Hypereosinophilia with abnormal T cells, trisomy 7 and elevated TARC serum level.
The idiopathic hypereosinophilic syndrome (HES) is a rare heterogeneous disorder, characterized by persistent blood eosinophilia with possible organ involvement. We describe here the case of a 20-year-old atopic male presenting chronic hypereosinophilia and eczema since childhood. Biological findings included hypereosinophilia (9.5 x 10(9)/L), hyperlymphocytosis (10.9 x 10(9)/L), polyclonal hypergammaglobulinemia and elevated IgE serum level. Flow cytometric analysis of blood lymphoid cells showed a population of CD2+CD3-CD4+TCRab-TCRgd- lymphocytes. These cells displayed a Th0/Th2 cytokine profile, and a clonal TCR rearrangement pattern. A high serum TARC level was observed. Karyotype studies on blood stimulated culture or lymph nodes revealed a cellular hyperdiploïd clone 47, XY, +7. To our knowledge, this chromosomal aberration has never been reported in such case.
Roumier
A S
AS
INSERM U547, Institut Pasteur, 1 rue du Professeur Calmette, BP 245, 59019 LILLE Cedex, FRANCE. asroumier@yahoo.fr
Grardel
N
N
Laï
J L
JL
Becqueriaux
I
I
Ghomari
K
K
de Lavareille
A
A
Roufosse
F
F
Prin
L
L
Capron
M
M
D000328:Adult; D054414:Chemokine CCL17; D019742:Chemokines, CC / Q000097:blood*; D002897:Chromosomes, Human, Pair 7; D002999:Clone Cells; D016207:Cytokines / Q000032:analysis / Q000097:blood; D015329:Gene Rearrangement, T-Lymphocyte; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000096:biosynthesis; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000175:diagnosis* / Q000276:immunology*; D016130:Immunophenotyping; D015848:Interleukin-5 / Q000096:biosynthesis; D007621:Karyotyping; D008214:Lymphocytes / Q000145:classification; D008218:Lymphocytosis / Q000175:diagnosis; D008297:Male; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology*; D014314:Trisomy
C106230:CCL17 protein, human; D054414:Chemokine CCL17; D019742:Chemokines, CC; D016207:Cytokines; D015848:Interleukin-5; D016178:Granulocyte-Macrophage Colony-Stimulating Factor