/dL | 2.5 | — | 2.7 | 7.1 (dose 1) 3.0 (dose 2) | — | 2.1 (dose 1) 1.2 (dose 2) |
| AST prevaccination, U/L | 70 | 18 | 19 | 26 | 17 | 32 |
| AST postvaccination, U/L | 102 | — | 25 | 24 (dose 1) 22 (dose 2) | — | 31 (dose 1) 24 (dose 2) |
| Hospitalization/emergency department | Yes | No | Yes | No | No | No |
| Thrombotic complication | Microvascular small bowel thrombosis | No | No | No | No | No |
| Transfusion required† | No | No | 2 units RBC | No | No | No |
| Vaccine reaction | Abdominal pain, fever | Fever, myalgia, headache, fatigue, hemoglobinuria | Fever, chills | Fever, fatigue, dark urine, vomiting, diarrhea | Headache, fatigue | None |
— denotes no information is available; AST, aspartate amino transferase; LDH, lactate dehydrogenase; N/A, not applicable; RBC red blood cell.
Additionally on danicopan; however, 2 doses were missed immediately following vaccination.
None of these patients were previously transfused in the past year.