Advertisement

International Journal of Hematology

, Volume 75, Issue 3, pp 281–284 | Cite as

More Than 13 Years of Hypereosinophila Associated With Clonal CD3CD4+ Lymphocytosis Of TH2/TH0 Type

  • Koichi Sugimoto
  • Kenji Tamayose
  • Makoto Sasaki
  • Takashi Danbara
  • Yosuke Aikawa
  • Hideoki Ogawa
  • Naotake Sato
  • Eiji Tashiro
  • Kazuo Oshimi
Case Report

Abstract

A 65-year-old Japanese woman was referred to our hospital because of hypereosinophilia lasting for more than 10 years, and skin ulceration, especially on the hands. Closer examination revealed the clonal proliferation of CD3-CD4+ T-lymphocytes. The patient had generalized pruritus without severe end-organ involvement and high serum levels of IgE. A diagnosis of monoclonal CD3-CD4+ T-lymphocyte-associated idiopathic hypereosinophilic syndrome (HES) was made based on these findings.This case showed that this newly recognized entity of HES is not restricted to Western countries. The abnormal T-cell clone was not merely T h 2 type but was clearly T h 2/T h 0 type. Although this disease is considered prelymphoma, this patient did not develop lymphoma during more than 13 years of follow-up.Therefore, in some patients, clonal CD3-CD4+ lymphocyte-associated HES may take a more indolent course. In this subgroup, the control of clinical manifestations seems very important. In the present case, treatment with hydroxyurea quite dramatically improved the intractable skin manifestations, although the treatment lessened only the number of peripheral eosinophils and not the number of clonal CD3-CD4+ T-lymphocytes.

Key words

IgE Hydroxyurea Hypereosinophilia ThTh

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Romagnani S. Th1 and Th2 in human diseases.Clin Immunol Immunopathol. 1996;80:225–235.CrossRefPubMedGoogle Scholar
  2. 2.
    Roboz GL, Rafii S. Interleukin-5 and the regulation of eosinophil production.Curr Opin Hematol. 1999;6:164–168.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Sanderson CJ. Interleukin-5, eosinophils, and disease.Blood. 1992;79:3101–3109.PubMedGoogle Scholar
  4. 4.
    Rothenberg ME. Eosinophilia.N Engl J Med. 1998;338:1592–1600.CrossRefPubMedGoogle Scholar
  5. 5.
    Cogan E, Schandene L, Crusiaux A, et al. Clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome.N Engl J Med. 1994;330:535–538.CrossRefPubMedGoogle Scholar
  6. 6.
    Brugnoni D, Airo P, Rossi G, et al. A case of hypereosinophilic syndrome is associated with the expansion of a CD3-CD4+ T-cell population able to secrete large amounts of interleukin-5.Blood. 1996;87:1416–1422.PubMedGoogle Scholar
  7. 7.
    Bank I, Reshef A, Beniaminov M, Rosenthal E, Rechavi G, Mon-selise Y. Role of γ/δ T cells in a patient with CD4+CD3- lymphocytosis, hypereosinophilia, and high levels of IgE.J Allergy Clin Immunol. 1998;102:621–630.CrossRefPubMedGoogle Scholar
  8. 8.
    Picker LJ, Singh MK, Zdraveski Z, et al. Direct demonstration of cytokine synthesis heterogeneity among human memory/effector T cells by flow cytometry.Blood. 1995;86:1408–1419.PubMedGoogle Scholar
  9. 9.
    Bendelac A, Schwartz RH. Th0 cells in the thymus: the question of T-helper lineages.Immunol Rev. 1991;123:169–188.CrossRefPubMedGoogle Scholar
  10. 10.
    Roufosse F, Schandené L, Sibille C, et al. Clonal Th2 lymphocytes in patients with the idiopathic hypereosinophilic syndrome.Br J Haematol. 2000;109:540–548.CrossRefPubMedGoogle Scholar
  11. 11.
    Kitano K, Ichikawa N, Mahbub B, et al. Eosinophilia associated with proliferation of CD3+4-8-αβ+ T cells with chromosome 16 anomalies.Br J Haematol. 1996;92:315–317.CrossRefPubMedGoogle Scholar
  12. 12.
    Brugnoni D, Airo P, Tosoni C, et al. CD3-CD4+ cells with a Th2-like pattern of cytokine production in the peripheral blood of a patient with cutaneous T cell lymphoma.Leukemia. 1997;11:648–653.CrossRefGoogle Scholar
  13. 13.
    Simon HU, Plotz SG, Dummer R, Blaser K. Abnormal clones of T cells producing interleukin-5 in idiopathic eosinophilia.N Engl J Med. 1999;341:1112–1120.CrossRefPubMedGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2002

Authors and Affiliations

  • Koichi Sugimoto
    • 1
  • Kenji Tamayose
    • 1
  • Makoto Sasaki
    • 1
  • Takashi Danbara
    • 2
  • Yosuke Aikawa
    • 3
  • Hideoki Ogawa
    • 3
  • Naotake Sato
    • 4
  • Eiji Tashiro
    • 5
  • Kazuo Oshimi
    • 1
  1. 1.Divisions of HematologyJuntendo University School of MedicineTokyo
  2. 2.Divisions of Pulmonary Medicine, Department of Internal MedicineJuntendo University School of MedicineTokyo
  3. 3.Departments of DermatologyJuntendo University School of MedicineTokyo
  4. 4.Departments of Clinical PathologyJuntendo University School of MedicineTokyo
  5. 5.Otsuka Assay LaboratoriesTokyoJapan

Personalised recommendations