A 49-Year-Old Man With Steroid-Refractory Chronic Graft Versus Host Disease - Episode 2

Definition of GvHD

May 21, 2020
Corey S. Cutler, MD, MPH, FRCPC

Corey S. Cutler, MD, MPH, FRCPC: How do we define chronic graft-versus-host disease [GvHD]? First and foremost, chronic graft-versus-host disease is a clinical syndrome and is defined based on a clinical diagnosis. The name implies that the disease occurred late after transplant. But in fact, the temporal relationship of GvHD to transplant actually does not matter. You can have chronic GvHD that occurs early. It more typically occurs late. But the clinical constellation of symptoms that this patient has defines this as chronic GvHD with lichenoid changes in the mouth, the skin, the nail bed, and the joint involvement. All of those factors together make this chronic graft-versus-host disease.

Chronic GvHD occurs between 20% and 50% of all transplant recipients, depending on a number of factors, such as donor, stem cell source, and the type of GvHD prevention regimen that was used. Again, you make the diagnosis based on clinical factors. There are no real imaging tests that are required. However, I do recommend that pulmonary function tests are done on all patients at the time of initial diagnosis of chronic GvHD, so that you can exclude subclinical lung involvement. That’s very important because lung involvement can be subclinical until it gets fairly severe. We don’t biopsy organs for the most part unless there’s a clinical conundrum. Usually the presentation of the skin rash, or the erythema, or ulcers in the mouth are generally diagnostic enough. But in certain circumstances, we do biopsies when there is a clinical differential diagnosis.

Transcript edited for clarity.


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