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Stem Cell Transplant in Severe Glanzmann Thrombasthenia in an Adult Patient
Mani Ramzi1, Mehdi Dehghani, Shirin Haghighat
1From the Hematology Research Center, Department of Hematology, Medical Oncology, and Stem Cell Transplantation, Shiraz University of Medical Sciences, Shiraz, Iran.
Abstract:
Glanzmann thrombasthenia is an inherited auto-somal recessive disorder characterized by normal platelet count but lack of platelet aggregation due to absence of platelet glycoprotein IIb/IIIa. The disease usually is associated with mild bleeding, but severe fatal hemorrhage may occur. Allogeneic stem cell transplant is the only curative method of treatment. A literature search showed 18 previously reported cases of Glanzmann thrombasthenia treated with allogeneic hematopoietic stem cell transplant. We report an 18-year-old woman with severe Glanzmann thrombasthenia who was treated with allogeneic hematopoietic stem cell transplant from her sister. After 24-month follow-up, the patient was well, had no bleeding tendency, and had mild chronic skin graft-versus-host disease.
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