The lymphoid variant of hypereosinophilic syndrome: study of 21 patients with CD3-CD4+ aberrant T-cell phenotype.

摘要: The CD3-CD4+ aberrant T-cell phenotype is the most described in lymphoid variant of hypereosinophilic syndrome (L-HES), a rare form HES. Only few cases have been reported, and data for these patients are scarce. To describe characteristics outcome L-HES patients, we conducted national multicentric retrospective study French Eosinophil Network. All who met recent criteria hypereosinophilia (HE) or HES had persistent subset on blood phenotyping were included. Clinical laboratory retrospectively collected by chart review. was diagnosed 21 (13 females, median age 42 years [range, 5-75 yr]). Half (48%) history atopic manifestations. manifestations dermatologic (81%), superficial adenopathy (62%), rheumatologic (29%), gastrointestinal (24%), pulmonary (19%), neurologic (10%), cardiovascular (5%). absolute count 0.35 G/L (range, 0.01-28.3), with clonal TCRγδ rearrangement 76% patients. mean follow-up duration after diagnosis 6.9 ± 5.1 years. treated oral corticosteroids (CS) (n = 18) obtained remission, but 16 required CS-sparing treatments. One patient lymphoma 8 diagnosis, 3 deaths occurred during follow-up.In conclusion, clinical related to T cell-associated not limited skin, can involve all tissue organs affected other types HE. Contrary FIP1L1-PDGFRA chronic eosinophilic leukemia CS always effective treatments frequently needed. occurrence lymphoma, although our cohort, remains major concern follow-up.