Acute and chronic graft-versus-host disease (GVHD) are multisystem disorders that are common complications of allogeneic hematopoietic cell transplant (HCT). GVHD occurs when immune cells transplanted from a non-identical donor (the graft) recognize the transplant recipient (the host) as foreign, thereby initiating an immune reaction that causes disease in the transplant recipient.

Definitions

• acute vs chronic: <100 vs >100 days.
• However, also differentiate based on clinical presentation that can cross the 100-day threshold. Therefore the current National Institutes of Health (NIH) consensus criteria use clinical findings, rather than a set time period, to differentiate between acute and chronic GVHD.

Epidemiology

The exact incidence of acute GVHD after allogeneic HCT is unknown. Clinically significant acute GVHD occurs in patients who receive an allogeneic hematopoietic cell transplant (HCT) despite intensive prophylaxis with immunosuppressive agents.

Risk Factors

Acute GVHD

Major

•Degree of HLA disparity (HLA mismatch or unrelated donor) •GVHD prophylaxis regimen

Minor

•Donor and recipient sex disparity (female donor to male recipient) •Intensity of the transplant conditioning regimen

Clinical Manifestations

The skin, gastrointestinal tract, and liver are the principal target organs in patients with acute GVHD:

• Skin - first clinical manifestation in most patient is a maculopapular rash initially involving the nape of the neck, ears, shoulders, palms and soles. Described as a sunburn, can be pruritic or painful. From there, it typically spreads to the whole skin and can become confluent
  • in severe cases can form bullous lesions with toxic epidermal necrolysis mimicking SJS
  • degree of skin involvement is graded depending on the degree and severity of lesions:
    • Stage 1 – Maculopapular rash over <25 percent of body area
    • Stage 2 – Maculopapular rash over 25 to 50 percent of body area
    • Stage 3 – Generalized erythroderma
    • Stage 4 – Generalized erythroderma with bullous formation, often with desquamation
• gastrointestinal tract which can involve the upper and lower tracts. Commonly presents as diarrhea, abdominal pain, nausea, vomiting, anorexia.
  • diagnosis is via biopsy, most of the time rectal biopsy can confirm this
  • degree of GI involvement is graded as such:
    • Stage 1 – Diarrhea 500 to 1000 mL/day
    • Stage 2 – Diarrhea 1000 to 1500 mL/day
    • Stage 3 – Diarrhea 1500 to 2000 mL/day
    • Stage 4 – Diarrhea >2000 mL/day or pain or ileus