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An Immune Suppression-associated EBV-positive Anaplastic Large Cell Lymphoma With a BRAF V600E Mutation

Gru, Alejandro A., MD*; Williams, Eli, PhD; Junkins-Hopkins, Jacqueline M., MD

The American Journal of Surgical Pathology: January 2019 - Volume 43 - Issue 1 - p 140–146
doi: 10.1097/PAS.0000000000001174
Case Report

Iatrogenic lymphoproliferative disorders have been described in patients receiving immunosuppressive/immunomodulatory agents outside the transplantation setting. Novel biological agents such as TNF-α blockers and JAK-inhibitors have also proven to be effective in many disorders including rheumatoid arthritis, inflammatory bowel disease (ulcerative colitis and Crohn disease), psoriasis, and others. A significant dilemma exists in those lymphoproliferative disorders associated with immunosuppressants and rheumatologic conditions, that relies on whether the association of the process is with the medication or the underlying autoimmune condition. In the current case report, we describe an extraordinary case of Epstein-Barr virus–positive anaplastic large cell lymphoma, in association with rheumatoid arthritis and the use of JAK-inhibitors. Comprehensive molecular testing (fluorescence in situ hybridization, OncoScan microarray, pyrosequencing) was done comparing sequential biopsies in this patient from skin and lung, which revealed a driving mutation in the BRAF V600E gene, a crucial finding, given the potential use of targeted therapy in this pathway.

Departments of *Pathology & Dermatology

Pathology, University of Virginia, Charlottesville, VA

Dermatopathology, Geisinger Medical Laboratories, Danville, PA

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Alejandro A. Gru, MD, Department of Pathology & Dermatology, University of Virginia, Charlottesville, VA 22908 (e-mail:

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