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Post‐transplantation lymphoproliferative disorder (PTLD) has been recognised as a significant complication affecting 2% of all organ allograft recipients.1 The incidence of PTLD has increased in recent years because of the steady increase in the number of organs transplanted.1 The early diagnosis of PTLD is complicated by its wide spectrum of clinical presentations and the difficulty in distinguishing it from other more frequent complications—allograft rejection and opportunistic infection.
A 20‐year‐old man presented with fever, headache and cough. He was a recipient of live‐related renal allograft 7 years previously, at which time he started receiving standard immunosuppressive treatment. He was found to be positive for hepatitis B s antigen and e antigen. Other systemic examinations were normal.
Laboratory tests revealed bicytopenia. Despite appropriate antibiotic coverage, the patient sustained cardiac arrest.
A complete autopsy was performed after obtaining informed consent. A grey–white fish flesh mass measuring 2×1.5×1.5 cm was noted in the pericardium (fig 1A1A).). Microscopically, the mass comprised a monomorphic population of tumour cells possessing oval and indented nuclei with prominent nucleoli (fig 1B1B).). The cells infiltrated the myocardium, encroaching up to the inner surface of the endocardium. Vascular dissemination of the tumour to capillaries of the lung, sinusoids of the spleen and liver, choroid plexus (fig 1C1C)) and kidney (fig 1D1D)) was noted. Immunostaining for CD45, CD20, CD3, CD5, epithelial membrane antigen, anaplastic lymphoma kinase 1 protein and Epstein–Barr virus‐related latent membrane protein‐1 antigen (Dako, Glostrup, Denmark) was performed. The cells showed positivity for leucocyte common antigen, CD3 (fig 1E1E)) and CD5 only. Bone marrow revealed erythroid hyperplasia and haemophagocytosis.
According to the World Health Organization, PTLD has been classified as:
T cell PTLD was first described in 1987,2 and since then >70 cases have been described. The incidence of T cell lymphomas after solid organ transplant is approximately 14%.3 In comparison with B cell PTLD, T cell PTLD tends to occur later, is less likely to involve the allograft and is less frequently associated with Epstein–Barr virus infection (33%). T cell PTLD is also different in that polyclonality is seen in a lower percentage.3 Only one case in the literature shows human T cell lymphocyte virus‐1 genome.4
Our case of PTLD is unusual for several reasons: its primary pericardial involvement, T cell phenotype, pattern of spread and the late onset. The literature search revealed that the involvement of heart and pericardium was seen in only three cases in the previously recorded T cell PTLDs (table 11).5,6,7 The index case is a 30‐year man who developed T cell PTLD 7 years after renal transplantation. This is in accordance with T cell PTLD, which usually is of late onset. The reasons for bicytopenia in the index case are anaemia of a chronic disorder, chemotherapy, megaloblastosis and haemophagocytosis. Kaplan et al8 have described haemophagocytosis associated with post‐transplantation T cell lymphoma arising in the vulva. The direct cause‐and‐effect relationship of haemophagocytosis and T cell lymphoma is unclear. T cell PTLD has a poor prognosis when it is monoclonal and/or possesses multiorgan involvement, as seen in the index case.
The mode of spread of lymphoma cells to various visceral organs and the brain is interesting in our case. This seems to be vascular in nature—that is, through the systemic circulation.
This case exemplifies that post‐transplantation lymphoma can develop in various unusual sites with protean clinical manifestations. In a post‐transplant patient who presents, a long time after transplantation, with serous cavity effusions or pericarditis, the possibility of lymphoproliferative disease apart from the opportunistic infections should be considered and investigated, so that the immunosuppression can be reduced or the patient treated with specific therapy such as interferon α or monoclonal antibodies.
Competing interests: None declared.