CASE STUDY: Surgical Procedure in an Adolescent Patient with Severe Hemophilia A on Emicizumab Prophylaxis
This case study is narrated by MUDr. Bohumír Blažek from the Clinic of Pediatric Medicine at LF OU and FN Ostrava. The patient is a 17-year-old boy with severe hemophilia A. Several months prior to the procedure under discussion, he was switched from prophylactic factor VIII administration to emicizumab prophylaxis. Subsequently, an extraction of all four molars was planned for orthodontic reasons. The case study describes the hematologist's approach to the procedure and the subsequent healing process.
Family History
The mother is being treated for thyroid disease, the father is healthy, and the patient has no siblings. There is no known occurrence of blood or other serious diseases in the family.
Personal History
Perinatal history is unremarkable, birth at 40 weeks of gestation. He only had a significant disease, varicella. He was hospitalized a total of 3 times for insertion or removal of a central venous catheter concerning the primary disease and once in the past for dental restoration. He has atopic eczema; other allergic manifestations are absent. Pharmacological history pertains only to the treatment of the primary hematological disease.
At the age of 9 months (in February 2005), he was diagnosed with severe hemophilia A with a factor VIII level of 0.9%.
Current Illness
At the age of 3 years, the presence of a factor VIII inhibitor was detected in the form of a high responder, with a neutralizing antibody value up to 10 BU. Immune tolerance was induced with the elimination of the inhibitor, followed by prophylaxis with factor VIII concentrates—initially with short and later prolonged action. Prophylaxis required relatively high doses of factor VIII (about 50 IU/kg), and even during this, minor bleeding occurred. Gradually, the options for venous system application were also exhausted.
In March 2021, the patient was switched to emicizumab prophylaxis, which proceeded without problems and without any side effects. During this prophylaxis, there was no bleeding. In July 2021, a stomatological surgery—extraction of all 4 molars—was indicated for orthodontic reasons. The emicizumab level at the 14-day prophylaxis interval was checked just before administration, and a value of 42.8 mg/ml was found.
MUDr. Bohumír Blažek
Department of Pediatric Hematology and Hematooncology, Clinic of Pediatric Medicine LF OU and FN Ostrava
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