Initial spontaneous remission of posttransplantation Epstein Barr virus-related B-cell lymphoproliferative disorder of the skin in a renal transplant recipient - Case report and review of the literature on cutaneous B-cell posttransplantation lymphoproliferative disease

Primary cutaneous posttransplantation B-cell lymphoproliferative disorder is rare. The few previously reported patients were all treated with surgery, radiotherapy, or lowering of immunosuppression. We describe a 65-year-old woman presenting with an intermammary skin ulcer 21 years after renal transplantation, proving on biopsy to be an Epstein Barr virus (EBV)related posttransplantation B-cell lymphoproliferative disorder. A few weeks later, without further clinical intervention, the skin ulcer showed complete clinical regression. Hematologic staging evaluation showed no evidence of extracutaneous involvement. Despite continuation of immunosuppression, the patient stayed free of disease until 18 months after initial diagnosis, when she developed a progressive hemiparesis and died of acute myocardial infarction. At autopsy, a recurrent B-cell posttransplantation lymphoproliferative disorder in the left side of the thalamus region (measuring 1 x 0.8 cm) was established. The long interval between the primary cutaneous lesion and the localized brain recurrence supports primary skin posttransplantation lymphoproliferative disorder, especially because the patient was not treated for her posttransplantation lymphoproliferative disorder. Review of the literature on primary cutaneous posttransplantation B-cell lymphoproliferative disorder and this case gives the impression that cutaneous posttransplantation B-cell lymphoproliferative disorders of B-cell lineage behave in a more benign manner than identical lesions arising extracutaneously. Because of the rare occurrence of posttransplantation B-cell lymphoproliferative disorder primarily involving the skin, extracutaneous origin should be excluded. If B-cell lineage can be established, EBV is present, alterations in oncogenes or tumor suppressor genes associated with malignant lymphoma are absent, and bcl-6 gene mutation associated with progression is absent, initially aggressive treatment might be avoided. However, long-term clinical follow-up with prolonged maintenance therapy (reduction of immunosuppression or antiviral therapy) for prevention of recurrent Posttransplantation lymphoproliferative disorder seems indicated, as is demonstrated by the case reported in the current study.