A 61-Year-Old Man With Acute Graft-Versus-Host Disease

Case Overview:

Initial presentation

• A 61-year old man presented with a generalized pruritic rash, abdominal pain and diarrhea (8-12 BMs/day)
• PMH: he underwent haploidentical allogeneic hematopoietic-cell transplantation for AML 40 days ago
• PE: maculopapular erythematous rash on bilateral palms, cheeks, ears and upper back (rash involvement >50% of skin); abdomen is tender to palpation diffusely
  
  

Clinical workup

• Labs: ANC 0.7 x 109/L, plt 75 x 109/L, total bilirubin 8 mg/dl, AST 54 U/L, ALT 60 U/L
  • Negative for HBV, HBV, CMV, EBV, HHV-6
• Negative stool tests
• Flexible sigmoidoscopy with biopsy:
  • Lower GI biopsy showed moderate mixed inflammation in the lamina propria and scattered apoptotic bodies without crypt loss
• Skin punch biopsy of the back shows hyperkeratosis, hypergranulosis associated with lichenoid inflammatory infiltrate
• Modified Glucksberg criteria: grade 3; MAGIC criteria: grade III
  
  

Treatment

• Systemic corticosteroids (prednisone 2 mg/kg/day) + topical steroids (triamcinolone)
  • No treatment response after day 5
• He was started on ruxolitinib 5 mg PO BID, increased to 10 mg PO BID on day 6