Management of delivery of a woman with antibodies against high-prevalence red blood cell antigen Lan (Langereis) and von Willebrand disease

Background

Anti-Lan (Langereis) is a rare antibody against a high-prevalance red blood cell antigen. It has been described as having variable clinical significance for hemolytic transfusion reactions. Only few cases of hemolysis disease of the fetus and newborn caused by anti-Lan are reported yet. Von Willebrand Disease (VWD) is the most common human inherited bleeding disorder due to a defect of Von Willebrand Factor (VWF), which a glycoprotein uniquely involved in key aspects of hemostasis.

Methods

The disease's principal manifestations are spontaneous bleeding from mucous membranes, excessive bleeding from wounds, and menorrhagia. Various bleeding phenotypes are due to different types of VWD and severity of a defect of VWF. Case report: A 30 years old woman presented in the 33rd gestational week was referred to our hospital for differentiation of alloantibodies against red blood cells. Serological testing revealed an anti-Lan titer of 32. Ultrasound and fetal blood samples were not suggestive for hemolytic disease in utero. External medical records indicated a Willebrand disease of unknown type. The patient reported mild bleeding symptoms. The ISTH-Bleeding Score was 8 of 56 points.

Results

Interestingly, at the age of ten years she had a gastrointestinal bleeding of unknown origin with anemia requiring blood transfusion. After laparotomy, there was a pronounced abdominal wall hematoma. Because of the positive bleeding history and due to the difficult transfusion medical supply situation, we recommend prophylactic administration of FVIII-containing von Willebrand factor medication at delivery to minimize the risk of bleeding. In addition, blood donation by a compatible family member should be considered in these rare cases.

Conclusion

The availability of Lan-negative blood units is very rare and only a few products are frozen in specialized laboratories. Fetus of women with Anti-Lan have to be screened regularly for hemolytic disease. For the management of Anti-Lan positive patients, hemostaseological optimization is recommended.

Offenlegung Interessenkonflikt:

Nothing to declare