BI19 Cutaneous post-transplant lymphoproliferative disorder in an immunosuppressed patient

Abstract

Abstract Post-transplant lymphoproliferative disorders (PTLDs) are a common form of de novo neoplasia developing in patients with immunosuppression following a transplant. The majority are associated with Epstein–Barr virus (EBV) infection. Cutaneous PTLD manifestations are rare and not well documented in literature. We present a case of systemic PTLD with secondary extensive cutaneous involvement 4 months after allogenic stem cell transplant for JAK2-mutated myelofibrosis. A 68-year-old man was admitted to the haematology department with fevers, diarrhoea, cytopenia, EBV viraemia and a nodular rash behind his ears. Biopsies from colonoscopy confirmed monomorphic PTLD with subsequent extensive ileocalceal lymphadenopathy area detected on computed tomography. Asymptomatic, large, hardened violaceous nodules on the head and neck worsened further. Skin biopsy showed an infiltrate of atypical lymphocytes in the dermis, composed predominantly of large lymphoid cells with visible prominent nucleoli. A limited immunohistochemical panel showed positivity for BCL-2 but not CD20. EBV-encoded RNA in situ hybridization was also positive. Features were compatible with cutaneous involvement by the known monomorphic PTLD. Unfortunately, the patient deteriorated with haemodynamic instability due to sepsis, despite two further infusions of rituximab, and was directed to the palliative team. He died soon after the discontinuation of all treatments. PTLD has a wide spectrum of clinical manifestations. It is classified into three nondestructive types: polymorphic, monomorphic and classic Hodgkin lymphoma type. It can have a poor prognosis so it should always be considered as a diagnosis in transplant patients. Monomorphic PTLDs tend to be more aggressive and rarely respond to immunosuppression reduction. On the contrary, primary cutaneous involvement is rare and seems to have a favourable outcome. Treatment depends on the severity of the disease, and both localized and systemic therapies have been used. Transplant patients should have long-term follow-up to diagnose and promptly treat possible complications.