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When It Is More Than Just Pneumonia: A Case of Pulmonary Waldenstrom Macroglobulinemia FREE TO VIEW

Hazel Abool, MD; Ifeoma Okeke, MD; Lokesh Venkateshaiah, MD
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Akron General Medical Center, Akron, OH

Chest. 2013;144(4_MeetingAbstracts):630A. doi:10.1378/chest.1698924
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SESSION TITLE: Cancer Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Waldenstrom Macroglobulinemia (WM) is a distinct clinicopathological entity characterized by bone marrow infiltration by lymphoplasmacytic lymphoma and IgM monoclonal gammopathy in the serum. A US survey for WM conducted from 1988-1994 showed age-adjusted incidence rates of 3.4 and 1.7 per 1 million person-years at risk for male and female patients, respectively. The presenting features are the result of infiltration of hematopoietic tissues. However, WM rarely presents as primary lung involvement.

CASE PRESENTATION: We report the case of a 48 year old Caucasian male presenting with a two-week history of cough and shortness of breath. Physical examination did not reveal any lymphadenopathy or hepatosplenomegaly. Chest X-ray showed consolidation of the right middle and lower lobes. The patient was empirically treated with intravenous antibiotics. CT scan confirmed the presence of lung consolidation and moderately large pleural effusion. There was also a soft tissue density on the right side of the mediastinum and subcarinal region. Pleural fluid cytology was positive for malignant cells suggestive of lymphoma. SPEP with IFE was consistent with an IgM-lambda monoclonal protein. Immunophenotyping on the pleural fluid specimen revealed an IgM restricted lymphoplasmacytic population comprising 25% of the total cellularity. Biopsy of the anterior right pretracheal lymph nodes and bone marrow revealed B-cell lymphoproliferative disorder with plasmacytic differentiation. A PET scan prior to treatment showed intense FDG uptake to correlate with the area of lung consolidation. Treatment with rituximab and bortezomib was initiated. A repeat PET scan 3 months later showed new foci of abnormal FDG uptake in the right supraclavicular and internal mammary lymph nodes. Treatment was switched to bendamustine and rituximab. Following six cycles of chemotherapy, CT scan showed clearing of the lung consolidation and reduced soft tissue density in the subcarinal region. The patient has since been symptomatic.

DISCUSSION: Pleuropulmonary involvement as a presenting symptom of WM is rare. To our knowledge, this is the first successful treatment of pulmonary WM with bendamustine and rituximab. Lung involvement of WM can follow an indolent course, but can be decompensated by other processes such as pneumonia.

CONCLUSIONS: It is recommended to empirically treat for pneumonia when pulmonary WM presents with worsening signs and symptoms. This report underscores the importance of recognizing this rare manifestation of WM to promote earlier treatment and reduce the rate of complications.

Reference #1: Owen RG, Treon SP, Al-Katib, et al. Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the second International Workup on Waldenstrom's Macroglobulinemia. J Clin Oncol 2005; 23: 1564

Reference #2: Groves FD, Travis LB, Devesa SS et al. Waldenstrom's macroglobulinemia: incidence patterns in the United States, 1988-1994. Cancer 1998; 82: 1078

DISCLOSURE: The following authors have nothing to disclose: Hazel Abool, Ifeoma Okeke, Lokesh Venkateshaiah

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