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Transient and chron...
Transient and chronic childhood immune thrombocytopenia are distinctly affected by Fc-γ receptor polymorphisms
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- Schmidt, DE (author)
- Karolinska Institutet
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Heitink-Polle, KMJ (author)
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Laarhoven, AG (author)
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Bruin, MCA (author)
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Veldhuisen, B (author)
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Nagelkerke, SQ (author)
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Kuijpers, TW (author)
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Porcelijn, L (author)
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van der Schoot, CE (author)
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Vidarsson, G (author)
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de Haas, M (author)
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(creator_code:org_t)
- 2019-07-03
- 2019
- English.
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In: Blood advances. - : American Society of Hematology. - 2473-9537 .- 2473-9529. ; 3:13, s. 2003-2012
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Abstract
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- In childhood immune thrombocytopenia (ITP), anti-platelet autoantibodies mediate platelet clearance through Fc-γ receptor (FcγR)–bearing phagocytes. In 75% to 90% of patients, the disease has a transient, self-limiting character. Here we characterized how polymorphisms of FcγR genes affect disease susceptibility, response to intravenous immunoglobulin (IVIg) treatment, and long-term recovery from childhood ITP. Genotyping of the FCGR2/3 locus was performed in 180 children with newly diagnosed ITP, 22 children with chronic ITP, and 180 healthy control children by multiplex ligation-dependent probe amplification. Children with newly diagnosed ITP were randomly assigned to a single administration of IVIg or observation, and followed for 1 year (Treatment With or Without IVIg for Kids With ITP [TIKI] trial). We defined transient ITP as a complete recovery (≥100 × 109/L) 3 months after diagnosis, including both self-limiting disease/IVIg responders and chronic ITP as absence of a complete recovery at 12 months. ITP susceptibility, as well as spontaneous recovery and response to IVIg, was associated with the genetic variants FCGR2C*ORF and FCGR2A*27W and the FCGR2B promoter variant 2B.4. These variants were overrepresented in patients with transient (N = 131), but not chronic (N = 43), disease. The presence of FCGR2C*ORF predisposed to transient ITP with an odds ratio of 4.7 (95% confidence interval, 1.9-14.3). Chronic ITP was associated with a deletion of FCGR2C/FCGR3B (copy number region 1) with an odds ratio of 6.2 (95% confidence interval, 1.8-24.7). Taken together, susceptibility to transient and chronic ITP is distinctly affected by polymorphic variants of FCGR2/3 genes. Our data suggest that genotyping of the FCGR2/3 locus may be useful for prognosis and guidance of treatment decisions in newly diagnosed childhood ITP.
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- art (subject category)
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- By the author/editor
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Schmidt, DE
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Heitink-Polle, K ...
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Laarhoven, AG
-
Bruin, MCA
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Veldhuisen, B
-
Nagelkerke, SQ
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show more...
-
Kuijpers, TW
-
Porcelijn, L
-
van der Schoot, ...
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Vidarsson, G
-
de Haas, M
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show less...
- Articles in the publication
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Blood advances
- By the university
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Karolinska Institutet