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mavorixaforBlue Cross Blue Shield of Texas

WHIM (warts, hypogammaglobulinemia, infections and myelokathexis) syndrome

Initial criteria

  • 1. BOTH of the following:
  • A. ONE of the following:
  • 1. The patient has a diagnosis of WHIM (warts, hypogammaglobulinemia, infections and myelokathexis) syndrome AND ALL of the following:
  • A. Genetic analysis confirms mutation in the CXC chemokine receptor 4 (CXCR4) gene [chart notes required] AND
  • B. 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) AND
  • C. The prescriber has assessed baseline status prior to therapy (e.g., ANC, ALC, number of infections) OR
  • 2. The patient has another FDA labeled indication for the requested agent and route of administration
  • B. If the patient has an FDA labeled indication, then ONE of the following:
  • A. The patient’s age is within FDA labeling for the requested indication for the requested agent OR
  • B. There is support for using the requested agent for the patient’s age for the requested indication AND
  • 2. The patient will NOT be using the requested agent in combination with any other CXCR4 antagonists (e.g., plerixafor) for the requested indication AND
  • 3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., dermatologist, geneticist, hematologist, immunologist), or has consulted with a specialist AND
  • 4. The patient does NOT have any FDA labeled contraindications to the requested agent.
  • Additional approval pathway when ONE of the following is met:
  • 1. For BCBS NM Fully Insured or NM HIM member ALL of:
  • A. No FDA labeled contraindications AND
  • B. Requested indication is a rare disease AND
  • C. ONE of:
  • 1. The patient has another FDA labeled indication and route of administration OR
  • 2. Another indication supported in compendia for the requested agent and route of administration OR
  • 2. For Ohio fully insured or HIM Shop (SG) members ALL of:
  • A. Member resides in Ohio AND plan is Fully Insured or HIM Shop AND
  • B. No FDA labeled contraindications AND
  • C. ONE of:
  • 1. Another FDA labeled indication and route of administration OR
  • 2. Another indication supported in compendia OR
  • 3. Prescriber submitted TWO peer-reviewed journal articles supporting the use (accepted designs include randomized, double blind, placebo controlled clinical trials; case studies not acceptable).

Reauthorization criteria

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

Approval duration

12 months