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

• Abstract: Regular self-infusion of an activated prothrombin complex concentrate (APCC) has been successfully introduced to a 14-year-old boy with hemophilia A. The child was diagnosed as a neonate, and at age 7 years, developed a high titer (127 BU/mL) factor VIII inhibitor coincident with a protracted ankle joint bleeding. From age 7-10 years, he received on-demand therapy using a prothrombin complex concentrate (PCC), PROPLEX-ST. From age 10-14 years, he received prophylaxis with PROPLEX-ST, initiated after an intracranial hemorrhage and coincident anamnestic inhibitor response. Throughout 7-year period of PCC treatment, he experienced recurrent bleeding episodes. Self-prophylaxis with APCC, FEIBA VH [Anti-inhibitor Coagulant Complex] (50 U/kg/dose three times per week) using infusion pump was initiated at 14 years of age and has continued for 2 years. There were no bleeding, thrombotic events or other adverse events after initiation of this prophylaxis, and inhibitor levels decreased to 1 BU/mL. His quality of life was improved, particularly with respect to school. Our long observation proposes a well-disciplined home-based FEIBA prophylaxis in inhibitor-positive hemophiliacs.
• Authors: Shouichi Ohga, Akihiko Nomura, Hidetoshi Takada, Naohiro Suga, Toshiro Hara (Affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. ohgas at pediatr.med.kyushu-u.ac.jp)
• Journal: American journal of hematology (Am J Hematol) Vol. 82 Issue 2 Pg. 145-9 (Feb 2007) ISSN: 0361-8609 [Print] United States
• PMID: 16955463 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: (c) 2006 Wiley-Liss, Inc.

Chemical References

• Blood Coagulation Factor Inhibitors
• Blood Coagulation Factors
• prothrombin complex concentrates

Topics

• Adolescent
• Asian Continental Ancestry Group
• Blood Coagulation Factor Inhibitors (blood)
• Blood Coagulation Factors (administration & dosage)
• Hemophilia A (blood, complications)
• Hemorrhage (etiology, prevention & control)
• Humans
• Japan
• Joint Diseases (etiology, prevention & control)
• Male
• Self Administration
• Thrombosis (etiology, prevention & control)