Disseminated Pulmonary Kaposi Sarcoma After Lung Transplantation: The Increased Risk Donor

Introduction

Kaposi sarcoma (KS) is a rare complication after lung transplantation (LTx). It is caused by human herpes virus 8 (HHV-8). We report a case of primary disseminated pulmonary KS after LTx from an increased risk donor.

Case Report

A 66-year-old male with end-stage COPD underwent a bilateral LTx from an increased risk donor with positive HBsAb, HBcAb and Lues Ab, in November 2020. Apart from Valganciclovir (vGCV) and co-trimoxazole, entecavir and benzylpenicillin were given as prophylaxis. Routine immunosuppression included basiliximab, tacrolimus, mycophenolate mofetil (MMF) and prednisolone. vGCV prophylaxis was discontinued at 3 months (CMV-positive donor and recipient). The patient was treated effectively for acute rejection with pulse methylprednisolone in February 2021. Unfortunately, he was readmitted with dyspnea and lung function decline in April 2021. CT-scan showed bilateral consolidations and pleural thickening and the PET-scan showed an intense uptake in both lungs and pleura. Transthoracic needle biopsy showed HHV-8 positive spindle cell lesions. Subsequently, our patient developed skin lesions on his chest and in his mouth compatible with KS (biopsy-proven). In retrospect, the recipient was HHV-8 seronegative before LTx. Therefore, the diagnosis primary HHV-8 infection leading to disseminated pulmonary Kaposi sarcoma was made. Which appeared after cessation of the vGCV prophylaxis. Valganciclovir was initiated and immunosuppression was adjusted: MMF discontinued and tacrolimus switched to sirolimus (target through levels 15 µg/l). Nevertheless, KS was progressive and liposomal doxorubicin 20mg/m2 was started. Our patient deteriorated and was transferred to the ICU for intubation. Bronchoscopy revealed brown endobronchial lesions compatible with KS. The effect of the treatment could not be assessed, because the patient decided to refuse further treatment. He died soon after his decision was made.

Summary

Scarcity of donor organs led to the acceptance of an increased risk donor. Prophylaxis against hepatitis B and Lues was given, but after discontinuation of vGCV our patient developed a primary HHV-8 infection leading to disseminated KS and ultimately the death of our patient. HHV-8 testing of donor serum and lifelong prophylaxis might have prevented this clinical course and should be considered in recipients of increased risk donors.