Z Geburtshilfe Neonatol 2021; 225(S 01): e3
DOI: 10.1055/s-0041-1739719
Abstracts | DGPM

Fetal lupus with severe anemia and pancytopenia due to maternal lupus erythematodes treated with cordocentesis – a case report

M Rullmann
1   Universitätsspital Zürich, Geburtshilfe, Zürich, Schweiz
,
K Scheibner
2   Kantonsspital Aarau, Aarau, Schweiz
,
N Ochsenbein-Kölble
1   Universitätsspital Zürich, Geburtshilfe, Zürich, Schweiz
,
R Zimmermann
1   Universitätsspital Zürich, Geburtshilfe, Zürich, Schweiz
,
JC Fauchère
3   Universitätsspital Zürich, Neonatologie, Zürich, Schweiz
,
C Haslinger
1   Universitätsspital Zürich, Geburtshilfe, Zürich, Schweiz
› Author Affiliations
 

Introduction Systemic lupus erythematodes (SLE) is an autoimmune disease affecting mainly women of reproductive age. About 40% of pregnant patients have autoantibodies that can cross the placenta and lead to neonatal lupus. Although pancytopenia is one of the main symptoms in adult patients with SLE, fetal pancytopenia in this context is scarcely described. We report a case of fetal lupus manifesting initially with 1st degree heart block and later with severe fetal anemia requiring transfusion via cordocentesis, accompanied by transient fetal thrombocytopenia and leucopenia.

Case report We present a 31-year-old gravida 2 para 2 suffering from SLE with anti-SSA-antibodies. She was treated with hydroxychloroquine due to bicytopenia. At 19+1 weeks, fetal 1st degree AV-block was diagnosed and dexamethasone initiated. At 24+0 weeks, the heart block had resolved but the fetus showed signs of anemia (MCA-PSV max>1.5 MoM) and mild tricuspid valve regurgitation. Rhesus-alloimmunization or maternal infection as possible etiologies of fetal anemia were ruled out and the patient was referred to the University Hospital Zurich. We performed a cordocentesis at 24+3 weeks showing fetal pancytopenia (hemoglobin 46 g/l, thrombocytes 125 G/l, leucocytes 2.0 G/l), severe neutropenia (0.3 G/l) and lymphopenia (1.32 G/l). The fetus was transfused 35 ml erythrocyte concentrate in the umbilical vein. Post-interventional hemoglobin was 140 g/l and the MCA-PSV within normal ranges. After the cordocentesis, the fetus had transient mild ascites and a small subcapsular liver hematoma, the latter probably due to thrombocytopenia. Both resolved spontaneously. Since MCA-PSV remained stable in regular outpatient visits, no further fetal transfusions were necessary. The fetus was delivered by cesarean section at 35+1 weeks due to suspected intraamniotic infection after PPROM at 34+6 weeks. Postnatal blood count again showed pancytopenia (hemoglobin 121 g/l, thrombocytes 31 G/L, leucocytes 0.1 G/l). As expected, complete resolution with normal blood cell counts was observed at the age of 3 months.

Conclusion As shown in this case, maternal antibodies in SLE can not only affect fetal conductive heart tissue but may also severely impair hematopoiesis. Clinically, the most apparent manifestation is fetal anemia, which can be detected by assessment of MCA-PSV and treated with intrauterine transfusion. We thus recommend to include screening for fetal anemia in antenatal surveillance of SLE patients.



Publication History

Article published online:
26 November 2021

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