A 34-year-old woman presents to the ED with several days of worsening abdominal pain, anorexia, nausea, and copious diarrhea. Three months ago, she had underwent matched unrelated-donor allogeneic stem cell transplant for acute lymphocytic leukemia (ALL). Examination reveals an:
She is given 30 mL/kg IVF and SBP remains in the 80s, prompting ICU admission. Laboratory test results are notable for:
Which of the following statements about the most likely diagnosis is true?A. Liver biopsy should be a high priority
Correct Answer: B
The most likely diagnosis here is acute graft-versus-host disease (GVHD), a multisystem inflammatory attack on the host’s tissues by the grafted immune system. Risk factors include the degree of HLA mismatch and the prophylactic regimen employed posttransplant. The pathogenesis involves T lymphocytes primarily, though neutrophils and activated macrophages contribute to a lesser extent. The most common sites of acute GVHD are the GI tract, skin, and liver, and presentation typically occurs within the first few months of transplant. A grading system exists based on the extent of skin involvement, severity of diarrhea, and bilirubin elevations, with higher grades associated with reduced survival. Diagnosis may be made on clinical grounds along with the classic rash of GVHD (maculopapular, involving palms/soles), though biopsy of skin or the distal GI tract (typically not liver) may be performed in cases where infection is higher in the differential diagnosis. Although topical steroids may be effective in GVHD limited to the skin, for any signs of systemic involvement systemic glucocorticoids are indicated as first line treatment. Notably, intense immunosuppression over a prolonged period exposes the host to many risks, including a reduction in the graft-versus-malignancy effect that helps allogeneic stem cell recipients maintain long-term remissions.