Lymphedema, lymphocytic myocarditis, and sarcoidlike granulomatosis. Manifestations of Whipple's disease.

TitleLymphedema, lymphocytic myocarditis, and sarcoidlike granulomatosis. Manifestations of Whipple's disease.
Publication TypeJournal Article
Year of Publication1989
AuthorsSouthern JF, Moscicki RA, Magro C, Dickersin GR, Fallon JT, Bloch KJ
JournalJAMA
Volume261
Issue10
Pagination1467-70
Date Published1989 Mar 10
ISSN0098-7484
KeywordsAdult, Diagnosis, Differential, Granuloma, Humans, Intestine, Small, Lymph Nodes, Lymphedema, Macrophages, Male, Myocarditis, Myocardium, Prednisone, Sarcoidosis, Whipple Disease
Abstract

A patient with Whipple's disease presented with a long prodromal period characterized by granulomatous lymphadenitis and progressive lymphedema of the extremities. No gastrointestinal tract symptoms were present and a small bowel biopsy sample was normal. His clinical condition deteriorated with the onset of lymphocytic myocarditis. At autopsy, intestinal involvement with macrophages that stained positively with periodic acid-Schiff was limited primarily to the submucosa. Diffuse fibrous effacement of lymph nodes with afferent lymphangiectasia seemed to be the mechanism of diffuse lymphedema, protein-losing enteropathy, and hypoproteinemia. Whipple's disease, therefore, should be considered in the differential diagnosis of patients presenting with granulomatous disease, lymphocytic myocarditis, or unusual lymphedema.

Alternate JournalJAMA
PubMed ID2465422
Related Faculty: 
Cynthia M. Magro, M.D.

Pathology & Laboratory Medicine 1300 York Avenue New York, NY 10065 Phone: (212) 746-6464
Surgical Pathology: (212) 746-2700