The lymph node pathology of Omenn's syndrome.
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.
Martin
J V
JV
Department of Pathology, Orlando Regional Medical Center, FL 32806, USA.
Willoughby
P B
PB
Giusti
V
V
Price
G
G
Cerezo
L
L
D015703:Antigens, CD / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000473:pathology*; D007231:Infant, Newborn; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology*; D008214:Lymphocytes / Q000276:immunology / Q000473:pathology
D015703:Antigens, CD