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Once in a Blue...liver? A Rare Case of Delayed Gra ...
Once in a Blue...liver? A Rare Case of Delayed Graft Versus Host Disease After Liver Transplant
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Pdf Summary
This case describes a rare, fatal episode of graft-versus-host disease (GVHD) occurring three years after orthotopic liver transplantation for cirrhosis due to nonalcoholic fatty liver disease. The 41-year-old woman initially presented with three months of diarrhea and odynophagia, followed by one week of fevers. Her evaluation showed new leukopenia, but extensive infectious testing, CT imaging, and liver Doppler studies were unrevealing.<br /><br />During hospitalization, she developed classic GVHD features including glossitis, mucositis, and a painful erythematous rash involving the trunk and palms. Skin biopsy showed vacuolar interface changes and keratinocyte degeneration, while sigmoid colon biopsy demonstrated crypt apoptotic activity and immune cell depletion. Viral stains were negative, and the pathology was consistent with GVHD. Despite high-dose steroids and broad-spectrum antimicrobials, she worsened and died of multiorgan failure and sepsis.<br /><br />The report emphasizes that GVHD is a rare but highly lethal complication of solid organ transplantation, with mortality greater than 80%. It most commonly presents with fever, pancytopenia or leukopenia, maculopapular rash, mucositis, and diarrhea. Although symptoms usually occur within 2 to 8 weeks after transplant, late-onset cases can occur years later, as in this patient.<br /><br />Diagnosis is based on clinical suspicion supported by histopathology, which typically shows epidermal vacuolar change and keratinocyte apoptosis in skin, and crypt epithelial apoptosis in the intestine. Chimerism testing demonstrating donor lymphocytes in recipient tissues is diagnostic when available. There is no standard treatment; reported approaches include either reducing immunosuppression to encourage recipient immunity or increasing immunosuppression with corticosteroids and other agents to suppress donor immune cells.<br /><br />The key lesson is to consider GVHD in any solid organ transplant recipient with fever, rash, and diarrhea, since early recognition may improve outcomes.
Asset Subtitle
Courtney Mullins
Meta Tag
Author List
Alina Iuga, Courtney Mullins, Darryl Kalil, Evan Raff, Sam Wu
Category
Clinical Vignettes
Concept
Graft-versus-Host Disease
Concept
Liver Transplantation
Concept
Crypt Epithelial Apoptosis
Concept
Organ transplantation
Concept
Pancytopenia
Distinguished
Non-Finalist
Presenter Organization
UNC Hospitals
Presenting Author
Courtney Mullins
Track
Adult
Keywords
graft-versus-host disease
liver transplantation
solid organ transplant
late-onset GVHD
diarrhea
mucositis
skin biopsy
crypt apoptosis
leukopenia
multiorgan failure
Graft-versus-Host Disease
Liver Transplantation
Crypt Epithelial Apoptosis
Organ transplantation
Pancytopenia
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