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Successful self-infusion of activated prothrombin complex concentrate for prophylaxis in a child with a factor VIII

Shouichi Ohga1, Akihiko Nomura, Hidetoshi Takada

  • 1Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. ohgas@pediatr.med.kyushu-u.ac.jp

American Journal of Hematology
|September 7, 2006
PubMed

Insights

Home-based prophylaxis with activated prothrombin complex concentrate (APCC) effectively managed a hemophilia A patient with a high-titer inhibitor. This treatment reduced bleeding and improved quality of life.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Coagulation Disorders

Background:

  • Hemophilia A is a genetic bleeding disorder.
  • Factor VIII inhibitors complicate treatment, necessitating alternative strategies.
  • Previous treatments with prothrombin complex concentrate (PCC) were insufficient.

Observation:

  • A 14-year-old boy with hemophilia A and a high-titer factor VIII inhibitor (127 BU/mL) was treated.
  • He experienced recurrent bleeding episodes despite on-demand and prophylactic PCC (PROPLEX-ST) therapy.
  • Intracranial hemorrhage at age 10 led to intensified PCC prophylaxis.

Findings:

  • Initiation of self-prophylaxis with activated prothrombin complex concentrate (APCC), FEIBA VH (50 U/kg thrice weekly), at age 14 resulted in no bleeding or thrombotic events over 2 years.
  • Factor VIII inhibitor levels decreased significantly to 1 BU/mL.
  • Patient reported improved quality of life, particularly in school attendance and participation.

Implications:

  • Home-based, self-administered APCC prophylaxis is a viable and effective strategy for managing inhibitor-positive hemophilia A patients.
  • This approach can lead to improved clinical outcomes, including reduced bleeding and enhanced quality of life.
  • Long-term, disciplined prophylaxis with APCC may contribute to inhibitor titer reduction.

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