pnrobinson
commented
6 years ago This appears to be a disease but there are a number of phenotypes that are not currently in HPO Consider:
- [x] NTR intracellular CD3 by flow cytometry after permeabilization (or T cells with lack of surface CD3 ??)
- [x] presence of a monoclonal T cell population (Can we define this better? How do we know it is monoclonal, it is be determination of the TCR beta chain for instance?)
Note though that these features are in the HPO already: Patients with LHES may present with a range of cutaneous findings, including eczematous dermatitis, nodules, papules, poikiloderma, plaques, pruritis, urticaria and/or angioedema and erythroderma.
New term request: Lymphoid hypereosinophilic syndrome. Synonyms: L-HES; lymphocytic hypereosinophilic syndrome\ Definition: Subtype of hypereosinophilic syndrome defined by the presence of a monoclonal T cell population detected in conjunction with immunophenotypically aberrant T cell populations. Source: Subtype of hypereosinophilic syndrome defined by the presence of a monoclonal T cell population detected in conjunction with immunophenotypically aberrant T cell populations. Parent: http://www.hematology.org/Thehematologist/Mini-Review/7884.aspx Disease association: Eosinophilia