Ebstein-Barr Virus+ Mucocutaneous Ulceration (EBVMCU) in transplant organ recipient.

Recently recognized lymphoproliferative disorder driven by latent EBV infection occurring with age related or iatrogenic immunosuppression often with Hodgkin like features. Epidemiological data about incidence are cumulating. Reported mostly from elderly (64-101yrs), but younger (18-81yrs) on immunosuppression (recipients of solid organ or allogenic bone marrow), HIV+ patients and patients with primary immunodeficiency are also affected. Immunosuppression seems to be a major risk factor (related to senescence and/or treatment with methotrexate, azathioprine, cyclosporine or TNF inhibitors).

It represents an early polyclonal EBV-driven LPD which may show molecular progression to cHL, analogous to post-transplant LPDs in which polymorphic B-cell proliferations may regress upon removal of immunosuppression or sometimes progress to lymphoma. Morphology may vary show Hodgkin-like, DLBCL-like or polymorphic post-transplant lymphoproliferative disorder picture.
Clonality studies showed IgH monoclonal in 40% and oligoclonal TCR rearrangements in 70%. Clinical picture show ulcerated, sharply circumscribed, indurated lesions in the oral mucosa (tonsils, tongue, palate, buccal mucosa), skin or gastrointestinal tract (esophagus, large bowel, rectum, perianal region) and also in genital mucosa. Usually there is no evidence of generalized lymphadenopathy, hepatosplenomegaly or bone marrow involvement. Most often there is good response to reduction of immunosuppression, but some patients may need rituximab, local radiotherapy or chemotherapy. The relapse or progression is seen only in few cases. Prognosis is generally superior to other EBV-driven immunodeficiency associated disorders.

Diagnosis of EBVMCU requires a combination of clinical, morphologic and parameters of immunophenotype. Differential diagnosis against monomorphic Post-transplant lymphoproliferative disorder (mPTLD) may be difficult and is based on clinical features.

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