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Xolremdi (mavorixafor)Blue Cross Blue Shield of Kansas

WHIM (warts, hypogammaglobulinemia, infections and myelokathexis) syndrome

Initial criteria

  • The patient has a diagnosis of WHIM syndrome AND ALL of the following:
  • Genetic analysis confirms mutation in the CXC chemokine receptor 4 (CXCR4) gene
  • Confirmed absolute neutrophil count (ANC) or total white blood cell (WBC) count ≤ 400 cells/microliter (prior to therapy and during no clinical evidence of infection)
  • The prescriber has assessed baseline status (prior to therapy) of the patient’s symptoms (e.g., ANC, ALC, number of infections)
  • The patient’s age is within FDA labeling for the requested indication OR there is support for use by age for the indication
  • The patient will NOT be using the requested agent in combination with any other CXCR4 antagonists (e.g., plerixafor)
  • The prescriber is a specialist in the area of the patient’s diagnosis (e.g., dermatologist, geneticist, hematologist, immunologist) or has consulted with such a specialist
  • The patient does NOT have any FDA labeled contraindications to the requested agent

Reauthorization criteria

  • The patient has been previously approved for the requested agent through the plan’s Prior Authorization process
  • The patient has had clinical benefit with the requested agent
  • The patient will NOT be using the requested agent in combination with any other CXCR4 antagonists (e.g., plerixafor)
  • The prescriber is a specialist in the area of the patient’s diagnosis (e.g., dermatologist, geneticist, hematologist, immunologist) or has consulted with such a specialist
  • The patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

12 months