Lymph nodes - not lymphoma
Primary immunodeficiency disorders
Reviewer: William D. DePond, M.D. (see Reviewers page)
Revised: 11 June 2013, last major update June 2006
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.
- Rare, autosomal recessive form of severe combined immunodeficiency of infancy
- Recurrent infections, early diffuse erythrodermia, failure to thrive, protracted diarrhea, hepatosplenomegaly, elevated serum IgE and eosinophilia, lymphadenopathy, oligoclonal T cell expansions
- Often recombinase activating gene mutations (Clin Immunol 2005;116:246)
- Also increased circulating and infiltrating activated T cells that are unable to proliferate in response to mitogenic stimuli
- Total effacement of nodal architecture with no distinct cortex and no follicles
- Also B cell depletion and accumulation of interdigitating reticulum cells (Am J Surg Pathol 1995;19:1082)
End of Lymph nodes - not lymphoma > Primary immunodeficiency disorders > Omennís syndrome
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