Case Report: Donor derived Kaposi sarcoma following liver transplantation (2021)

Status: 
Ready to upload
Record number: 
2200
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Council of Europe, 2022: Risk assessment: Kaposi’s sarcoma, Merkel cell carcinoma and skin sarcoma are considered an unacceptable risk
Time to detection: 
5 months. Despite adjustment of immunosuppression the recipient died 9 months after liver transplant .
Alerting signals, symptoms, evidence of occurrence: 
Unspecific: acute kidney injury, fever, night sweats, maculopaular rash at different sites, B-symtoms, pancytopenia, respiratory symptoms, adenopathy, neck lump. Specific: contrast CT: pulmonary nodules, lymphadenopathy, pleural effusion. Biopsy of neck lump: medullary lymphoid tissue with prominent vascular structures, scattered lymphocytes & plasma and B-cells PET showed increased tracer uptake within multiple lymph nodes and the liver. In light of the prominence of vascular structures, HHV8 immunostain was performed and was positive, conclusively identifying the presence of Kaposi sarcoma. There was also evidence of a painless cutaneous Kaposi sarcoma within and overlying the post-transplant scar.
Demonstration of imputability or root cause: 
Donor was anti-HHV8 seropositive, recipient at liver-TX seronegative but NAT positive after 4.5 months with antibodies becoming detectable (primary HHV8 infection and seroconversion).
Imputability grade: 
2 Probable
Groups audience: 
Suggest new keywords: 
Malignancy
Reference attachment: 
Suggest references: 
Copeland MMM, Trainor J, Cash WJ, Braniff C. Fatal donor-derived Kaposi sarcoma following liver transplantation. BMJ Case Rep. 2021 Jun 22;14(6):e236061. doi: 10.1136/bcr-2020-236061. PMID: 34158319; PMCID: PMC8220532.
Note: 
Uploaded MN 5/13/22 First review CLFF 5/23/22 (note: I added from infection chapter of 2022 guide some sentence in the comment - if you are not happy with it please adjust or let me know what to do; may be helpful to contac Paolo Grossi) second review CLFF 21/05/2024
Expert comments for publication: 
Seroconversion of recipient with vireamia after receiving an organ form a seropositive donor may indicate donor derived infection. Unfortunately in donors with latent HHV8 infection transmission is for sure and development of complications such as Kaposi-Sarkom is possible. According to Council of Europe 2022 , Chapter 8 - infection: HHV 8 seroprevalence is estimated to be between 0 % and 5 % in North America, northern Europe and Asia; between 5 % and 20 % in the Mediterranean and Middle East; and > 50 % in some parts of Africa. In immunocompromised persons, fever, splenomegaly, lymphoid hyperplasia, pancytopaenia and occasionally rapid-onset Kaposi sarcoma have all been described in association with apparent primary KSHV infection, However, in immunocompromised transplant recipients, KSHV is more often associated with neoplastic diseases. Early identification of primary or reactivated infection offers the possibility of careful alteration of immunosuppression, where appropriate, or pre-emptive antiviral treatment; this is associated with more favourable outcomes when compared to late diagnosis of symptomatic disease. Available tests for donor screening are of inferior quality, so this is not recommented. Of note, universal screening of donors for KSHV is generally not necessary. However, since donor-derived primary KSHV infection may be associated with severe disease, screening of donors for KSHV anti-lytic and anti-latent antibodies is recommended for donors and recipients coming from areas with high prevalence (unfortunately until 2024 we have no appropriate test available in some countries). In cases of D+/R− mismatch, close monitoring of the recipient for KSHV-DNA in blood is recommended in order to identify infection early.