• J Coll Physicians Surg Pak · Dec 2022

    Case Reports

    Stem Cell Transplantation in Glanzmann's Thrombasthenia: A Report of Two Adult Patients.

    • Jehanzeb Ur Rehman, Fayyaz Hussain, Syed Kamran Mahmood, Qamar Un Nisa, Nighat Shahbaz, and Mehreen Ali Khan.
    • Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Pakistan.
    • J Coll Physicians Surg Pak. 2022 Dec 1; 32 (12): 162616281626-1628.

    AbstractGlanzmann's thrombasthenia (GT) is an autosomal recessive bleeding disorder characterised by mucocutaneous bleeding. At molecular level, defect in platelet receptor glycoprotein (GP) IIb/IIIa leads to defective platelet aggregation. Anti-fibrinolytic agents, platelet transfusions, and factor rVIIa are used for prophylaxis before invasive procedures and treatment of bleeding events. Allogeneic stem cell transplant is the only curative option. Here, we report cases of two adult male patients who underwent matched sibling donor stem cell transplantation for GT with recurrent bleeding requiring platelet and red cell transfusions. Both showed marked improvement in quality of life. To conclude, stem cell transplant is a viable treatment option for severe, difficult-to-control cases of GT. Key Words: Platelet disorders, Hematopoietic stem cell transplantation, Thrombasthenia.

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