Severe hemophilia A is a bleeding disorder indicating plasma factor levels of <1% and usually presents with episodes of spontaneous bleeding. Typically, a patient with severe untreated hemophilia A has at most 20 joint bleeds a year. In contrast, the patient of interest in this case report previously had up to 250 spontaneous bleeding episodes in a year, despite receiving prophylactic treatment. Here, we present a 65-year-old patient with an extreme phenotype of severe hemophilia A who, despite the excessive number of spontaneous bleeds, has demonstrated strong resilience and an uncommon achievement of significant milestones. The patient was managed with a multivariable approach, which included a thorough and consistent physical therapy regimen, prophylactic measures, and multiple different treatments, which most recently incorporated emicizumab (Hemlibra ® ). Because this patient is 65 years old, he has essentially grown up with the scientific advancements in his treatments. This case report suggests the possibility of a significant improvement of quality of life in older populations with severe hemophilia A by considering a multivariable approach.
Oh et al. (Thu,) studied this question.