March 12, 2021
Yi-Bin Chen, MD


Case: A 50-Year-Old Woman With Steroid-Refractory Acute Graft Versus Host Disease

Initial Presentation

  • A 50-year-old woman presents on day +32 for a routine follow-up visit after myeloablative matched unrelated donor peripheral blood stem cell transplant for AML.She complains of a new erythematous macular rash and some mildly loose stools which she estimates is 450cc in 24 hours.
  • PMH: unremarkable
  • PE: rash noted on her neck, shoulders, and upper trunk (~60% BSA)

Clinical Work-up

  • Labs: total bilirubin 2.7 mg/dl, AST 60 U/L, ALT 75 U/L
  • Stool testing negative for bacterial/viral infection
  • Negative for HBV, HBV, CMV, EBV, HHV-6
  • Skin biopsy of the rash showed sparse inflammation and abundant dyskeratotic keratinocytes
  • Flexible sigmoidoscopy showed patchy erosion and biopsy showed inflammatory cells with cryptitis
  • She was diagnosed with aGvHD:
    • Skin stage 2
    • GI stage 1
    • Liver stage 1
    • Modified Glucksberg Criteria: grade II; MAGIC Criteria: grade II
  • ECOG 1

Treatment

  • Admitted as an inpatient for evaluation and initiated methylprednisolone 1.0 mg/kg and topical steroids
  • No treatment response after 3 days, after dose increase of up to 2.0 mg/kg/day IV methylprednisolone
  • After 7 days of systemic steroids diarrhea was around 1500 cc/day, rash was better
  • She was started on ruxolitinib 5 mg PO BID which was tolerated well; increased to 10 mg PO BID 3 days later