INHIBITOR DEVELOPMENT IN CONGENITAL FACTOR VII DEFICIENCY – CASE PRESENTATION

, , ,

##plugins.themes.vojs.article.main##

Abstract

Congenital factor VII defi ciency is a rare disease. Inhibitor development in congenital factor VII defi ciency is an extreme rare phenomen, as the main challenge of replacement therapy by a high rate of life-threatening bleeding, that renders replacemrnt therapy less eff ective. Due to the important of the issue, we would like to present this case-report, It was a female 11 months with congenital factor VII defi ciency experiencing recurrent epistaxis, hematoma, intracranien
hemorrhages from second month of life, and successful treatment by double dose of recombinant activated VII. Some conclusions are that, inhibitor detection in congenital factor VII defi ciency with severe recurrent hemorrhage or less successful of replacement therapy is always needed; and high dose of rFVIIa in treatment of congenital factor VII defi ciency can successfully stop bleeding.

##plugins.themes.vojs.article.details##

References

1. Montgomery RR., Scott JP. (1992). Hemostasis:
disease of the fl uid phase. In: Nathan and Oski
(eds), Hematology of Infancy and Childhood, 4
th

edition, Saunders, V2: 1611.
2. Nguyen Cong Khanh (2008). Hereditary
clotting factor disorders. Pediatric Clinical
Hematology, Second Edition: 304-321.
3. See WS, Chang KO., Cheuk DL., Leung YY.,
Ha SY (2016). Inhibitor development after
liver transplantation in congenital factor VII
defi ciency. Hemophilia 22, e417- e422 [CrossRef]
[PubMed].
4. Mariani G, Napolitano M, Doice A, Garrido
RP, Batorova A, Karimi M, Batorova A, et al. (2013)
Replacement therapy for bleeding episodes in
factor VII defi ciency. A prospective evaluation.
Thromb. Hemostas. 109: 238-247.
5. Napolitano M, Doice A, Batorova A, Giangiti -
Blaizot M, Ingersley J, et al. Replacement therapy in
inherited factor VII defi ciency. Occurrence adverse
events and relation with surgery. Hemophilia, 21,
e513-e517 [CrossRef].
6. Shams M, Dorgalaleh A, Safarian N, et al.
(2019). Inhibitor development in patients with
congenital factor VII defi ciency, A study on 50
Iranian patients. Blood Coagulation and
Fibrinolysis, 30: 24–28.
7. Ramenzapour N, Zaker F, Biswas a, et al.
(2021). Inhibitor in Congenital Factor VII
Deficiency; a Rare but Serious Therapeutic
Challenge - A Systematic Literature Review. J.
Clin. Med. 10, 211.
8. Borhany M, Delbes C, Giansily-Blaizot M,
et al. (2015). A new report of FVII-inhibitor in a
patient suffering from severe congenital FVII
defi ciency. Hemophilia. 21, e336-e338 [CrossRef]
[PubMed].
9. Cattivelli K, Distefano C, Bonetti L, Testa S,
et al. (2018). Recurrent bleedings in newborn: A
Factor VII Defi ciency Case Report. Transfusion
Medicine Hemotherapy. 45: 104-106, DOI:
10.1159/000481993.
10. Tokgoz H, Caliskan U, Lavigne - Lissalde
M., et al. (2012) Successful prophylactic use
recombinant activated factor VII [rFVIIa] in a
patient congental FVII defi ciency and inhibitors
to FVII. Hemophilia, 18: e25-e27 [CrossRef].
11. Frachini M, Lippi G. (2008). Acquired factor
VIII inhibitors. Blood, 112:250- 255 [CrossRefB]
12. IngerslevJ. Christiansen K, Sorensen B,
(2005). Inhibitor to factor VII in severe factor VII
defi ciency: Detection and course of the inhibitory
response.J ThrombHaemost 2005; 3: 799–800
[CrossRef] [Pubmed].