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May 1962

Primary Esophageal AmyloidosisReport of a Case with Bleeding, Perforation, and Survival Following Resection

Author Affiliations


Associate Attending Physician, Department of Medicine (Dr. E. J. Heitzman), Assistant Attending Surgeon, Department of Thoracic Surgery (Dr. G. C. Heitzman), Resident in General Practice (Dr. Elliott), St. Joseph's Hospital, Syracuse.

Arch Intern Med. 1962;109(5):595-600. doi:10.1001/archinte.1962.03620170093015

Although primary amyloidosis has been recognized since the middle of the nineteenth century,1 little clinical interest was evidenced until Lubarsch2 reported 3 new cases in 1929. In 1935, Reimann, Kouchky, and Eklund3 presented the first case of primary amyloidosis diagnosed clinically. Since then, over 200 cases have been published. With the advent of these data, understanding of the protean manifestations of the disease has grown, and subsequently several classifications have been proposed. Probably the most widely accepted is that of Dahlin4:

  • Primary Amyloidosis

    1. Systemic

    2. Tumefactive

  • Amyloidosis complicating Myeloma

    1. Systemic

    2. Tumefactive

  • Secondary Amyloidosis

    1. Systemic

    2. Tumefactive One year after this classification was published, a further subdivision of primary amyloidosis was recognized—familial amyloidosis.5 Since then, over 108 cases have been reported and indicate a definite clinical pattern (neuropathy, gastrointestinal and cardiovascular involvement, and ocular manifestations) with a simple dominant

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