Document Detail

Giant cell granulomatous pulmonary and myocardial lesions in necrobiotic xanthogranuloma with paraproteinemia.
MedLine Citation:
PMID:  9374976     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: To report three cases of pulmonary or myocardial disease (or both) and necrobiotic xanthogranuloma.
MATERIAL AND METHODS: Giant cell granulomas of the lung and myocardium were demonstrated in three patients who had pulmonary and myocardial lesions of necrobiotic xanthogranuloma in conjunction with skin lesions, leukopenia, paraproteinemia, and complement deficiencies. The patients were two men who were 47 and 64 years of age and a 39-year-old woman.
RESULTS: Biopsies of skin and visceral lesions showed asteroid and cytoplasmic inclusions. B-cell lymphoid nodules were found. In one of the male patients, a major clonal T-cell receptor gene rearrangement was detected in the peripheral blood. Prednisone was ineffective in two of the patients. The other patient experienced regression of skin lesions and diminishment of a chest nodule after receiving alkylating agent therapy.
CONCLUSION: Establishing the correct diagnosis is important, and apparently it is possible to establish the nature of the myocardial and pulmonary lesions with use of appropriate scans and by biopsy. Successful treatment of necrobiotic xanthogranuloma skin lesions with corticosteroids or alkylating agents (or both) implies that evolution of serious disease that compromises the heart and lungs could be controlled.
R K Winkelmann; M R Litzow; I J Umbert; J T Lie
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Mayo Clinic proceedings     Volume:  72     ISSN:  0025-6196     ISO Abbreviation:  Mayo Clin. Proc.     Publication Date:  1997 Nov 
Date Detail:
Created Date:  1997-12-11     Completed Date:  1997-12-11     Revised Date:  2013-12-13    
Medline Journal Info:
Nlm Unique ID:  0405543     Medline TA:  Mayo Clin Proc     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1028-33     Citation Subset:  AIM; IM    
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MeSH Terms
Cardiomyopathies / complications,  pathology*
Diagnosis, Differential
Granuloma, Giant Cell / complications,  pathology*
Lung Diseases / complications,  pathology*
Middle Aged
Necrobiosis Lipoidica / complications,  pathology*
Paraproteinemias / complications*,  pathology
Skin / pathology*
Xanthomatosis / complications,  pathology*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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