Nilam M Shah1, Ashwin P Patel. 1. Saumrut laboratory, 402, Narayan complex, Near Navrangpura bus stand, Ahmedabad, 380 009 India.
Abstract
BACKGROUND: Factor X deficiency is a rare coagulation defect. There are occasional reports of factor X deficiency from India. Difficulty in accurate diagnosis and non-availability of ideal treatment is discussed. METHODS: Eight cases of factor X deficiency, diagnosed from 1992 to 2007 are reported here RESULTS: Seven were male while one was female. Seven patients were symptomatic from early childhood. One patient became symptomatic from 18 years of age. Factor X assay was done in 4 patients, 3 had severe deficiency and one had mild deficiency. One patient had associated factor IX deficiency. Three patients had repeated bleeding episodes requiring multiple transfusions. Two patients had intracranial bleed and one had umbilical cord bleeding at birth. There was no mortality. No patient received prophylactic transfusion. CONCLUSIONS: Factor X deficiency is a rare coagulation defect. Hereditary deficiency should be distinguished from acquired deficiency. CNS, joints and skin are the common sites of bleeding.
BACKGROUND:Factor X deficiency is a rare coagulation defect. There are occasional reports of factor X deficiency from India. Difficulty in accurate diagnosis and non-availability of ideal treatment is discussed. METHODS: Eight cases of factor X deficiency, diagnosed from 1992 to 2007 are reported here RESULTS: Seven were male while one was female. Seven patients were symptomatic from early childhood. One patient became symptomatic from 18 years of age. Factor X assay was done in 4 patients, 3 had severe deficiency and one had mild deficiency. One patient had associated factor IX deficiency. Three patients had repeated bleeding episodes requiring multiple transfusions. Two patients had intracranial bleed and one had umbilical cord bleeding at birth. There was no mortality. No patient received prophylactic transfusion. CONCLUSIONS:Factor X deficiency is a rare coagulation defect. Hereditary deficiency should be distinguished from acquired deficiency. CNS, joints and skin are the common sites of bleeding.
Entities:
Keywords:
Factor X deficiency; Fresh frozen plasma; Multiple factor deficiency; Prothrombin complex concentrate; Stuart factor; Vitamin K
Authors: F H Herrmann; G Auerswald; A Ruiz-Saez; M Navarrete; H Pollmann; S Lopaciuk; A Batorova; K Wulff Journal: Haemophilia Date: 2006-09 Impact factor: 4.287