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Congenital Deficiency of FVII and Acquired Idiopathic Thrombocytopenic Purpura – the Rare Combination of Two Bleeding Disorders


Authors: J. Šlechtová
Authors‘ workplace: Ústav klinické biochemie a hematologie LF UK a FN, Plzeň
Published in: Čas. Lék. čes. 2008; 147: 431-433
Category: Case Report

Overview

The congenital deficiency of FVII is an autosomal recessive rare disorder. We have observed 15 patients with this deficiency in our haematology department including the only one patient with the FVII level

Key words:
congenital deficiency of FVII, idiopathic thrombocytopenic purpura, haptoglobin.


Sources

1. Friedman, K. D., Rodgers, G. M.: Inherited Coagulation Disorders,. Wintrobe’s Clinical Hematology (11th edition) Lippincott Wiliams & Wilkins, 2004, s. 1620-1667

2. Penka, M., Buliková, A., Matýšková, M., Zavřelová, J.: Hematologie I, neonkologická hematologie. Praha, Grada Publishing, 2001, s. 101–117, s. 137–138.

3. Berkow, R., Fletcher, A. J. et al.: Kompendium klinické medicíny. Krvácivé poruchy. Praha, X-Egem, 1996, s. 1057–1068

4. Cetkovský, P. et al.: Intenzivní péče v hematologii. Praha, Galén, 2004, s. 268–269

5. Koda, Y. et al.: Simple PCR detection of haptoglobin gene deletion in anhaptoglobinemic patiens with antihaproglobin antipody that cause anaphylactic transfusion reactions. Blood, 2000,95, s. 1138–1143.

6. Shimada, E. et al.: Anaphylactic transfusion reactions in haptoglobin-deficient patiens with IgE and IgG haptoglobin antibodies. Transfusion, 2002, 42, s. 766–773.

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