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

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