Chest
Volume 92, Issue 4, October 1987, Pages 704-708
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Clinical Investigations
Pulmonary Immunologic Features of Alveolar Septal Amyloidosis Associated with Multiple Myeloma

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A 74-year-old man presented with interstitial pulmonary disease which was proven to be alveolar septal amyloidosis by transbronchial biopsy. Multiple myeloma was diagnosed on the basis of monoclonal IgG-λ protein in serum, monoclonal λ light chains in urine, a bone marrow plasmacytosis of 22 percent, and serum IgA and IgM levels less than 100 mg/dl and 50 mg/dl, respectively. Appropriate investigations failed to show additional sites of deposition of amyloid. Analysis of fluid from bronchoalveolar lavage showed an increase in total cells recovered, a lymphocytosis with a ratio of T helper over T suppressor cells greater than that in peripheral blood, the presence of an IgG-λ paraprotein, and an IgG/albumin ratio greater than that in serum. While plasma cells could not be identified in the recovered cell population, cultured cells from bronchoalveolar lavage fluid showed increased production of IgG. These findings provide evidence of an ongoing pulmonary immune response resulting in excess IgG-λ protein in the pulmonary compartment, a factor which may contribute to the development of amyloidosis.

Section snippets

CASE REPORT

A 74-year-old man was evaluated because of mild dyspnea on exertion and tightness in the chest. He denied fever, chills, loss of weight, hemoptysis, dyspnea at rest, or edema of the ankles. The patient had had no past medical or surgical illnesses and no potentially adverse occupational exposures. He had smoked one-half pack of cigarettes daily for 40 years but quit 15 years prior to this evaluation. The review of systems was positive for urinary frequency, nocturia, and a single recent episode

Study Population

Healthy volunteers participating in studies assessing cellular and humoral characteristics of fluid from bronchoalveolar lavage made up the group for comparison. Because the patient is an ex-smoker and little information exists on the characteristics of fluid from bronchoalveolar lavage in such people, values from the patient were compared to the results from such fluid in a healthy control population which included smokers and nonsmokers. All participants gave informed consent prior to

Analysis of Cells and Sediment from Bronchoalveolar Lavage Fluid

In fluid recovered from bronchoalveolar lavage of the patient, the total number of cells, both macrophages and lymphocytes, was increased. The ratio of T helper over T suppressor cells was higher than either that of the control group or the patient’s own peripheral blood. The number but not the percentage of B-lymphocytes was also higher (Table 1).

No plasma cells were identified by light or electron microscopy. When viewed by electron microscopy, the macrophages contained irregular

DISCUSSION

A diagnosis of multiple myeloma was made on the basis of a bone marrow plasmocytosis of 22 percent, a serum IgG-λ paraprotein, and serum concentrations of IgA and IgM less than 100 mg/dl and 50 mg/dl, respectively.16 The diagnosis of diffuse septal amyloidosis was made by transbronchial biopsy.17 No other clinical or histologic evidence suggesting additional deposition of amyloid was obtained. The notable findings from the bronchoalveolar lavage were the lymphocytosis, the high ratio of T

ACKNOWLEDGMENTS

We thank Ms. P. Williams for technical assistance, Ms. D. Delwiche for typing the manuscript, and Dr. Martha Skinner for advice and help.

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Manuscript received August 25; revision accepted February 13.

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