The hypereosinophilic syndrome associated with CD4+CD3- helper type 2 (Th2) lymphocytes.
We describe herein the clinical and laboratory manifestations of a unique group of patients (pts) presenting with hypereosinophilic syndrome (HES) who were treated in our medical centers for 4-13 years. Skin biopsies, flow cytometry of peripheral blood mononuclear cells (PBMC), assays for cytokines and immunoglobulin (Ig) production in vitro, and Southern blots of T-cell receptor (TCR) genes were performed. All four pts had a persistent hypereosinophilia (> 1.9 x 10(9)/L) and chronic skin rash. Three of four had elevated IgE, thrombotic manifestations and lung involvement (asthma and/or infiltrates), and one had deforming sero-negative arthritis of the hands. 66-95% of their peripheral T-cells expressed CD4 but not CD3 or TCR molecules on the cell surface membrane. Activated CD4+CD3- cells secreted interleukin (IL)-4 and/or 5, and were required for maximal IgE secretion by autologous B-cells. Two pts had evidence of rearrangement of TCR genes of the CD4+CD3- cells, one of whom died of anaplastic lymphoma. In conclusion, HES with CD4+CD3- lymphocytosis may be associated with high serum IgE, dermatological, pulmonary, thrombotic and rheumatic manifestations which may be due to Th2 effects of CD4+CD3- cells migrating to end organs. Fatal systemic lymphoid malignancy may also develop in some pts with monoclonal expansion of the CD4+CD3- T-cells.
Bank
I
I
Department of Medicine, Laboratory for Immunoregulation, Chaim Sheba Medical Center, Tel-Hashomer 52621 Israel. ibank@post.tau.ac.il
Amariglio
N
N
Reshef
A
A
Hardan
I
I
Confino
Y
Y
Trau
H
H
Shtrasburg
S
S
Langevitz
P
P
Monselise
Y
Y
Shalit
M
M
Rechavi
G
G
D000328:Adult; D000368:Aged; D017252:CD3 Complex / Q000032:analysis*; D015704:CD4 Antigens / Q000032:analysis; D002999:Clone Cells / Q000276:immunology / Q000473:pathology; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000097:blood* / Q000150:complications / Q000209:etiology; D007073:Immunoglobulin E / Q000097:blood; D016130:Immunophenotyping; D008218:Lymphocytosis / Q000097:blood / Q000150:complications / Q000276:immunology; D008297:Male; D008875:Middle Aged; D018418:Th2 Cells / Q000276:immunology / Q000473:pathology*
D017252:CD3 Complex; D015704:CD4 Antigens; D007073:Immunoglobulin E