Journal of Clinical Immunology

, Volume 39, Issue 1, pp 26–29 | Cite as

Childhood Hodgkin Lymphoma: Think DADA2

  • Fahad AlabbasEmail author
  • Ghaleb Elyamany
  • Omar Alsharif
  • Michael Hershfield
  • Isabelle Meyts
Letter to Editor

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 [1]. 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.

Funding Information

This work was supported by the SANAD Children’s Cancer Support Association and SANAD Research Grants Program (grant number: RGP-2017-02).

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no competing interests

Supplementary material

10875_2019_590_MOESM1_ESM.pdf (3.6 mb)
ESM 1 (PDF 3717 kb)


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Pediatric Hematology/Oncology and Bone Marrow TransplantPrince Sultan Military Medical City (PSMMC)RiyadhSaudi Arabia
  2. 2.Prince Sultan Military Medical City (PSMMC)RiyadhSaudi Arabia
  3. 3.Department of Central Military Laboratory and Blood BankPrince Sultan Military Medical City (PSMMC)RiyadhSaudi Arabia
  4. 4.Department of Medicine and BiochemistryDuke University Medical CenterDurhamUSA
  5. 5.Department of Pediatrics, Department of Microbiology and ImmunologyUniversity Hospitals LeuvenLeuvenBelgium

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