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April 1984

Angioimmunoblastic Lymphadenopathy With Fisher Syndrome

Author Affiliations

From the Department of Ophthalmology, University of Connecticut Medical Center, Farmington (Drs Monteiro and Coppeto) and St Mary's Hospital, Waterbury, Conn (Drs Greco and Pittard). Dr Monteiro was a Fellow in Neuroophthalmology from the Department of Ophthalmology, Hospital das Clínicas, University of São Paulo (Brazil) Medical School. Dr Monteiro is now with the University of California San Francisco.

Arch Neurol. 1984;41(4):456-457. doi:10.1001/archneur.1984.04050160122028

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a lymphomalike disease consisting of fever, malaise, polyclonal gammopathy, and diffuse lymphadenopathy. Histopathologic study of lymph nodes characteristically shows obliteration of normal lymph node architecture by proliferation of immunoblasts and plasma cells, arborizing small blood vessels, and interstitial deposition of homogeneous eosinophilic material.1-5 The disease has features of both immune deficiency and hyperimmunity, accounting for both reduced resistance to infection and autoimmune phenomena.1-6

Although AILD is sometimes a benign disorder, supervening lethal infections and transformation into malignant lymphoma (immunoblastic sarcoma) are dreaded complications.2-5,7 Central nervous system dysfunction occurring in the course of AILD may be an ominous sign, suggesting intracranial hemorrhage or invasion of tumor or opportunistic infection in an immunosuppressed patient.3,4,8,9 Therefore, it is of interest to report the first case of AILD, to our knowledge, associated with the Fisher syndrome, a generally benign variant of Guillain-Barré syndrome (GBS).

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