Title of article :
A Rare Case of Co-Inheritance of Beta Thalassemia Intermedia and Coagulation FVII Deficiency
Author/Authors :
Barati Shourijeh, Zahra Department of Biochemistry - Islamic Azad University - Fars Science and Research Branch, Shiraz , Karimi, Mehran Hematology Research Center - Shiraz University of Medical Sciences
Abstract :
We present a 34-year-old man with combination of beta thalassemia intermedia
and coagulation factor VII deficiency who was presented with pallor and
irregular nose bleeding episodes. On physical examination, he had splenomegaly
and yellow sclera. Pallor and splenomegaly could be reminder of thalassemic
syndromes or hemoglobinopathies including thalassemia intermedia.
Association with unusual bleeding tendency such as prolonged and repeated
episodes of epistaxis without any evidence of liver failure in hemoglobinopathies
suggest the possibility of coinheritance of a bleeding disorder. As a result,
coagulation assay for proper diagnosis, management and prevention of probable
life-threatening bleeding episodes is suggested.
Keywords :
Thalassemia intermedia , Factor VII deficiency , Coinheritance , Liver failure , Hypercoagulability , Bleeding tendency
Journal title :
Iranian Journal of Blood and Cancer (IJBC)