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10.1097/MD.0000000000000088

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suck abstract from ncbi


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pmid25398061
      Medicine+(Baltimore) 2014 ; 93 (17 ): 255-266
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  • 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.
  • |*CD3 Complex [MESH]
  • |*CD4-Positive T-Lymphocytes [MESH]
  • |Adolescent [MESH]
  • |Adult [MESH]
  • |Aged [MESH]
  • |Child, Preschool [MESH]
  • |Female [MESH]
  • |Humans [MESH]
  • |Hypereosinophilic Syndrome/drug therapy/genetics/*immunology [MESH]
  • |Male [MESH]
  • |Middle Aged [MESH]
  • |Phenotype [MESH]
  • |Retrospective Studies [MESH]
  • |T-Lymphocytes/immunology [MESH]


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