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Title: Severe Gastrointestinal Bleeding and Thrombocytopenia in a Child With an Anti-GATA1 Autoantibody
Authors: de Waele, Liesbeth ×
Freson, Kathleen
Louwette, Sophie
Thys, Chantal
Wittevrongel, Christine
De Vos, Rita
Debeer, Anne
Van Geet, Christel #
Issue Date: Mar-2010
Publisher: Int pediatric research foundation, inc
Series Title: Pediatric research vol:67 issue:3 pages:314-319
Abstract: We describe a patient, who developed during the first week of life petechiae and hematomas caused by severe thrombocytopenia and gastrointestinal bleeding due to Multiple small gastric hemangiomata. Bone marrow examination showed hypermegakaryocytosis and dysmegakaryopoiesis. Alloimmune thrombocytopenia was excluded. Only 3 y later, platelet counts normalized and bleedings disappeared but small skin hemangiomata remained. Electron microscopy showed enlarged round platelets with a paucity of alpha granules similar as in GATA1-deficient patients but no GATA1 mutation was found. Immunoblot analysis showed a strong interaction between patient Igs and recombinant GATA1, GATA2, and the N finger (Nf) of GATA1. The lymphocyte transformation test with recombinant GATA1Nf was positive. In vitro culturing of normal CD34(+) cells with purified patient Igs showed a decreased number of megakaryocyte colonies but an increased overall size of the colonies compared with control Igs. Mice injected with patient Igs showed a reduced platelet Count compared with mice injected with control Igs. Thrombopoiesis was also reduced after injection of patient Igs in transgenic zebrafish compared with control Igs. In conclusion, this study is the first report of an anti-GATA1 autoantibody leading to severe thrombocytopenia and gastrointestinal bleeding from multiple pinpoint hemangiomata. (Pediatr Res 67: 314-319, 2010)
ISSN: 0031-3998
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Molecular and Vascular Biology
Animal Research Center
Translational Cell & Tissue Research
Section Newborn (-)
× corresponding author
# (joint) last author

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