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Endobronchial involvement as an extremely rare manifestation of the Waldenström's disease

H. Bartáková, J. Novák, R. Jakša, J. Beneš, J. Votruba,

. 2018 ; 12 (2) : 816-819. [pub] 20161031

Language English Country England, Great Britain

Document type Case Reports

Pulmonary infiltration is an infrequent organ involvement in Waldenström's disease (WD). Diffuse infiltration, isolated parenchymatous nodules, and pleural effusion are the most common manifestations of WD, while endobronchial mass is extremely rare. We present a case report of a 66-year-old man with a long-standing history of WD, who developed febrile neutropenia after therapy with rituximab, cyclophosphamide, and dexamethasone. X-ray and CT scan showed consolidation consistent with right-sided pneumonia. Surprisingly, bronchoscopy revealed an endobronchial tumor obstructing the right lower lobe (RLL) and two smaller granulations. Biopsies were obtained and recanalization of the RLL bronchus was performed. Immunohistological staining of the samples was consistent with lymphoplasmacytic lymphoma. Despite the change in therapy the patient died 6 weeks later. A review of published literature revealed only two case reports of endobronchial involvement in WD to this day. While one of the case reports described a patient with diffuse submucosal infiltration of the airways, the other one presented a patient with bronchus-obstructing tumor similar to the case reported here.

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$a Pulmonary infiltration is an infrequent organ involvement in Waldenström's disease (WD). Diffuse infiltration, isolated parenchymatous nodules, and pleural effusion are the most common manifestations of WD, while endobronchial mass is extremely rare. We present a case report of a 66-year-old man with a long-standing history of WD, who developed febrile neutropenia after therapy with rituximab, cyclophosphamide, and dexamethasone. X-ray and CT scan showed consolidation consistent with right-sided pneumonia. Surprisingly, bronchoscopy revealed an endobronchial tumor obstructing the right lower lobe (RLL) and two smaller granulations. Biopsies were obtained and recanalization of the RLL bronchus was performed. Immunohistological staining of the samples was consistent with lymphoplasmacytic lymphoma. Despite the change in therapy the patient died 6 weeks later. A review of published literature revealed only two case reports of endobronchial involvement in WD to this day. While one of the case reports described a patient with diffuse submucosal infiltration of the airways, the other one presented a patient with bronchus-obstructing tumor similar to the case reported here.
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$a Novák, Jan $u Department of Internal Medicine and Haematology, Third Faculty of Medicine, Charles University in Prague and Faculty Hospital Královské Vinohrady, Prague, Czech Republic.
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$a Jakša, Radek $u Institute of Pathology, General University Hospital, First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic.
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$a Beneš, Jiří $u Department of Radiology, General University Hospital, First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic.
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