The lymphoid variant of hypereosinophilic syndrome: study of 21 patients with CD3-CD4+ aberrant T-cell phenotype.
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 TCRγδ 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.
Institut d'Immunologie, Réseau Eosinophile, EA2686 (GL, FL, DL, ML, JT, JEK), Service d'Immunologie Clinique et de Médecine Interne, Centre de Référence Maladies Auto-Immunes Systémiques (GL, DL, LT, NLG, PYH), Institut de Pathologie (MCC), Service de Dermatologie (DSS), Service des Maladies du Sang (FM), Institut de Génétique Médicale, U837 Inserm (CRL), Réseau Eosinophile, U995 Inserm (MC), CHRU de Lille, Université Lille Nord de France, Lille; Service de Dermatologie (MAA, CL), CHU d'Angers, L'UNAM Université, Angers; Service de Dermatologie et Dermatologie Pédiatrique, Centre de Référence pour les Maladies Rares de la Peau, U1035 (AT), CHU de Bordeaux, Bordeaux; Service d'Hématologie (GS) and Service de Radiologie, CREATIS, UMR5220 CNRS, U1044 Inserm (FC), Hôpital Sud, Service de Pneumologie, Centre de Référence pour les Maladies Pulmonaires Rares (CK), Hospices Civils de Lyon, Université Claude Bernard Lyon I, Lyon; Service d'Onco-Hématologie (HM), Centre Hospitalier de La Roche-Sur-Yon, La Roche-Sur-Yon; Service d'Onco-Hématologie (KG), Centre Hospitalier de Beauvais, Beauvais; Service de Médecine Interne (FA), Hôpital Bicêtre, APHP, Université Paris Sud XI, Le Kremlin-Bicêtre; Service d'Hématologie et Immunologie Pédiatrique (AB), Hôpital Robert Debré, APHP, Université Paris Diderot and Service de Pneumologie A, Centre de Compétence Maladies Pulmonaires Rares (RB), Hôpital Bichat, APHP, Paris; Service de Médecine Interne (CMH), Centre Hospitalier d'Annecy, Annecy; Service de Médecine Interne, Hôpital Foch, Suresne; EA4340, Biomarqueurs en Cancérologie et en Onco-Hématologie, Université Versailles-Saint-Quentin-en-Yvelines (JEK), France.
Guillaume
G
Lefèvre
Marie-Christine
MC
Copin
Delphine
D
Staumont-Sallé
Martine
M
Avenel-Audran
Hélène
H
Aubert
Alain
A
Taieb
Gilles
G
Salles
Hervé
H
Maisonneuve
Kamel
K
Ghomari
Félix
F
Ackerman
Fanny
F
Legrand
André
A
Baruchel
David
D
Launay
Louis
L
Terriou
Christian
C
Leclech
Chahera
C
Khouatra
Chafika
C
Morati-Hafsaoui
Myriam
M
Labalette
Raphäel
R
Borie
François
F
Cotton
Noémie Le
NL
Gouellec
Franck
F
Morschhauser
Jacques
J
Trauet
Catherine
C
Roche-Lestienne
Monique
M
Capron
Pierre-Yves
PY
Hatron
Lionel
L
Prin
Jean-Emmanuel
JE
Kahn
D000293:Adolescent; D000328:Adult; D000368:Aged; D017252:CD3 Complex; D015496:CD4-Positive T-Lymphocytes; D002675:Child, Preschool; D005260:Female; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy / Q000235:genetics / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012189:Retrospective Studies; D013601:T-Lymphocytes / Q000276:immunology; D055815:Young Adult
D017252:CD3 Complex