Epstein-Barr virus–associated lymphoproliferative disease after long-standing cyclosporine therapy for psoriasis: A case of spontaneous regression

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Posttransplant lymphoproliferative disorders are lymphoid proliferations or lymphomas, usually associated with Epstein-Barr virus infection, that develop as the consequence of immunodepression. These disorders usually affect patients receiving high doses of cyclosporine in the context of bone marrow or organ transplantations. Posttransplant lymphoproliferative disorders can regress when cyclosporine is discontinued. Such lymphoproliferations rarely occur for patients receiving low-dose cyclosporine treatments for autoimmune disorders. In the following report, we describe a patient with psoriasis vulgaris treated with long-term low-dose cyclosporine who developed an acute Epstein-Barr virus–associated clonal lymphoproliferative disorder associated with hemophagocytic syndrome. This lymphoproliferative disorder resembling classic posttransplant lymphoproliferative disorder regressed when cyclosporine was discontinued.

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Case report

A 52-year-old white man was enrolled in 1986 in a cyclosporine therapy trial for psoriasis vulgaris. Methotrexate has been given before for 6 months but was discontinued because of the occurrence of hepatic side effects. Cyclosporine given at the level of 3 mg/kg/d resulted in a complete disappearance of dermatologic lesions. He was referred to our hospital in May 2001 for a 3-week fever with sweats, marked asthenia, and weight loss. Physical examination disclosed splenomegaly and axillary,

Results

The histologic examination of the lymph node biopsy specimen disclosed an effacement of the nodal architecture (Fig 1, A) by a polymorphic population of lymphoid cells consisting of atypical medium lymphocytes with plasmocytic differentiation admixed with scattered large cells displaying immunoblastic characteristics, a few plasmocytes, and small lymphocytes (Fig 1, B). Mitosis and apoptotic figures were numerous. Immunohistochemistry revealed the B-cell phenotype of the atypical medium cells

Discussion

EBV is associated with Hodgkin's disease, especially in patients with HIV infection, and non-Hodgkin's lymphomas.3 It is, however, also associated with a spectrum of lymphoproliferative disorders in patients with congenital, acquired (namely HIV infection), or iatrogenic immunodeficiencies especially in transplant recipients.4 The association between EBV infection and lymphoproliferative disorders in transplant recipients—posttransplant lymphoproliferative disorders (PTLD)—was first noted in

Conclusion

To our knowledge regressive EBV-associated lymphoproliferative disorders with aggressive presentation during low-dose cyclosporine therapy given for psoriasis has not been described before. Our observation emphasizes two points. First, the 15-year delay between treatment initiation, and the occurrence of lymphoproliferation observed in our patient, suggests that low-dose cyclosporine treatment entails a risk of PTLD that persists as long as the immunosuppressive drug is given. Second, although

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This supplement is made possible through the generous support of Stiefel Laboratories for the American Academy of Dermatology.

Funding sources: None.

Conflicts of interest: None identified.

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