Extranodal Marginal Zone Lymphoma of the Lung: Evolution from an Underlying Reactive Lymphoproliferative Disorder
- *Corresponding Author:
- Colleen Beatty
Post-Sophomore Fellow, Department of Pathology
West Virginia University School of Medicine, USA
E-mail: [email protected]
Received Date: December 22, 2014; Accepted Date: January 28, 2015; Published Date: February 03, 2015
Citation: Rubenstein NJ, Beatty C, Kinkade Z, Bryan C, Paul Hogg J, et al. (2015) Extranodal Marginal Zone Lymphoma of the Lung: Evolution from an Underlying Reactive Lymphoproliferative Disorder. J Clin Exp Pathol 5:208. doi: 10.4172/2161-0681.1000208
Copyright: © 2015 Rubenstein JN, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Extranodal Marginal Zone Lymphoma of mucosa-associated lymphoid tissue (ENMZL) is a problematic and sometimes controversial diagnosis. While commonly seen in the stomach in the setting of chronic Helicobacter pylori infection, other extranodal sites, such as the lung, may also present with disease. ENMZL is clinically and morphologically heterogeneous; however, regardless of presentation, the etiology lies in the accumulation of lymphoid tissue in non-traditional sites. This phenomenon is typically secondary to an underlying inflammatory stimulus such as chronic infection or autoimmune states. The current case report details the clinical history of a patient with Sjögren syndrome over a four year period who eventually developed ENMZL. The patient initially presented with an atypical, but polyclonal, lymphoproliferative process diagnosed as lymphocytic interstitial pneumonia. Over time, the patient showed evolution to a monoclonal process with associated radiologic progression of disease. This evolution manifested as a dense lymphoid infiltrate with prominent plasmacytic differentiation and the development of a lung mass radiologically. This case contributes to the growing body of knowledge that suggests ENMZL lies along a biological spectrum of lymphoproliferative disorders whereby a benign, reactive process may eventually undergo malignant transformation. This evolution likely represents the acquisition of genetic abnormalities that allow autonomous proliferation in the absence of the initial immune stimulus. In practice, determining when this event occurs and, thus, distinguishing between reactive and neoplastic disorders within this spectrum may be difficult as no single clinicopathologic feature may be present to establish the diagnosis. This case further illustrates the importance of correlating the clinical, radiologic and pathologic data to evaluate patients with atypical pulmonary lymphoproliferative disorders and to allow the optimal management of their disease.