Clinical features of autoimmune hemolytic anemia (AIHA) develop during pregnancy or postpartum/puerperium in age-matched healthy women
| . | All patients . | AIHA in pregnancy . | AIHA in puerperium . | Controls . |
|---|---|---|---|---|
| Mean age at pregnancy (y) (range) | 32 (21-41) | 32 (21-39) | 29.5 (24-41) | 32 (20-40) |
| Number of observed pregnancies | 45 | 18 | 6 | 56 |
| AIHA type∗ | ||||
| Warm | 14 | 7 | 2 | |
| Warm IgG+C | 9 | 6 | 3 | |
| Atypical | 5 | 3 | 1 | — |
| Mixed | 3 | — | — | |
| Cold | 2 | 2 | — | |
| Associated conditions, n (%) | 8 | 6 | 2 | — |
| Week of gestation, median (range) | — | 20.5 (5-32) | — | — |
| Week from delivery, median (range) | — | — | 6 (4-6) | — |
| AIHA therapies | ||||
| Steroids | 23 | 17 | 6 | |
| Blood transfusions | 14 | 10 | 4 | |
| Immunoglobulins injection | 7 | 6 | 1 | — |
| Rituximab (postpartum) | 4 | 4 | — | |
| Cyclosporin (postpartum) | 1 | — | 1 | |
| Antithrombotic prophylaxis | ||||
| Heparin | 4 | 3 | 1 | |
| ASA | 2 | 1 | 1 | — |
| LMWH+ASA | 2 | 2 | — | |
| Total, n (%) | 8 (33) | 6 (33) | 2 (33) | |
| Maternal complications | ||||
| Risk of preterm birth | — | — | — | 2 |
| Preeclampsia | 2 | 1 | — | 1 |
| Placental detachment | 1 | 1 | — | 1 |
| PPROM | 1 | 1 | — | 1 |
| Biliary colic | 1 | — | — | — |
| Cholestasis of pregnancy | — | — | — | 1 |
| Infective complication | 2 | 2 | — | 1 |
| Venous thrombosis | — | — | — | 1 |
| Gestational diabetes | — | — | — | 1 |
| Decrease in Hb level | — | — | — | 1 |
| Total, n (%) | 7 (15) | 5 (28) | — | 10 (18) |
| Miscarriages, n (%) | 6 (13)† | 1 (5) | — | — |
| Fetal complications | ||||
| Fetal growth restriction | 3 | 2 | 1 | 3 |
| Death | 2 | 2 | — | — |
| AIHA of the newborn | 1 | 1 | — | — |
| Preterm birth | 2 | 2 | — | — |
| Epilepsy | 1 | 1 | — | — |
| Perinatal respiratory distress | 1 | 1 | — | — |
| Total n (%) | 10 (22) | 9 (50) | 1 (16) | 3 (5) |
| . | All patients . | AIHA in pregnancy . | AIHA in puerperium . | Controls . |
|---|---|---|---|---|
| Mean age at pregnancy (y) (range) | 32 (21-41) | 32 (21-39) | 29.5 (24-41) | 32 (20-40) |
| Number of observed pregnancies | 45 | 18 | 6 | 56 |
| AIHA type∗ | ||||
| Warm | 14 | 7 | 2 | |
| Warm IgG+C | 9 | 6 | 3 | |
| Atypical | 5 | 3 | 1 | — |
| Mixed | 3 | — | — | |
| Cold | 2 | 2 | — | |
| Associated conditions, n (%) | 8 | 6 | 2 | — |
| Week of gestation, median (range) | — | 20.5 (5-32) | — | — |
| Week from delivery, median (range) | — | — | 6 (4-6) | — |
| AIHA therapies | ||||
| Steroids | 23 | 17 | 6 | |
| Blood transfusions | 14 | 10 | 4 | |
| Immunoglobulins injection | 7 | 6 | 1 | — |
| Rituximab (postpartum) | 4 | 4 | — | |
| Cyclosporin (postpartum) | 1 | — | 1 | |
| Antithrombotic prophylaxis | ||||
| Heparin | 4 | 3 | 1 | |
| ASA | 2 | 1 | 1 | — |
| LMWH+ASA | 2 | 2 | — | |
| Total, n (%) | 8 (33) | 6 (33) | 2 (33) | |
| Maternal complications | ||||
| Risk of preterm birth | — | — | — | 2 |
| Preeclampsia | 2 | 1 | — | 1 |
| Placental detachment | 1 | 1 | — | 1 |
| PPROM | 1 | 1 | — | 1 |
| Biliary colic | 1 | — | — | — |
| Cholestasis of pregnancy | — | — | — | 1 |
| Infective complication | 2 | 2 | — | 1 |
| Venous thrombosis | — | — | — | 1 |
| Gestational diabetes | — | — | — | 1 |
| Decrease in Hb level | — | — | — | 1 |
| Total, n (%) | 7 (15) | 5 (28) | — | 10 (18) |
| Miscarriages, n (%) | 6 (13)† | 1 (5) | — | — |
| Fetal complications | ||||
| Fetal growth restriction | 3 | 2 | 1 | 3 |
| Death | 2 | 2 | — | — |
| AIHA of the newborn | 1 | 1 | — | — |
| Preterm birth | 2 | 2 | — | — |
| Epilepsy | 1 | 1 | — | — |
| Perinatal respiratory distress | 1 | 1 | — | — |
| Total n (%) | 10 (22) | 9 (50) | 1 (16) | 3 (5) |
ASA, acetylsalicylic acid; C, complement; LMWH, low molecular weight heparin; PPROM, preterm premature rupture of membranes.
Five patients had Evans syndrome (ie, the association of AIHA with immune thrombocytopenia and/or neutropenia); atypical AIHA included 1 IgA-mediated and 4 DAT-negative forms. In the latter case, the diagnosis was established after excluding all other potential causes of hemolysis and was based on the steroid response.
Five out of 6 miscarriages occurred in a woman with refractory AIHA, who never achieved a complete response, even without frank relapses during pregnancy.