SESSION TYPE: Cancer Cases III
PRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM
INTRODUCTION: Vasculitides are associated with malignancies, as a manifestation or as a paraneoplastic syndrome. However, patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) developing malignancies is rarely reported in literature. We report a case of a patient with Microscopic Polyangiitis (MPA) who developed peripheral T-cell lymphoma.
CASE PRESENTATION: A 56 year-old caucasian female with MPA presented with a non-productive cough and exertional dyspnea worsening over few weeks. She was diagnosed with MPA, three years prior to this presentation when she presented with pulmonary renal syndrome. A renal biopsy was diagnostic of pauci immune glomerulonephritis. She had positive perinuclear ANCA (p-ANCA) and Myeloperoxidase (MPO) antibodies. She required plasmapheresis and induction with cyclophosphamide and rituximab. She was subsequently switched to mycophenolate and prednisone, and later maintained on mycophenolate 500 mg BID. She also had an ischemic stroke with right hemiplegia. During this presentation she denied any hemoptysis, rash or hematuria. A chest radiograph showed bilateral patchy consolidation and chest CT scan revealed bilateral diffuse nodular opacities and patchy consolidation. A broncho-alveolar lavage was performed to rule out infections and diffuse alveolar hemorrhage. She became febrile during her stay, with worsening respiratory failure requiring initiation of non-invasive mechanical ventilation. Her antimicrobial coverage was broadened to include antifungal agents. ANCA titers were unremarkable. A video assisted thoracotomy biopsy of the right lung was performed along with excision of a mediastinal lymph node. Biopsy of both the lung tissue and lymph node revealed proliferation of lymphoid cells with immunohistochemical stains consistent with peripheral T-Cell lymphoma. EBV immunostains were negative. A PET scan showed hypermetabolic activity in both lung fields and in lymph nodes. No chemotherapy was recommended due to her poor functional status and patient elected for home hospice.
DISCUSSION: Our case is a rare malignancy of EBV negative peripheral T-cell lymphoma. Studies have reported varying incidence of lymphomas in AVA. Other commonly reported malignancies in AAV patients are non-melanoma skin cancers, leukemias, and bladder cancer. Immunosuppressant therapy, which is the main stay of treatment in this group of patients, increases the risk, the mechanisms being poorly understood.
CONCLUSIONS: Lymphoma should be a part of differential diagnoses in patients with AVA with nodular lung infiltrates and negative work up for vasculitis flare up, and infections.
1) Heijl C, Harper L, Flossmann O et al. Incidence of malignancy in patients treated for antineutrophil cytoplasm antibody-associated vasculitis: follow-up data from European Vasculitis Study Group clinical trials. Ann Rheum Dis.2011 Aug; 70(8):1415-21.
DISCLOSURE: The following authors have nothing to disclose: Mohammad Siddiqui, Khalid Mohammad, Sabah Saleem, Manish Joshi
No Product/Research Disclosure InformationUniversity of Arkansas for Medical Sciences, Little Rock, AR