The Lymphoid Variant of Hypereosinophilic Syndrome
| Autor: | Martine Avenel-Audran, François Cotton, Chafika Morati-Hafsaoui, Hervé Maisonneuve, Guillaume Lefèvre, Marie-Christine Copin, Franck Morschhauser, Noémie Le Gouellec, Felix Ackerman, André Baruchel, Catherine Roche-Lestienne, Gilles Salles, Jean-Emmanuel Kahn, Louis Terriou, Alain Taieb, Lionel Prin, Delphine Staumont-Sallé, Hélène Aubert, Myriam Labalette, Raphael Borie, Fanny Legrand, Chahéra Khouatra, Kamel Ghomari, David Launay, Monique Capron, Christian Leclech, Jacques Trauet, Pierre-Yves Hatron |
|---|---|
| Rok vydání: | 2014 |
| Předmět: |
Pathology
medicine.medical_specialty Chronic eosinophilic leukemia Hypereosinophilic syndrome business.industry Hypereosinophilia Retrospective cohort study General Medicine Eosinophil medicine.disease Gastroenterology 3. Good health Lymphoma medicine.anatomical_structure Internal medicine Cohort medicine medicine.symptom Young adult business |
| Zdroj: | Medicine. 93:255-266 |
| ISSN: | 0025-7974 |
| DOI: | 10.1097/md.0000000000000088 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|