SESSION TITLE: Cancer Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Wednesday, October 29, 2014 at 11:00 AM - 12:15 PM
INTRODUCTION: Patients with rheumatological diseases are at risk of developing lymphoproliferative disorders (LPD) with immunosuppression. LPD as a complication of azathioprine use in dermatomyositis has never been reported.
CASE PRESENTATION: 67 year old woman with a history of dermatomyositis presented with complaints of fatigue, weight loss, and fever. She had taken azathioprine for 7 years for dermatomyositis, but no other medications. Chest CT showed multiple pulmonary nodules concerning for metastatic disease. She underwent bronchoscopy with transbronchial biopsy, and pathology showed Epstein-Barr Virus (EBV) driven lymphoproliferative disease. Despite aggressive medical management patient developed multi-organ failure with deterioration of her clinical status and subsequent death. Autopsy showed primary extra nodal EBV positive diffuse large B-cell lymphoma involving the lungs, kidneys, heart and gastric antrum.
DISCUSSION: Lymphoproliferative disorder (LPD), is a heterogeneous group of expanding, monoclonal or oligoclonal, lymphoid cells that occur in the setting of immune dysfunction. Primary extra nodal EBV-positive diffuse large B-cell lymphoma is a rare neoplasm mostly occurring as a complication of solid organ transplant, allogenic hematopoetic bone marrow transplant and immunosuppression for Crohn’s Disease. LPD development in dermatomyositis is due to many variables including type of immunosuppression, duration, and severity of disease. Previous reports have identified LPD in dermatomyositis with patients using methotrexate, but azathioprine has not been associated with LPD in dermatomyositis. Treatment focuses on three goals: restoration of recipient’s immunity (to limit EBV infection), elimination of EBV and removal of neoplastic B cells. Initially, cessation or reducing immunosuppression is used to help restore patient’s immunity. The second stage of treatment requires control of EBV with use of acyclovir or gancyclovir. The last stage depends on the location and aggressiveness of the lymphoid proliferation. Surgery, radiotherapy and chemotherapy with Rituximab may be of utility if there is no therapeutic response to the previous treatment modalities.
CONCLUSIONS: We present the first published case of EBV driven lymphoproliferative disorder with azathioprine use in dermatomyositis.
Reference #1: Kojima, K., Motoori, T. Benign, atypical and malignant lymphoproliferative disorders in rheumatoid arthritis. Biomedicine and Pharmacotherapy. (2006) 60: 663-672
DISCLOSURE: The following authors have nothing to disclose: Saba Hamiduzzaman, Richard Moreahead
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