Erythrodermic cutaneous T cell lymphoma with hypereosinophilic syndrome: Treatment with interferon alfa and extracorporeal photopheresis

Int J Dermatol. 2007 Nov;46(11):1198-204. doi: 10.1111/j.1365-4632.2007.03190.x.

Abstract

Background: Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders characterized by a sustained eosinophilia leading to end organ dysfunction. The lymphoproliferative variant of HES is thought to be mediated by an underlying hematologic process that drives eosinophil production through specific cytokines. Eosinophilia is also recognized as a poor prognostic factor in cutaneous T-cell lymphoma (CTCL) in which neoplastic T cells may produce eosinophilopoietic cytokines.

Objective: We report a case of HES with cardiac involvement that developed in the context of erythrodermic mycosis fungoides, discuss the possible relationship between these diseases, and speculate on the role that eosinophils might play as a prognostic factor in CTCL.

Methods: Case report and review of the literature.

Results: CTCL may be added to the group of lymphoproliferative disorders associated with HES.

Conclusion: A patient with erythroderma and concomitant diagnosis of "idiopathic" HES may warrant further investigation for CTCL.

Publication types

  • Case Reports

MeSH terms

  • Cytokines / metabolism
  • Eosinophilia / drug therapy
  • Eosinophilia / metabolism
  • Humans
  • Hypereosinophilic Syndrome* / drug therapy
  • Hypereosinophilic Syndrome* / metabolism
  • Hypereosinophilic Syndrome* / pathology
  • Interferon-alpha / therapeutic use*
  • Lymphoma, T-Cell, Cutaneous* / drug therapy
  • Lymphoma, T-Cell, Cutaneous* / metabolism
  • Lymphoma, T-Cell, Cutaneous* / pathology
  • Male
  • Middle Aged
  • Photopheresis*
  • Skin Neoplasms / drug therapy
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / pathology
  • T-Lymphocyte Subsets / metabolism

Substances

  • Cytokines
  • Interferon-alpha