GLANZMAN THROMBASTHENİA AND CELİAC DİSEASE

İ. SHEN¹, M. SHAHİN², O. DORUKOZ¹, O. GUL UTKU¹, B. ÇOLAK¹, H. ERDAL¹, M. İBİŞ¹, M. ARHAN¹, K. ACAR³, G. AYGENCER⁴, S. ÜNAL¹

Gazi University, Ankara, TURKEY

Glanzmann thrombasthenia (GT) is an autosomal recessive disorder characterized by a lifelong bleeding tendency due to quantitative and qualitative abnormalities of the platelet integrin aIIbb3 (glycoprotein IIb; CD41/IIIa; CD61). Common clinical manifestations include purpuric type skin bleeding, prolonged bleeding from minor cuts, epistaxis, gingival bleeding and menorrhagia. Gastrointestinal bleeding is not very common but when present it is usually very severe. The patient, 26 year old, was urgently hospitalised in our department after endoscopic deudenal forceps biopsy. The aim of our procedure was to find a possible reason of the B12 defficiency. massive hemorhage developed after procedure, first interferred with epinephrine injection. Patient hospitalised in the intensive care unit because of massive melena and hypovolemic shock.The patient was therefore submitted to repeated infusions of red blood cell and fresh frozen plasma concentrates and antisecretory therapy (intravenous Lansaprasole 40 mg/hour). Because of the reccurent bleeding episode we performed again an endoscopic procedure which showed a leakage of blood in the deudenal wall. As another option we performed by the endoscopic way Ankaferd injection. Factor VIIa 5 mg was also injected via an intravenosly every 3 hours. on the second day need for replacement was decreased. The definitive control of the bleeding with improvement of both clinical condition and laboratory data was obtained only on the third day. On the seventh day the patient was discharged. In total support therapy required the infusion of 11 units of red blood cells, 3 units of fresh plasma, and 24 units of platelet. Biopsy showed in the deudenal biopsy sample mucosal villous atrophy. We find also a pozitive antiendomycium Ig A value which suits to a diognosis of celiac disease. In conclusion we would like to empasise that a single endoscopic gastrointestinal forceps biopsy can be resulted with a life threatening bleeding episode in GT cases. We showed in our case also the role of FVIIa in controlling the hemoragic episode. we determined celiac disease in patient with GT and cobalamin deficiency and started treatment for celiac disease.

Date: 2014-12-28; view: 816