Childhood Hodgkin Lymphoma: Think DADA2
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To the Editor
Deficiency of adenosine deaminase 2 (DADA2) is a recently described inborn error of immunity caused by biallelic deleterious mutations in adenosine deaminase 2 (ADA2) gene (formerly known as CECR1). It is an auto-inflammatory disorder characterized by fevers and vasculopathy, ranging from livedo racemosa over Raynaud phenomenon and digital necrosis to lacunar ischemic stroke. Cytopenia (e.g., pure red cell aplasia), mild immunodeficiency, and chronic liver disease are also part of the phenotype . Over 170 DADA2 cases have been reported; however, the pathophysiology of DADA2 is still ill defined [2, 3]. Adenosine deaminase 2 (ADA2) may play a role in endothelial integrity and induces differentiation of monocytes into macrophages. Steroids, cyclosporine, tacrolimus, azathioprine, and mycophenolate mofetil have been unsuccessful in controlling the disease. The mainstay of treatment is TNF-inhibition with etanercept/infliximab/adalimumab as it controls fever and...
We would like to thank Saudi Society of Blood and Marrow Transplantation (SSBMT) for support and enhance hematology research in the KSA.
Wrote the manuscript: Alabbas F, Elyamany G, and Meyts I. Contributed to the writing of the manuscript: Alsharief O and Hershfield M. Agree with manuscript conclusions: Alabbas F, Elyamany G, and Meyts I. Made critical revisions and approved final version: Alabbas F and Meyts I. All authors reviewed and approved the final manuscript.
This work was supported by the SANAD Children’s Cancer Support Association and SANAD Research Grants Program (grant number: RGP-2017-02).
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Conflict of Interest
The authors declare that they have no competing interests
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- 18.Springer JM, Gierer SA, Jiang H, Kleiner D, Deuitch N, Ombrello AK, et al. Deficiency of adenosine deaminase 2 in adult siblings: many years of a misdiagnosed disease with severe consequences. https://doi.org/10.3389/fimmu.2018.0136.