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Treatment of congenital afibrinogenemia with cryoprecipitate collected through a plasmapheresis program using
H W Thompson1, S Touris, S Giambartolomei
1Department of Pathology, Bonfils Blood Center, Denver, Colorado 80262, USA. hannis.thompson@uchsc.edu
Insights
Establishing a limited donor program using apheresis donors for a child with afibrinogenemia significantly reduced donor exposure while minimizing bleeding through prophylactic cryoprecipitate transfusions.
Area of Science:
- Hematology
- Transfusion Medicine
Background:
- Afibrinogenemia presents challenges for managing traumatic bleeding.
- Prophylactic transfusion therapy is considered for patients with recurrent bleeding episodes.
Observation:
- A child with afibrinogenemia experienced recurrent traumatic bleeding.
- Parents requested a limited donor program for ongoing transfusion therapy.
Findings:
- Automated plasmapheresis from parents and selected donors yielded cryoprecipitate with a mean of 606 mg fibrinogen per 500 mL donation.
- Prophylactic cryoprecipitate transfusions every 2-3 weeks over 16 months (166 U from 84 donations) resulted in an 87% reduction in donor exposure compared to random donors.
- The patient experienced minimal bleeding during the study period.
Implications:
- A limited apheresis donor program is a viable strategy for managing rare bleeding disorders like afibrinogenemia.
- This approach reduces the risk of alloimmunization and infectious disease exposure.
- It offers a practical solution for long-term prophylactic transfusion therapy.
Abstract:
A child with afibrinogenemia was evaluated for prophylactic cryoprecipitate transfusion due to recurrent episodes of traumatic bleeding. The parents would only consider ongoing transfusion therapy if a limited donor program could be established. Automated plasmapheresis was performed on a regular basis on the patient's parents and a limited number of selected donors. Studies on the initial units collected demonstrated that a single 500 mL plasmapheresis yielded a mean of 606 mg fibrinogen in the cryoprecipitate, which was stored in two bags. A total of 166 U of cryoprecipitate from 84 individual plasmapheresis donations were transfused prophylactically every 2-3 weeks over a 16-month period. Compared to transfusions from random donors, the use of a limited number of apheresis donors resulted in a donor exposure reduction of 87%. Clinically, the patient has had minimal bleeding during this period.