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Case of the Month

University of Cincinnati Medical Center

Pathology Department

Anatomic Pathology

September Case of the Month

Clinical Presentation:

A 38-year-old woman with ESRD secondary to type 1 diabetes mellitus received an SKP transplant. Fever, chills, abdominal pain, and diarrhea developed 24 days post-transplant. An initial work up for infectious causes was unrevealing. Chimeric studies revealed 23% donor T cell numbers. GI biopsy findings were compatible with GvHD. She was treated with intravenous steroids, ruxolitinib, and ECP. Despite interventions, chimeric studies showed a rising proportion of donor T-cells and the patient developed a diffuse rash. She was transferred to an outside hospital for consideration of a bone marrow transplant but was deemed ineligible secondary to multiple opportunistic infections. The patient developed persistent bacteremia with multi-drug resistance and Epstein Barr viremia, with rapid clinic decline thereafter.

Histopathologic examination:

 September Case of the Month_GI Biopsy_Image1

September Case of the Month_GI Biopsy_Image2

Ancillary studies: Chimerism studies performed:

 September Case of the Month_Figure 1 & 2

Diagnosis:

Graft-versus-Host Disease (following pancreas-kidney transplant).

Discussion:

This case illustrates the disease progression of GvHD following SKP transplant. Graft-versus-host disease (GvHD) is an uncommon but often fatal complication following solid organ transplantation. GvHD develops when donor lymphocytes immunologically attack host tissue in immunosuppressed recipients.

 Commonly reported risk factors include age > 50 years old, donor-recipient age difference more than 20 years, younger donor age, any HLA class I match, and glucose intolerance. This case did have an age difference greater than 20 years, younger donor age, significant HLA matching, and glucose intolerance factors at play. Multiple treatment modalities were implemented for clinical suspicion of GvHD. Biopsy findings and T cell chimerism studies were used to guide management and treatment course. It is unknown if alternate or earlier administration of therapy may have been beneficial. There is no consensus approach to treat solid organ GvHD. Identification of these rare cases and detailed review of treatments and outcomes are needed to develop optimized treatment protocols for this commonly fatal disease.

References:

1.   Smith DM, Agura E, Netto G, et al. Liver transplant-associated graft-versus-host disease. Transplantation 2003; 75: 118-126. 2003/01/25. DOI: 10.1097/00007890-200301150-00022.

2.   Schulman JM, Yoon C, Schwarz J, et al. Absence of peripheral blood chimerism in graft-vs-host disease following orthotopic liver transplantation: case report and review of the literature. Int J Dermatol 2014; 53: e492-498. 2014/01/01. DOI: 10.1111/ijd.12149.

3.   Murali AR, Chandra S, Stewart Z, et al. Graft Versus Host Disease After Liver Transplantation in Adults: A Case series, Review of Literature, and an Approach to Management. Transplantation 2016; 100: 2661-2670. 2016/08/09. DOI: 10.1097/TP.0000000000001406

 

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Department of
Pathology and Laboratory Medicine

UC Health University Hospital
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Phone: (513) 584-7284
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Email: pathology@uc.edu