title: Stem Cell Transplant in Severe Glanzmann Thrombasthenia in an Adult Patient.
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.
authors.affiliation: From the Hematology Research Center, Department of Hematology, Medical Oncology, and Stem Cell Transplantation, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mani
authors.initials: M
authors.lastname: Ramzi
authors.forename: Mehdi
authors.initials: M
authors.lastname: Dehghani
authors.forename: Shirin
authors.initials: S
authors.lastname: Haghighat
authors.forename: Hourvash Haghighi
authors.initials: HH
authors.lastname: Nejad
mesh_terms: D000293:Adolescent; D005260:Female; D006086:Graft vs Host Disease; D018380:Hematopoietic Stem Cell Transplantation; D006470:Hemorrhage; D006801:Humans; D013915:Thrombasthenia / Q000503:physiopathology / Q000628:therapy*; D013997:Time Factors; D014184:Transplantation, Homologous