Lung Pathology |

Primary Pulmonary Diffuse Large B-Cell Lymphoma in Scleroderma- A Case Report FREE TO VIEW

Mandeep Hundal, MD; Christian Ghattas, MD; Bridget Collins, MD
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St. Elizabeth's Medical Center, Stow, MA

Chest. 2015;148(4_MeetingAbstracts):612A. doi:10.1378/chest.2232090
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SESSION TITLE: Lung Pathology Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Primary Non Hodgkin's Lymphoma (NHL) of the lung is rare and accounts for 0.4% of all lymphomas1. Increased incidence of lymphoproliferative neoplasms has been reported in scleroderma patients2.

CASE PRESENTATION: An 85-year-old woman with Scleroderma and Interstitial lung disease presented with 3 weeks of productive cough and fever. Computed Tomography (CT) scan of the chest revealed a right upper lobe (RUL) cavitary lesion and a 1.6 x 1.8 cm left lingular density. She underwent bronchoscopy with negative fungal, AFB and bacterial cultures. She was treated with levofloxacin for 2 weeks but continued to have dry cough and night sweats. A repeat chest CT 3 months later demonstrated almost complete resolution of the RUL cavitary lesion, but enlargement of the lingular mass (3.2 x 4.7 cm) as well as mediastinal adenopathy. There was no evidence of extrathoracic disease on clinical examination or on CT scan of the abdomen & pelvis. Endobronchial Ultrasound (EBUS) guided mediastinal lymph node biopsy was negative for malignant cells. A CT guided core biopsy of the lingular mass was consistent with diffuse large B-cell lymphoma (DLBCL) with positive CD20, CD79a and BCL-6 markers. The patient declined chemotherapy and opted for hospice care.

DISCUSSION: Scleroderma patients have a higher incidence of NHL compared to the general population2. Risk factors for NHL in scleroderma include older age, female sex and diffuse cutaneous involvement2. Defective B-cell and natural killer cell activity is thought to cause impaired tumor surveillance in scleroderma resulting in increased risk of lymphoproliferative disorders2. Primary NHL of the lung usually presents with nonspecific symptoms. The most common primary pulmonary lymphoma is mucosa-associated lymphoid tissue (MALT) lymphoma. DLBCL occurs only in 10% cases of primary pulmonary NHL1. Treatment should be based on histology, stage and performance status. Anthracycline-based chemotherapy with anti-CD20 monoclonal antibody is a reasonable option for primary DLBCL of the lung1.

CONCLUSIONS: Clinicians should be aware of the increased risk for lymphoproliferative disorders, including pulmonary NHL in scleroderma patients.

Reference #1: Wróbel T, Dzietczenia, J, Prochorec-Sobieszek M et al. Primary pulmonary diffuse large B-cell lymphoma. Am. J. Hematol., 87: 107-108.

Reference #2: Vettori S, Staibano S, Mascolo M et al. Non-Hodgkin's lymphoma in systemic sclerosis: case and literature review. Clin Rheumatol. 2010 Jan;29(1):1-6.

DISCLOSURE: The following authors have nothing to disclose: Mandeep Hundal, Christian Ghattas, Bridget Collins

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