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Management of Glanzmann's Thrombasthenia – Guidelines based on an expert panel consensus from gulf cooperation council countries
Ahmad Tarawah1, Tarek Owaidah2, Naima Al-Mulla3, Muhammad Faisal Khanani4, Jehan Elhazmi1, Muneer Albagshi5, Yaser Wali6, Shikah AlMohareb7, Abdulkareem Almomen8
1 Department of Hematology, Maternity and Children Hospital, King Abdullah Medical City, Madinah, Saudi Arabia
2 King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
3 Department of Hematology, Hamad Medical Corporation, Doha, Qatar
4 Department of Hematology, Tawam Hospital, Alain, UAE
5 Department of Gynecology, Hereditary Blood Diseases Center, Alhassa, Saudi Arabia
6 Department of Hematology, Sultan Qabos University, Masqat, Oman
7 Department of Hematology, Kuwait University, Kuwait
8 National Blood and Cancer Center, Riyadh, Saudi Arabia
Correspondence Address:
Dr. Ahmad Tarawah
Maternity and Children Hospital, King Abdullah Medical City, Madinah
Saudi Arabia
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/joah.joah_68_18
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Glanzmann thrombasthenia is a rare disorder, due to quantitative and/or qualitative abnormalities of the platelet integrin αIIbβ3 and/or αIIbβ3. Although it is considered a rare disorder with a global incidence of 1/1,000,000 population, the case is different at the Gulf Cooperation Council countries, where prevalence rate as high as 1/40,000 in Madinah, Saudi Arabia. This makes it necessary to develop patient's management guidelines. Due to limited resources in the literature, experts' consensus was important to develop such guidelines. Experts panel elected to use recombinant activated factor VII (rFVIIa) as the first line of treatment of acute bleeds and reserve platelets transfusion for nonresponding patients or severe bleeds, rFVIIa at high dose (270 μg/kg body weight) may tried upfront. rFVIIa may be tried as prophylactic treatment in patients with frequents bleeds. Experts panel elected to allow girls with Glanzmann thrombasthenia to have menstruation and to adapt a special protocol for this purpose (Madinah protocol). Pregnancy should be managed carefully, where normal delivery encouraged under coverage of rFVIIa. Risk of bleeding should be expected in neonates. Minor surgeries and dental workup can be managed under coverage of rFVIIa. Glanzmann thrombasthenia patients with severe disease should be encouraged for hematopoietic stem cell transplant. |
