Cutaneous presentation of post‐renal transplant lymphoproliferative disorder: a series of four cases

Abstract

We report detailed histological and molecular characteristics of four post transplant lymphoproliferative disorders (PTLD) presenting in the skin of renal transplant patients, and their clinical outcome. Three had B-cell lymphomas (cases 1-3), and one had a T-cell lymphoma (case 4). All B-cell lymphomas showed Epstein-Barr virus (EBV) by immunohistochemistry (IHC) or in situ hybridization (ISH). Cases 1 and 2 were large cell lymphomas, and case 3 a plasmacytoma. Case 1 showed light chain restriction and heavy chain gene rearrangement by polymerase chain reaction (PCR). The patient was then diagnosed with an abdominal lymphoma and died of sepsis. Case 2 had no recoverable DNA. Case 3 had a plasmacytoma that showed monoclonal light chain restriction on IHC and an oligoclonal heavy chain rearrangement by PCR. In cases 2 and 3, the lesions regressed following reduction of immunosuppression, and died 1.5 and 8 years later from unrelated medical causes. Case 4 was a CD 30+ anaplastic large T-cell lymphoma with no EBV detected by IHC, ISH and PCR, and died of heart failure 2 years later. Cutaneous manifestations of PTLD are rare, show wide array of clinical and pathological features, and generally have a favorable prognosis. EBV appears to be associated only with B-cell cutaneous lymphomas.

HIV‐associated primary cutaneous anaplastic large cell lymphoma: a clinicopathological subset with more aggressive behavior? Case report and review of the literature

“…By contrast, a ‘pyogenic’ picture with numerous neutrophils intermingled with anaplastic cells (a frequent finding in HIV‐associated systemic ALCL) was noted in only two of HIV‐associated primary cutaneous ALCL patients. Such neutrophil‐rich picture has been tentatively attributed to loss of immune system competence in sporadic cases of primary cutaneous ALCL; this inflammatory pattern, however, appears to be an unspecific finding in primary cutaneous ALCL, being detectable in up to 32% of cases in the general population . Of note, IHC positivity for EMA (a rare feature in conventional primary cutaneous ALCL, with only weak/focal expression in less than 30% of cases) was described in up to 75% of tested cases in our review; such finding is rather frequent (approximately 70% of cases) in HIV‐associated systemic ALCL, possibly being a marker of malignant course in combination with lack of ALK1 expression .…”

“…Multiple characteristics are shared between recipients of allogeneic transplants and HIV‐infected individuals with regard to lymphomagenesis, including increased frequency of incidental NHL, predominance of B‐cell phenotype, tropism for extranodal locations and more aggressive behavior . Allogeneic transplantation also increases the risk of primary cutaneous T‐cell lymphomas, with primary cutaneous ALCL being regarded as the most frequent among primary cutaneous lymphoproliferative disorders of T‐cell phenotype . Interestingly, a multitude of clinicopathological aspects are shared by post‐transplant primary cutaneous ALCL and HIV‐associated primary cutaneous ALCL, so that drawing a parallel between these primary cutaneous ALCL presentations might not be unreasonable (Table ) .…”

“…Viral agents are not regarded as primary pathogenetic factors in HIV associated and allogeneic transplant‐associated peripheral T‐cell lymphomas, including systemic ALCL . For instance, frequencies of EBV detection (showed by means of latent membrane protein‐1 IHC staining or EBER‐1 in situ hybridization) are below 30% of reported cases, with controversies regarding the nature of infected cells (neoplastic or reactive) and the prognostic significance of this finding; indeed, it is possible that any association with EBV is a coincidental occurrence favored by higher rates of productive infection among immunocompromised patients . Importantly, evaluation for EBV infection in our patient yielded negative results.…”

Epstein–Barr Virus in Cutaneous Pathology

“…250,268,269 Primary cutaneous PTLD has also been reported in the literature, 58,255,[270][271][272][273][274][275][276][277][278][279][280][281][282][283] including B-cell and T-cell lymphomas. 250,268,269 Primary cutaneous PTLD has also been reported in the literature, 58,255,[270][271][272][273][274][275][276][277][278][279][280][281][282][283] including B-cell and T-cell lymphomas.…”

Malignancies Confined to Disused Arteriovenous Fistulae in Renal Transplant Patients: An Important Differential Diagnosis

“…The more intense the immunosuppression used, especially from high doses of anti-T cell immunosuppressive agents, the higher the incidence of PTLD and the earlier it occurs, hence most EBV-related PTLD is in the first year post-transplant. There are several case reports of cutaneous PTLD [16]. Cutaneous presentations are variable and can range from erythematous plaques to ulcerated nodules, occurring in isolation or more generalized.…”