Monday, June 5, 2023
| 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) |
Table 1. Clinical features of autoimmune hemolytic anemia (AIHA) develop during pregnancy or postpartum/
puerperium in age-matched healthy women
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.
Physicians - maximum of 1.00 AMA PRA Category 1 Credit(s)™
This activity is intended for hematologists, immunologists, obstetricians, neonatologists, and other physicians caring for women with autoimmune hemolytic anemia (AIHA) during pregnancy and their offspring.
The goal of this activity is for learners to be better able to describe AIHA severity, treatment need, and impact on maternal and fetal outcomes during pregnancy/puerperium, according to the results of the first multicenter international cohort study evaluating AIHA, which reported on 45 pregnancies in 33 women followed at 12 centers from 1997 to 2022.
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CME Released: 4/20/2023
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Relapsing or occurring de novo autoimmune hemolytic anemia (AIHA) during pregnancy or puerperium is a poorly described condition. Here, we report 45 pregnancies in 33 women evaluated at 12 centers from 1997 to 2022. Among the 20 women diagnosed with AIHA before pregnancy, 10 had a relapse. An additional 13 patients developed de novo AIHA during gestation/puerperium (2 patients had AIHA relapse during a second pregnancy). Among 24 hemolytic events, anemia was uniformly severe (median Hb, 6.4 g/dL; range, 3.1–8.7) and required treatment in all cases (96% steroids ± intravenous immunoglobulin, IVIG, 58% transfusions). Response was achieved in all patients and was complete in 65% of the cases. Antithrombotic prophylaxis was administered to 8 patients (33%). After delivery, rituximab was administered to 4 patients, and cyclosporine was added to 1 patient. The rate of maternal complications, including premature rupture of membranes, placental detachment, and preeclampsia, was 15%. Early miscarriages occurred in 13% of the pregnancies. Fetal adverse events (22% of cases) included respiratory distress, fetal growth restriction, preterm birth, AIHA of the newborn, and 2 perinatal deaths. In conclusion, the occurrence of AIHA does not preclude the ability to carry out a healthy pregnancy, provided close monitoring, prompt therapy, and awareness of potential maternal and fetal complications.
It has long been known that pregnancy is associated with perturbations of the immune system homeostasis, including autoimmune phenomena. It has been estimated that 1 in 50 000 pregnancies may develop antierythrocyte autoantibodies with different phenotypic expressions. However, not all patients with antierythrocyte autoantibodies develop overt autoimmune hemolytic anemia (AIHA).[1] The latter is a rare disease with an estimated incidence of 0.8 to 3 out of 100 000 individuals per year and is predominant in females.[2] De novo or relapsing AIHA during pregnancy or puerperium is even rarer, with mainly case reports, mostly of warm immunoglobulin G (IgG) type.[3] Very little is known about disease characteristics, management, maternal-fetal outcomes, and safety concerns limit treatment. Here, we report the results of the first multicenter international cohort study evaluating AIHA in the setting of pregnancy/puerperium. We aimed to delineate AIHA severity, treatment need, and the impact on maternal and fetal outcomes.
