More than 13 years of hypereosinophila associated with clonal CD3-CD4+ lymphocytosis of TH2/TH0 type.
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 TH2 type but was clearly TH2/TH0 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.
Sugimoto
Koichi
K
Department of Internal Medicine, Juntendo University School of Medicine, Tokyo, Japan. ksugimot@med.juntendo.ac.jp
Tamayose
Kenji
K
Sasaki
Makoto
M
Danbara
Takashi
T
Aikawa
Yosuke
Y
Ogawa
Hideoki
H
Sato
Naotake
N
Tashiro
Eiji
E
Oshimi
Kazuo
K
D000328:Adult; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology*; D005260:Female; D005500:Follow-Up Studies; D015321:Gene Rearrangement; D019674:Genes, T-Cell Receptor beta; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000097:blood / Q000276:immunology*; D007073:Immunoglobulin E / Q000097:blood; D018655:Lymphocyte Count; D008214:Lymphocytes / Q000276:immunology*; D008218:Lymphocytosis / Q000097:blood*; D008875:Middle Aged; D012016:Reference Values; D015183:Restriction Mapping; D012883:Skin Ulcer / Q000097:blood; D013601:T-Lymphocytes / Q000276:immunology*
D007073:Immunoglobulin E