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Monoclonal Gammopathy as the Presenting Manifestation of Primary Pulmonary Lymphoma FREE TO VIEW

Sujith Cherian, MD; Subhraleena Das, MD; Shakuntala Mauzo, MD; Amanda Tchakarov, MD; Rahat Hussain, MD; Robert Lodato, MD
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University of Texas Health Science Center at Houston, Houston, TX

Chest. 2013;144(4_MeetingAbstracts):615A. doi:10.1378/chest.1705006
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SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Wednesday, October 30, 2013 at 11:30 AM - 12:30 PM

INTRODUCTION: Primary pulmonary lymphoma (PPL) accounts for around 0.5-1% of all lung neoplasms and < 1% of all cases of Non Hodgkins Lymphoma (NHL)

CASE PRESENTATION: A 55 year-old male was admitted with increasing exertional dyspnea of one year duration. Past history revealed multiple admissions for right lower lobe pneumonia, treated with antibiotics. Laboratory workup revealed white count of 5,500 cells/ µL, calcium level of 8.5 mg/dL, and an increased serum creatinine at 2.7mg/ dL . Chest X-ray and CT scan showed a persistent right lower lobe infiltrate. Bronchoscopy with biopsy revealed nodular proliferation of lymphocytes with predominant interstitial plasma cell infiltrate, with no endobronchial lesions. Serum protein electrophoresis showed Ig G monoclonal gammopathy, with lambda light chain restriction. Subsequent bone marrow biopsy revealed < 10 % plasma cells. Monoclonal gammopathy with end-organ damage confirmed the diagnosis of myeloma secondary to lung plasmacytoma and chemotherapy was initiated. At 3 month follow up, no change in pulmonary infiltrate necessitated a video assisted thoracoscopic (VATS) biopsy. Biopsy revealed dense homogenous infiltrate of small malignant B-lymphocytes with prominent nucleoli and focal plasmacytic differentiation, consistent with lymphaplasmacytic lymphoma (fig 1 & 2). Immunophenotyping confirmed mucosa associated lymphoid tissue (MALT) lymphoma. Staging workup showed no other organ involvement, thus confirming primary pulmonary lymphoma. Right lower lobectomy followed, with continued remission at 3 months with complete normalization of monoclonal gammopathy

DISCUSSION: PPL is an extremely rare neoplasm, accounting for 0.5 -1 % of all lung neoplasms. Of PPL, MALT lymphomas are the most frequent type, which comprises 70 % of reported cases. However, lymphoplasmacytic differentiation of primary pulmonary MALT lymphoma with associated paraproteinemia is an especially uncommon finding, with less than 20 reported cases in medical literature. Clinical presentation is varied, nonspecific and may be asymptomatic at times with an unexplained radiological infiltrate. Usual radiological presentation includes localized pulmonary infiltrate, bilateral in 70 % of cases. Diagnosis requires a high index of suspicion considering the nonspecific presentation, and in most cases successful tissue biopsy may need open lung biopsy. Treatment involves resection in localized cases, and chemotherapy in selected cases. Outcome is generally favorable with a 5 year survival rate of >80 %.

CONCLUSIONS: Our case not only reports a rare pulmonary neoplasm, but also reiterates the possibilities of confounding presentation stemming from varied histological differentiation and the need for proper tissue sample while dealing with PPL .

Reference #1: 1. Kyrtsonis MC, et al. Primary lung involvement in Waldenström's macroglobulinaemia: report of two cases and review of the literature. Acta Haematol. 2001;105(2):92-6. Review

DISCLOSURE: The following authors have nothing to disclose: Sujith Cherian, Subhraleena Das, Shakuntala Mauzo, Amanda Tchakarov, Rahat Hussain, Robert Lodato

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