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.

[1]  J. Di Paola,et al.  Current therapy for rare factor deficiencies , 2001, Haemophilia : the official journal of the World Federation of Hemophilia.

[2]  F. Gabreëls,et al.  Hereditary prothrombin deficiency presenting as intracranial haematoma in infancy. , 1999, Neuropediatrics.

[3]  P. Mannucci,et al.  Rare Coagulation Disorders , 1999, Thrombosis and Haemostasis.

[4]  M. Köhler.  Thrombogenicity of prothrombin complex concentrates. , 1999, Thrombosis research.

[5]  E. Lechler,et al.  Use of prothrombin complex concentrates for prophylaxis and treatment of bleeding episodes in patients with hereditary deficiency of prothrombin, factor VII, factor X, protein C protein S, or protein Z. , 1999, Thrombosis research.

[6]  R. Seitz,et al.  Prothrombin Complex Concentrates , 1999 .

[7]  A. Bertomoro,et al.  Congenital deficiencies and abnormalities of prothrombin. , 1998, Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis.

[8]  V. Catanzarite,et al.  Pregnancies in a patient with congenital absence of prothrombin activity: case report. , 1997, American journal of perinatology.

[9]  A. O'marcaigh,et al.  Genetic analysis and functional characterization of prothrombins Corpus Christi (Arg382-Cys), Dhahran (Arg271-His), and hypoprothrombinemia. , 1996, Blood.

[10]  H. Kato,et al.  A point mutation (Arg271→Cys) of a homozygote for dysfunctional prothrombin, prothrombin Obihiro, which has a region of high sequence variability , 1995, British journal of haematology.

[11]  P. Reitsma,et al.  Homozygosity for a Novel Missense Mutation in the Prothrombin Gene Causing a Severe Bleeding Disorder , 1994, Thrombosis and Haemostasis.

[12]  H. Roberts,et al.  Current management of hemophilia B. , 1993, Hematology/oncology clinics of North America.

[13]  O. Prou-Wartelle,et al.  Demi-vie de la prothrombine vraie (facteur II) , 1964 .

[14]  R. Biggs,et al.  The Fate of Prothrombin and Factors VIII, IX and X Transfused to Patients Deficient in these Factors , 1963, British journal of haematology.

[15]  K. Schimpf.  Substitutionstherapie bei angeborenen Gerinnungsstörungen , 1988 .

[16]  K. Schimpf.  Klinische Studien zur Infektiosität von konventionellen und virusinaktivierten Gerinnungsfaktorenkonzentraten , 1987 .

[17]  H. Ekelund,et al.  Combined deficiency of coagulation factors II, VII, IX, and X: a case of probable congenital origin. , 1986, Pediatric hematology and oncology.

[18]  K. Mann,et al.  Prothrombin Quick. A newly identified dysprothrombinemia. , 1978, Mayo Clinic proceedings.