Pulmonary involvement in Waldenström’s macroglobulinaemia (WM) occurs in 3–5% of cases, but lung involvement without bone marrow infiltration is extremely rare. We report 2 patients who presented with bilateral consolidations on chest X-ray and non-specific symptoms and were treated for a long period of time for pulmonary infections until the diagnosis was made by open lung biopsy. Both patients presented high monoclonal IgM in the serum and one also had blood lymphoplasmacytosis. Trephine bone biopsy and bone marrow smears were normal and there was no other site of involvement. Along with the presentation of our patients, we review the literature, discuss some of the possible underlying mechanisms and raise the attention of clinicians to this rare manifestation of the disease.

Waldenström J: Incipient myelomatosis or ‘essential’ hyperglobulinemia with fibrigenopenia – a new syndrome. Acta Med Scand 1944;117:216–247.
Pangalis GA, Angelopoulou MK, Vassilakopoulos ThP, Siakandaris MP, Kittas Ch: B-chronic lymphocytic leukemia, small lymphocytic lymphoma and lymphoplasmacytic lymphoma including Waldenström’s macroglobulinemia: A clinical, morphologic and biologic spectrum of similar disorders. Semin Hematol 1999;36:104–114.
Owen RG, Johnson SA, Morgan GJ: Waldenström’s macroglobulinaemia: Laboratory diagnosis and treatment. Hematol Oncol 2000;18:41–49.
Fadil A, Taylor DE: The lung and Waldenström’s macroglobulinemia. South Med J 1998;91:681–685.
Chong Toh H, Tiam Ang P: Primary pulmonary lymphoma – Clinical review from a single institution in Singapore. Leuk Lymphoma 1997;27:153–163.
Habermann TM, Ryu JH, Inwards DJ, Kurtin PJ: Primary pulmonary lymphoma. Semin Oncol 1999;26:307–315.
Suda T, Chida K, Hayakawa H, Imokawa S, Iwata M, Nakamura H, Sato A: Development of bronchus-associated lymphoid tissue in chronic hypersensitivity pneumonitis. Chest 1999;115:357–363.
Ward AM, Path MRC, Shortland JR, Darke CS: Lymphosarcoma of the lung with monoclonal (IgM) gammopathy. Cancer 1971;27:1009–1028.
Essig LJ, Timms ES, Hancock DE, Sharp GC: Plasma cell interstitial pneumonia and macroglobulinemia. Am J Med 1974;56:398–405.
Peterson H, Snider HL, Yam LT, Bowlds CF, Arnn EH, Yang C: Primary pulmonary lymphomas: A clinical and immunohistochemical study of six cases. Cancer 1985;56:805–813.
Kobayashi H, Li K, Hizawa K, Manda T: Two cases of pulmonary Waldenström’s macroglobulinemia. Chest 1985;88:287–289.
Subramanian D, Albrecht S, Gonzalez JM, Cagle PT: Primary pulmonary lymphoma. Diagnosis by immunoglobulin gene rearrangement study using a novel polymerase chain reaction technique. Am Rev Respir Dis 1993;148:222–226.
Koss MN, Hochholzer L, Nichols PW, Wehunt WD, Lazarus AA: Primary non-Hodgkin’s lymphoma and pseudolymphoma of the lung: A study of 161 patients. Hum Pathol 1983;14:1024–1038.
L’hoste RJ, Filippa DA, Lieberman PH, Bretsky S: Primary pulmonary lymphomas: A clinicopathologic analysis of 36 cases. Cancer 1984;54:1397–1406.
Kennedy JL, Nathwani BN, Burke JS, Hill LR, Rappaport H: Pulmonary lymphomas and other pulmonary lymphoid lesions: A clinicopathologic and immunologic study of 64 patients. Cancer 1984;56:539–552.
Li G, Hansmann ML, Zwingers T, Lennert K: Primary lymphomas of the lung: Morphological, immunohistochemical and clinical features. Histopathology 1990;16:519–531.
Cordier JF, Chailleux E, Lauque D, Reynaud-Gaubert M, Dietemann-Molard A, Dalphin JC, Blanc-Jouvan F, Loire R: Primary pulmonary lymphomas: A clinical study of 70 cases in nonimmunocompromised patients. Chest 1993;103:201–208.
Philippe B, Delfau-Larue MH, Epardeau B, Autran B, Clauvel JP, Farcet JP, Couderc LJ: B-cell pulmonary lymphoma: Gene rearrangement analysis of bronchoalveolar lymphocytes by polymerase chain reaction. Chest 1999;115:1242–1247.
Burke JS: Extranodal hematopoietic/lymphoid disorders Am J Clin Pathol 1999;111(suppl 1):40–45.
Fiche M, Captron F, Berger F: Primary pulmonary non-Hodgkin’s lymphomas. Histopathology 1995;26:529–537.
Isaacson PG: Mucosa associated lymphoid tissue lymphoma. Semin Hematol 1999;36:139–147.
Angelopoulou MK, Kondopidou FN, Pangalis GA: Adhesion molecules in B-chronic lymphoproliferative disorders. Semin Hematol 1999;36:178–197.
Pangalis GA: Introduction: Small lymphocyte cell disorders. Semin Hematol 1999;36:101–103.
Moore DF, Moulopoulos LA, Dimopoulos MA: Waldenström’s macroglobulinemia presenting as a renal or perirenal mass: Clinical and radiographic features. Leuk Lymphoma 1995;17:331–334.
Yasui O, Tukamoto F, Sasaki N, Saito T, Yagisawa H, Uno A, Nonjo H: Malignant lymphoma of the transverse colon associated with macroglobulinemia. Am J Gastroenterol 1997;92:2299–2301.
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