title: Allogeneic hematopoietic stem cell transplantation in Glanzmann thrombasthenia complicated by platelet alloimmunization.
abstract: BACKGROUND
  For Thrombasthenia Glanzmann (GT) patients presenting with a severe clinical phenotype due to complete lack of thrombocyte function or increased titres of anti-platelet antibodies hematopoietic stem cell transplantation (SCT) is the only curative therapy.
  
  
  METHODS
  We report the case of a 13-month-old boy, presenting with a severe course of GT, who was successfully treated with an HLA-identical sibling bone marrow transplant. SCT was complicated by anti-platelet alloimmunization after platelet transfusion successfully treated with high dosage immunoglobulins (2 g/kg) and partial plasma exchange.
  
  
  CONCLUSIONS
  SCT may be a viable option for selected patients with GT. However, SCT in GT carries its own significant risks, resulting from the development of anti-platelet antibodies. A critical risk-benefit analysis is mandatory prior to SCT.
authors.affiliation: Department of Pediatric Hematology, Oncology and Neurooncology, Pediatric Stem Cell Transplantation Program, University Children's Hospital Würzburg, Germany. Wiegering_V@klinik.uni-wuerzburg.de
authors.forename: V
authors.initials: V
authors.lastname: Wiegering
authors.forename: B
authors.initials: B
authors.lastname: Winkler
authors.forename: F
authors.initials: F
authors.lastname: Langhammer
authors.forename: M
authors.initials: M
authors.lastname: Wölfl
authors.forename: J
authors.initials: J
authors.lastname: Wirbelauer
authors.forename: K
authors.initials: K
authors.lastname: Sauer
authors.forename: A
authors.initials: A
authors.lastname: Kobsar
authors.forename: T
authors.initials: T
authors.lastname: Meyer
authors.forename: A
authors.initials: A
authors.lastname: Strauss
authors.forename: T
authors.initials: T
authors.lastname: Bakchoul
authors.forename: M
authors.initials: M
authors.lastname: Eyrich
authors.forename: P G
authors.initials: PG
authors.lastname: Schlegel
mesh_terms: D001792:Blood Platelets / Q000276:immunology*; D002869:Chromosome Aberrations; D005260:Female; D005808:Genes, Recessive / Q000235:genetics; D006580:Genetic Carrier Screening; D018380:Hematopoietic Stem Cell Transplantation; D006650:Histocompatibility Testing; D006801:Humans; D007116:Immunization, Passive; D007223:Infant; D007518:Isoantibodies / Q000097:blood*; D010951:Plasma Exchange; D010979:Platelet Function Tests; D013915:Thrombasthenia / Q000235:genetics / Q000276:immunology* / Q000628:therapy*; D014184:Transplantation, Homologous
chemical_list: D007518:Isoantibodies