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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Medicine+(Baltimore)
2014 ; 93
(17
): 255-266
Nephropedia Template TP
gab.com Text
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English Wikipedia
The lymphoid variant of hypereosinophilic syndrome: study of 21 patients with
CD3-CD4+ aberrant T-cell phenotype
#MMPMID25398061
Lefèvre G
; Copin MC
; Staumont-Sallé D
; Avenel-Audran M
; Aubert H
; Taieb A
; Salles G
; Maisonneuve H
; Ghomari K
; Ackerman F
; Legrand F
; Baruchel A
; Launay D
; Terriou L
; Leclech C
; Khouatra C
; Morati-Hafsaoui C
; Labalette M
; Borie R
; Cotton F
; Gouellec NL
; Morschhauser F
; Trauet J
; Roche-Lestienne C
; Capron M
; Hatron PY
; Prin L
; Kahn JE
Medicine (Baltimore)
2014[Oct]; 93
(17
): 255-266
PMID25398061
show ga
The CD3-CD4+ aberrant T-cell phenotype is the most described in the lymphoid
variant of hypereosinophilic syndrome (L-HES), a rare form of HES. Only a few
cases have been reported, and data for these patients are scarce. To describe
characteristics and outcome of CD3-CD4+ L-HES patients, we conducted a national
multicentric retrospective study in the French Eosinophil Network. All patients
who met the recent criteria of hypereosinophilia (HE) or HES and who had a
persistent CD3-CD4+ T-cell subset on blood T-cell phenotyping were included.
Clinical and laboratory data were retrospectively collected by chart review.
CD3-CD4+ L-HES was diagnosed in 21 patients (13 females, median age 42 years
[range, 5-75 yr]). Half (48%) had a history of atopic manifestations. Clinical
manifestations were dermatologic (81%), superficial adenopathy (62%),
rheumatologic (29%), gastrointestinal (24%), pulmonary (19%), neurologic (10%),
and cardiovascular (5%). The median absolute CD3-CD4+ T-cell count was 0.35 G/L
(range, 0.01-28.3), with a clonal TCR?? rearrangement in 76% of patients. The
mean follow-up duration after HES diagnosis was 6.9 ± 5.1 years. All patients
treated with oral corticosteroids (CS) (n = 18) obtained remission, but 16
required CS-sparing treatments. One patient had a T-cell lymphoma 8 years after
diagnosis, and 3 deaths occurred during follow-up.In conclusion, clinical
manifestations related to CD3-CD4+ T cell-associated L-HES are not limited to
skin, and can involve all tissue or organs affected in other types of HE.
Contrary to FIP1L1-PDGFRA chronic eosinophilic leukemia patients, CS are always
effective in these patients, but CS-sparing treatments are frequently needed. The
occurrence of T-cell lymphoma, although rare in our cohort, remains a major
concern during follow-up.