Cyclical hypereosinophilia with skin manifestations and a clonal T cell population

Hypereosinophilia is a common biological finding in clinical practice, in some cases without an identifiable cause. We describe the case of a 59-year-old woman with recurrent attacks of facial angioedema, fever, pruritic cutaneous nodules, and eosinophilia that reached up to 12.7 x 10(9) cells/L dur...

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Published inJournal of investigational allergology & clinical immunology Vol. 18; no. 5; p. 401
Main Authors González Delgado, P, de la Sen Fernández, M L, Soriano Gomis, V, Pérez Crespo, M, Muñoz Ruiz, C, Hernández Niveiro, E
Format Journal Article
LanguageEnglish
Published Spain 2008
Subjects
Angioedema
CD4-Positive T-Lymphocytes - immunology
Cell Proliferation
Clone Cells
Eosinophils - immunology
Eosinophils - pathology
Erythema Nodosum
Female
Humans
Hypereosinophilic Syndrome - drug therapy
Hypereosinophilic Syndrome - immunology
Hypereosinophilic Syndrome - physiopathology
Immunophenotyping
Interleukin-5 - secretion
Middle Aged
Periodicity
Prednisolone - administration & dosage
Receptors, Antigen, T-Cell, gamma-delta - genetics
Receptors, Antigen, T-Cell, gamma-delta - immunology
Skin - immunology
Skin - pathology
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Summary:Hypereosinophilia is a common biological finding in clinical practice, in some cases without an identifiable cause. We describe the case of a 59-year-old woman with recurrent attacks of facial angioedema, fever, pruritic cutaneous nodules, and eosinophilia that reached up to 12.7 x 10(9) cells/L during outbreaks. She had experienced 2 episodes every month for the last 12 years, and the episodes resolved with systemic corticosteroids. Other causes of eosinophilia were ruled out. The patient showed an aberrant T cell population with a CD3-CD4+ TCR- phenotype that accounted for up to 22% of circulating lymphocytes. Analysis of the T-cell receptor (TCR) gene showed evidence of clonal rearrangement. During the episodes, this cell population produced high levels of interleukin-5, which returned to normal levels between the outbreaks. However the aberrant T cell population remained unaffected after the treatment. We suggest that lymphocyte immunophenotyping analysis should be included in the diagnostic workup of patients with hypereosinophilic syndrome, including the variant type of episodic angioedema and eosinophilia (Gleich syndrome).
ISSN:1018-9068