Epstein Barr virus-associated lymphoproliferative-disorders primarily involving the skin.
In cases of solid organ or bone marrow transplantation, up to 2 to 10% of patients may develop lymphoproliferative disorders (LPD), often induced by Epstein-Barr virus (EBV). Despite a morphology mimicking malignant lymphoma, in some cases the lesions will disappear completely after the degree of immunosuppression is lowered. Lately, similar processes have been described in non-transplant, immunosuppressed patients. A SNOMED search was performed on the database of three hospitals between 1990 and 1997, to identify patients with immunosuppression-related lymphoproliferative disorders (IR-LPD) involving primarily the skin. Two patients were identified. One was 2 years after kidney transplantation, and the other was being treated with methotrexate for dermatomyositis. In both biopsies, there was a diffuse perivascular proliferation of large lymphocytes with ample cytoplasm and pleomorphic nuclei, associated with extensive dermal and subcutaneous necrosis. Immunohistochemical studies revealed expression of CD20, CD45RO, CD43, CD30, EBV-LMP1, and EBV-NA2 by the atypical lymphocytes in both cases and, in one case, of the EBV-transcriptional replication activation protein. In both cases the lesions completely disappeared and have not recurred. Primary involvement of the skin by IR-LPD is very rare. Based on our results, it is possible that some of these cases in the skin contain EBV and co-express CD30 and T- and B-cell markers. The diagnosis of IR-LPD should be considered in cutaneous lymphoid proliferations in immunosuppressed patients. Before rendering an unequivocal diagnosis of malignant lymphoma, reduction of immunosuppression and follow-up of 4-8 weeks should be considered.
Department of Pathology, Duke University Medical Center, Durham, North Carolina 27710, USA.
C
C
Chai
W L
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White
C R
CR
Shea
V G
VG
Prieto
D000328:Adult; D015703:Antigens, CD / Q000096:biosynthesis; D019309:Epstein-Barr Virus Nuclear Antigens / Q000096:biosynthesis; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D007150:Immunohistochemistry; D007165:Immunosuppression Therapy / Q000009:adverse effects; D008214:Lymphocytes / Q000378:metabolism / Q000473:pathology / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000378:metabolism / Q000473:pathology / Q000821:virology*; D008297:Male; D008875:Middle Aged; D012871:Skin Diseases / Q000378:metabolism / Q000473:pathology / Q000821:virology*; D014763:Viral Matrix Proteins / Q000096:biosynthesis; D014775:Virus Activation
D015703:Antigens, CD; C037662:EBV-associated membrane antigen, Epstein-Barr virus; D019309:Epstein-Barr Virus Nuclear Antigens; D014763:Viral Matrix Proteins