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Cystic Lung Disease Due to Primary Light Chain Deposition
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A6538 - Cystic Lung Disease Due to Primary Light Chain Deposition
Author Block: A. T. Fabro1, L. F. Biazim2, F. M. Lima3, V. Carvalho3, J. Castelli3, P. d. Leão4, S. S. Batah1, M. K. Santos5, J. Baddini-Martinez1; 1Pathology and Legal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil, 2Pulmonology Sector of the Department of Clinical Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil, 3Pathology, Fleury Laboratory, São Paulo, Brazil, 4Department of Pathology and Legal Medicine, University of São Paulo, Ribeirão Preto, Sã, Brazil, 5Radiology, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
The light-chain deposition disease is a systemic disorder caused by overproduction and deposition of the immunoglobulin light chain secreted by a B lymphocyte clone, usually associated with lymphoproliferative and autoimmune diseases. However, a lung form of the disease is rarely found. In this case, a 38-year-old, male, physician, asymptomatic, showed computed tomography with scattered cysts through the lungs, but with discrete inferior lung lobes predominance, as incidental findings due to medical monitoring of ankylosing spondylitis. The surgical lung biopsy reveals, at the low power field, a well-defined constrictive bronchiolitis related to several cystic dilations of various dimensions, some incomplete, others divided by delicate alveolar septa within a small vessel. At high magnification, their walls were thin due to compression of lung parenchyma with some luminal vascular protuberances. Furthermore, a prominent intimal hyalinization of some small vessels and small hyaline masses in broncovascular axis, both Congo-red staining negative, were observed. Moreover, HMB-45, CD1a, S-100, SMAα and D2-40 imunomarkers were all negatives. Measurement of free kappa and lambda chains, IgG, IgA and IgE in serum were normal. Lung function test were also normal. Patient remains asymptomatic with normal lung function and, in multidisciplinary meeting, clinical follow-up was chosen. The light chain deposition of Ig Kappa chain C region wasconfirmated by mass spectrometry. In conclusion, the multidisciplinary discussion are critical to clinical-radiological-pathological analysis for ruling out differential diagnosis and favor pulmonary light chain deposition disease.
Author Block: A. T. Fabro1, L. F. Biazim2, F. M. Lima3, V. Carvalho3, J. Castelli3, P. d. Leão4, S. S. Batah1, M. K. Santos5, J. Baddini-Martinez1; 1Pathology and Legal Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil, 2Pulmonology Sector of the Department of Clinical Medicine, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil, 3Pathology, Fleury Laboratory, São Paulo, Brazil, 4Department of Pathology and Legal Medicine, University of São Paulo, Ribeirão Preto, Sã, Brazil, 5Radiology, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.
The light-chain deposition disease is a systemic disorder caused by overproduction and deposition of the immunoglobulin light chain secreted by a B lymphocyte clone, usually associated with lymphoproliferative and autoimmune diseases. However, a lung form of the disease is rarely found. In this case, a 38-year-old, male, physician, asymptomatic, showed computed tomography with scattered cysts through the lungs, but with discrete inferior lung lobes predominance, as incidental findings due to medical monitoring of ankylosing spondylitis. The surgical lung biopsy reveals, at the low power field, a well-defined constrictive bronchiolitis related to several cystic dilations of various dimensions, some incomplete, others divided by delicate alveolar septa within a small vessel. At high magnification, their walls were thin due to compression of lung parenchyma with some luminal vascular protuberances. Furthermore, a prominent intimal hyalinization of some small vessels and small hyaline masses in broncovascular axis, both Congo-red staining negative, were observed. Moreover, HMB-45, CD1a, S-100, SMAα and D2-40 imunomarkers were all negatives. Measurement of free kappa and lambda chains, IgG, IgA and IgE in serum were normal. Lung function test were also normal. Patient remains asymptomatic with normal lung function and, in multidisciplinary meeting, clinical follow-up was chosen. The light chain deposition of Ig Kappa chain C region wasconfirmated by mass spectrometry. In conclusion, the multidisciplinary discussion are critical to clinical-radiological-pathological analysis for ruling out differential diagnosis and favor pulmonary light chain deposition disease.
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