We report a pediatric patient with xeroderma pigmentosum (XP) who developed hemophagocytic lymphohistiocytosis (HLH)
secondary to Brucella infection—an exceedingly rare occurrence. XP is a rare autosomal recessive genetic disorder characterized
by extreme ultraviolet radiation (UVR) sensitivity due to the inability to repair DNA pyrimidine dimers caused by UV exposure.
Tis defect leads to a markedly increased risk of skin cancer and progressive neurological degeneration (Leung, 2022). HLH is

a rare, potentially fatal hypersensitivity syndrome characterized by excessive activation and impaired downregulation of T-
lymphocytes and macrophages. Tis dysregulation results in an overproduction of proinfammatory cytokines, destruction of

blood cells, and subsequent tissue and organ damage (Fisman, 2000). While secondary HLH may follow various infections,
Brucella-induced HLH is rare (Wolska, 2006), and to our knowledge, this is the frst reported case in a patient with XP.

Title

Wiley Case Reports in Hematology

Publisher

wiley

Publisher Country

United States of America

Indexing

Scopus

Impact Factor

None

Publication Type

Both (Printed and Online)

Volume

--

Year

2025

Pages

5