Return to Contents in this Issue

Case Report

 

Cyclical Hypereosinophilia With Skin Manifestations and a Clonal T Cell Population

 

P González Delgado,1 ML de la Sen Fernández,2 V Soriano Gomis,1 M Pérez Crespo,1 C Muñoz Ruiz,2 E Niveiro Hernández1

1 Department of Allergology, University General Hospital of Alicante, Alicante, Spain
2 Department of Immunology, University General Hospital of Alicante, Alicante, Spain

J Investig Allergol Clin Immunol 2008; Vol. 18(5): 401-403

 

 Abstract


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 12.7 109 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).

Key words: Hypereosinophilia. Cyclical angioedema. Gleich syndrome. Interleukin 5. CD3-CD4+ T cells.