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The Omenn's syndrome: histological, immunohistochemical and ultrastructural evidence for a partial T cell deficiency evolving in an abnormal proliferation of T lymphocytes and S-100 +/T-6 + Langerhans-like cells.

Abstract:

A 7 month old female infant was affected by a rapidly fatal familial disease highly reminiscent of Omenn's syndrome. She presented with widespread eczematous lesions, hepatosplenomegaly, superficial lymphadenopathy, peripheral blood lymphocytosis, eosinophilia and hyper-IgE. An axillary lymph node was involved by a marked proliferation of T-3 +/T-10-- lymphocytes admixed with S-100+/T-6+/Leu-3a+/Ia + reticular cells which lacked typical LC granules; cell suspension study revealed that 90%-96%...

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Publisher copy:
10.1007/bf00701330

Authors


Stoppacciaro, A More by this author
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Institution:
University of Oxford
Department:
Oxford, MSD, RDM, Clinical Lab Sciences
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Publisher:
Springer-Verlag
Journal:
Virchows Archiv. A, Pathological anatomy and histopathology
Volume:
407
Issue:
1
Pages:
69-82
Publication date:
1985
DOI:
EISSN:
1432-2307
ISSN:
0174-7398
URN:
uuid:d2b8a935-19ed-4c1b-8b08-6f552eafa8eb
Source identifiers:
486757
Local pid:
pubs:486757

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