A 49-Year-Old Man With Steroid-Refractory Chronic Graft Versus Host Disease

Case: A 49-Year-Old Man With Steroid-Refractory Chronic Graft Versus Host Disease

Initial presentation

• A 49-year-old man complains of “color and texture” changes to his skin, nails and the inside of his mouth; he also complains of shoulder and elbow joint discomfort limiting his normal daily activity
• PMH: he underwent matched related allogenic transplant from his brother for treatment of AML
• PE: depigmentation and lichen planus-like features noted on his chin, nose, cheeks and forearms bilaterally, and in the oral mucosa (~40% BSA); longitudinal ridging of the fingernails; joint stiffness and decreased range of motion
  
  

Clinical workup

• Labs: plt 70 x 109/L, total bilirubin 7.6 mg/dl, AST 150 U/L, ALT 165 U/L, ALP 430 U/L
  • Negative for HBV, HBV, CMV, EBV, HHV-6
• NIH Global Severity of cGvHD moderate cGvHD; P-ROM score 4
• ECOG 1
  
  

Treatment

• Tacrolimus + dexamethasone oral rinse
  • No treatment response after 4 weeks
• He was started on ruxolitinib 5 mg PO BID which was tolerated well; increased to 10 mg PO BID on day 6