Key result
Home prothrombin complex concentrate prophylaxis prevents abnormal bleeding for 17 months in severe prothrombin deficiency.
Why the study?
Severe hereditary prothrombin deficiency requires effective prophylactic treatment strategies to prevent bleeding and improve quality of life.
Does prophylactic prothrombin complex concentrate prevent bleeding in severe hereditary prothrombin deficiency?
Case Report (n=1)
Does prophylactic prothrombin complex concentrate prevent bleeding in severe hereditary prothrombin deficiency?
Prophylactic prothrombin complex concentrate may be an effective and safe strategy for preventing bleeding and improving quality of life in severe hereditary prothrombin deficiency.
Supports prophylactic Bebulin VH in severe prothrombin deficiency; leaves open generalizability beyond this single case.
The authors describe the evaluation and course of severe hereditary prothrombin deficiency in a 14-year-old girl first diagnosed at age 4 years. Detailed is the evolution of her treatment from episodic fresh-frozen plasma after bleeding events to prophylactic home infusions with the prothrombin complex concentrate Bebulin VH. Pharmacokinetic data on factor II recovery and half-life are presented. The patient has been essentially free of abnormal bleeding while on this prophylactic regimen for 17 months, with no toxicities and with a much improved quality of life.
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Lobel et al. (2004) conducted a case report in Severe hereditary prothrombin deficiency (n=1). Prothrombin complex concentrate (Bebulin VH) vs. Episodic fresh-frozen plasma was evaluated on Abnormal bleeding. Prophylactic home infusions with the prothrombin complex concentrate Bebulin VH kept a 14-year-old girl with severe hereditary prothrombin deficiency essentially free of abnormal bleeding for 17 months.
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