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Published on: September 30, 2021
Successful prophylactic treatment for bleeding in a girl with severe hereditary prothrombin deficiency using a
Jeffrey S Lobel1, Suvankar Majumdar, Sue Kovats-Bell
1Pediatric Hematology/Oncology Program of Michigan State University/Kalamazoo Center for Medical Studies, Kalamazoo, Michigan 49007, USA. lobel@kcms.msu.edu
Abstract:
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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