Successful prophylactic treatment for bleeding in a girl with severe hereditary prothrombin deficiency using a prothrombin complex concentrate (Bebulin VH).
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.
Pediatric Hematology/Oncology Program of Michigan State University/Kalamazoo Center for Medical Studies, Kalamazoo, Michigan 49007, USA. lobel@kcms.msu.edu
Jeffrey S
JS
Lobel
Suvankar
S
Majumdar
Sue
S
Kovats-Bell
D000293:Adolescent; D001779:Blood Coagulation Factors / Q000627:therapeutic use*; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000517:prevention & control*; D006801:Humans; D007020:Hypoprothrombinemias / Q000188:drug therapy* / Q000235:genetics; D008297:Male; D010375:Pedigree; D011788:Quality of Life
D001779:Blood Coagulation Factors; C025667:prothrombin complex concentrates