INTRODUCTION: Bronchial Associated Lymphoid Tissue (BALT) lymphomas are an indolent type of B-cell non-Hodgkins lymphoma (NHL). They make up less than 1% of all NHL. BALT lymphoma rarely presents with endobronchial disease. In our patient the endobronchial disease resulted in completed obstruction of the right lower lobe (RLL) and recurrent post obstructive pneumonias.
CASE PRESENTATION: A 48 year-old previously healthy man, presented to the pulmonary clinic with a 2 month history of productive cough, dyspnea and subjective fevers. He initially was evaluated in the emergency department and had a chest radiograph demonstrating RLL pneumonia. He was treated with levofloxacin for 8 days. His symptoms transiently improved, however, approximately 4 weeks later his symptoms had returned. He underwent a computerized axial tomography (CAT) scan of the chest demonstrating RLL atelectasis along with a RLL endobronchial obstruction. The patient was subsequently evaluated by pulmonary and arranged to undergo a bronchoscopy. The bronchoscopy demonstrated very abnormal mucosa in the left main stem bronchus with diffuse nodular endobronchial lesions resulting in approximately 30% obstruction of the distal airway. In the RLL, the patient had a more localized area of nodular endobronchial lesions obstructing the anterior, lateral and posterior segments. The patient underwent an endobronchial biopsy that showed dense infiltration of mature appearing lymphocytes. Flow cytometry noted CD5 negative, CD10 positive, and CD20 positive consistent with a BALT lymphoma. The patient underwent a PET scan, bone marrow aspiration, along with CAT scan of the chest, abdomen, and pelvis for staging, demonstrating that the disease was localized to the lungs and the mediastinal lymph nodes. The patient was started on chemotherapy with rituximab along with cyclophosphomide, vincristine and prednisone (CVP) every 3 weeks for a total of six cycles. Re-staging with PET and CAT scan is planned following completion of the 4th cycle of chemotherapy. Radiation therapy, targeting the RLL obstruction, will be considered if there is no significant improvement after the chemotherapy.
DISCUSSION: BALT lymphoma is a rare type of B-cell NHL accounting for less than 1% of NHL. This extra-nodal type of marginal zone lymphoma arises from the abnormal BALT in the lungs. In normal adult lungs BALT is not present, however, with chronic antigenic exposures and in individuals with autoimmune diseases BALT may be abnormally present. The hyperplasia of BALT in the lungs may result in lympho-proliferative conditions and subsequently may also result in this indolent type of B-cell NHL. BALT lymphoma may have different manifestations in the lungs. Common symptoms include cough, dyspnea, along with constitutional symptoms. Typically, patients may present with nodules(s), consolidation(s), ground glass opacities (GGO), bronchiectasis, or bronchiolitis. Atelectasis maybe present from likely the obstruction from the endobronchial disease, like in our patient. The endobronchial disease tends to be nodular in appearance and only has been described in case reports. Typically, BALT lymphoma is best diagnosed with a surgical wedge biopsy. However, when endobronchial disease is found endobronchial biopsy is adequate to make a diagnosis. The therapies vary on the extent of disease. Surgical resection has been done with diagnostic and therapeutic intent. Radiation has been used effectively described in patients with endobronchial disease resulting in obstruction. Chemotherapy regimens (CVP) with or without rituximab have been used. Complete remission is common with unilateral lung disease, and less with bilateral.
CONCLUSIONS: BALT lymphoma is a rare type of indolent B-cell NHL. BALT lymphoma rarely presents with endobronchial disease. In our patient the endobronchial disease resulted in completed obstruction of the right lower lobe (RLL) and recurrent post obstructive pneumonias. The decision was to start chemotherapy due to the extensive disease.
Reference #1 Juan C. Cadavid et al. A 68-Year-Old Woman With Fever, Atelectasis, and Nodular Endobronchial Lesions. Chest January 2011; 139:1 208-211
Reference #2 Bae Ya et al. Marginal zone B-cell lymphoma of bronchus-associated lymphoid tissue: imaging findings in 21 patients. Chest. 2008 Feb;133(2):433-40
DISCLOSURE: The following authors have nothing to disclose: Miguel Rodriguez
No Product/Research Disclosure Information