Allogeneic stem cell transplantation for Glanzmann thrombasthenia

Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding disorder characterized by normal platelet count, but lack of platelet aggregation. The molecular basis is linked to quantitative and/or qualitative abnormalities of the membrane glycoprotein IIb/IIIa complexes. Usually it is associ...

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Published inPediatric Blood & Cancer Vol. 52; no. 5; pp. 682 - 683
Main Authors Ishaqi, M. Kashif, El-Hayek, M., Gassas, Adam, Khanani, M., Trad, Omar, Baroudi, M., Jamil, A.
Format Journal Article
LanguageEnglish
Published Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.05.2009
Subjects
alloimmunization
Child
Female
Follow-Up Studies
Glanzmann thrombasthenia
hematopoietic stem cell transplantation
Humans
megakaryocyte
Stem Cell Transplantation
Thrombasthenia - surgery
Transplantation, Homologous
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Summary:Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding disorder characterized by normal platelet count, but lack of platelet aggregation. The molecular basis is linked to quantitative and/or qualitative abnormalities of the membrane glycoprotein IIb/IIIa complexes. Usually it is associated with mild bleeding but may lead to severe and potentially fatal hemorrhages. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment. However, because of the risks associated with HSCT, it is generally not recommended unless there are life threatening hemorrhages, or the patient has developed refractoriness to platelet transfusion due to antibody formation. Herein, we report an 11‐year‐old female from United Arab Emirates (UAE) with severe GT and anti platelet alloimmunization successfully treated with HSCT from her HLA‐identical sibling. Pediatr Blood Cancer 2009;52:682–683. © 2008 Wiley‐Liss, Inc.
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ISSN:1545-5009
1545-5017
1096-911X
DOI:10.1002/pbc.21888