Chronic immune thrombocytopenia in a child with X-linked agammaglobulinemia-an uncommon phenotype

Abstract

Autoimmune disorders are common in patients with primary immunodeficiency diseases (PIDs). However, the prevalence of autoimmunity is low in patients with X-linked agammaglobulinemia (XLA), mostly due to the absence of antibodies. Chronic or persistent immune thrombocytopenia (ITP), which is usually considered an antibody-mediated disease, is uncommon in patients with XLA. In this study, we detailly described a surprising autoimmune phenomenon, chronic ITP, in a small boy diagnosed with XLA. This is an interesting phenotype found in XLA, and it is helpful to understand the immune pathogenesis of autoimmunity in patients with XLA.

Keywords:

• Autoimmunity immune
• thrombocytopenia
• X-linked agammaglobulinemia

Acknowledgements

We thank Dr Jian Hu, Dr Xiaodong Zhao and Dr Jun Yang for their treatment recommendations and assistance.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Consent to participate

Informed consent had been obtained from the patient’s legal guardians.

Ethics approval

The study was approved by the Ethics Committee of the Tianjin Children’s Hospital.

Authors’ contributions

JY, JM, XL, JX, QA and CL participated in clinical treatment and collected the clinical data. JY interpreted data and wrote the paper.

Additional information

Funding

This work was supported by the Science and Technology Fund of Tianjin Health Commission [ZC20066].