The lymph node pathology of Omenn's syndrome.
Am J Surg Pathol
; 19(9): 1082-7, 1995 Sep.
Article
en En
| MEDLINE
| ID: mdl-7661282
ABSTRACT
Omenn's syndrome is a rare, usually fatal immunologic disorder of infancy characterized by recurrent infections, skin lesion, lymphadenopathy, peripheral blood lymphocytosis, and eosinophilia. Histologic evaluation of a lymph node revealed total effacement of the microscopic architecture resulting from a diffuse proliferation of interdigitating reticulum cells and a depletion of B lymphocytes. The lymph node lacked a distinct cortex and had no follicle formation. The most striking feature was a diffuse hyperplasia of S-100-protein-positive nonphagocytic reticulum cells with large, pale Langerhans-like nuclei. Ultrastructural examination identified these cells to be interdigitating reticulum cells. The lymphocytes were small and predominantly of the CD8 cytotoxic/suppressor cell type.
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Base de datos:
MEDLINE
Asunto principal:
Síndromes de Inmunodeficiencia
/
Ganglios Linfáticos
Tipo de estudio:
Diagnostic_studies
/
Prognostic_studies
Límite:
Female
/
Humans
/
Newborn
Idioma:
En
Revista:
Am J Surg Pathol
Año:
1995
Tipo del documento:
Article
País de afiliación:
Estados Unidos