According to the World Health Organization, PTLD has been classified as:
- an early lesion: reactive plasmacytic hyperplasia and infectious mononucleosis‐like;
- PTLD, polymorphic: polyclonal and monoclonal;
- PTLD, monomorphic: B cell lymphomas and T cell lymphomas; and
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 1).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.
Table 1Review of T cell lymphomas affecting the heart after organ transplantation
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.