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Area of Science:

  • Immunology
  • Hematology
  • Infectious Diseases

Background:

  • VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is an emerging autoinflammatory syndrome associated with somatic mutations in UBA1.
  • Hematopoietic stem cell transplant (HSCT) is an increasingly utilized treatment for VEXAS syndrome, creating a high-risk population for infections.
  • Limited data exists on the spectrum and incidence of infections in VEXAS patients post-HSCT.

Purpose of the Study:

  • To investigate the incidence and types of infections in adult patients with VEXAS syndrome following HSCT.
  • To assess the effectiveness of prophylactic measures against infections in this cohort.
  • To inform clinical practice regarding infection surveillance and management in VEXAS patients undergoing HSCT.

Main Methods:

  • A retrospective review was conducted on ten adult patients diagnosed with VEXAS syndrome.
  • Patients included in the study had undergone HSCT at Mayo Clinic Rochester or within the Arizona medical record system.
  • Data on infection types, timing, and outcomes were analyzed.

Main Results:

  • Nine out of ten patients (90%) developed bacterial infections post-HSCT.
  • Bacteremia was present in eight of these patients (80%), despite receiving prophylactic antibiotics.
  • Viral infections occurred in six patients (60%), while no invasive fungal infections were documented. One patient had a parasitic infection.

Conclusions:

  • Bacterial infections, including bacteremia, are highly prevalent in VEXAS patients post-HSCT, irrespective of prophylaxis.
  • Healthcare providers must maintain a high index of suspicion for infections, especially bacterial, in this immunocompromised population.
  • Patient education on recognizing infection signs and symptoms is essential for early detection and management.