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Pulmonary Alveolar Proteinosis Associated With IgG Monoclonal Gammopathy

John N. Mork, MD; J. Richard Johnson, MD; Horace H. Zinneman, MD; John Bjorgen, MD
Arch Intern Med. 1968;121(3):278-283. doi:10.1001/archinte.1968.03640030070012.
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Pulmonary alveolar proteinosis was first described by Rosen et al in 1958.1 The entity is characterized clinically by dyspnea, recurrent pneumonia, and x-ray findings of an "acinar or alveolar infiltrate" radiating from the hila.2 The course is quite variable. Some patients show complete remission whereas others progress to cyanosis, pulmonary hypertension, and eventual death from respiratory insufficiency. Death, not infrequently, results from fungal or bacterial pulmonary infections.1,3-8 Pathologically, the alveoli are filled with a periodic acid Schiff positive, lipid rich, proteinaceous substance.9 The diagnosis is made by lung biopsy.

"Essential" hyperglobulinemia was first described by Waldenström in 1944.10 Other names subsequently applied to this condition include monoclonal gammopathy11 and dysimmunoglobulinemia.12 Briefly, this condition is characterized by an M peak on the patient's serum protein electrophoretic pattern. This protein peak may represent any one of the three major immunoglobulin types on immunoelectrophoresis.12 An


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