Uncommon Presentation of Undiagnosed B-Cell Lymphoproliferative Disorder as Nodular Pulmonary Amyloidosis

Harsh Patel, Aaiyat Sheikh, Gnana Deepthi Medarametla, Sri Abirami Selvam, Syed Nazeer Mahmood, Gurleen Johal, Janani Arunachalam, Haripriya Radhakrishnan, Viray Shah, Aditya Lal Vallath, Digantkumar Patel, Saketh Palasamudram Shekar, Urvish Patel, Nisarg Changawala

Abstract


B-cell lymphoproliferative disorders are characterized by the accumulation of mature B lymphocytes in the bone marrow, lymphoid tissues, and/or peripheral blood. They can cause amyloid deposits in the lungs. In rare cases, lung nodules can be the first sign of this disorder. We present the case of an 89-year-old woman with stable shortness of breath and lung nodules on imaging. A positron emission tomography-computed tomography (PET-CT) scan showed the most intense hypermetabolic nodule in the patients lung, which was 1.5 1.4 cm. A biopsy of this nodule showed amyloid material with trapped plasma cell infiltrate on microscopy. Congo red stain under polarizing microscopy showed apple-green birefringence, which is diagnostic for amyloidosis. Immunohistochemistry showed a mixture of kappa-positive and lambda-positive cells. B-cell gene rearrangement-clonal gene rearrangements were detected in the immunoglobulin heavy chain (IgH) gene and the kappa light chain (IGK). These findings suggest a B-cell lymphoproliferative disorder, such as a plasmacytoma or a marginal cell lymphoma with plasma cell differentiation. The patient was diagnosed with a B-cell lymphoproliferative disorder and pulmonary amyloidosis. Isolated amyloidosis in the lungs usually has a good prognosis, but it can be a sign of autoimmune diseases or B-cell lymphoproliferative disorders, as in this case. Early diagnosis of B-cell lymphoproliferative disorder can lead to successful treatment and prevents complications.




J Med Cases. 2023;14(1):36-43
doi: https://doi.org/10.14740/jmc4026

Keywords


Nodular pulmonary amyloidosis; Pulmonary amyloidosis; B-cell lymphoproliferative disorder; Pulmonology; Oncology; Evidence based medicine

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