title: Hypereosinophilic syndrome - lymphocytic variant transforming into peripheral T-cell lymphoma with severe oral manifestations.
abstract: Hypereosinophilic syndrome (HES) is a rare disease defined by organ damage directly attributable to hypereosinophilia of any type. Here, we report for the first time the case of a patient with a lymphocytic type of HES (HES-L) who had liver, skin, spleen, lung, bone marrow, digestive track, and mouth involvement. Associated T-cells displayed an aberrant CD30+ phenotype and were monoclonal. Thymus activated and regulated chemokine serum level was positive. Despite steroids (Cortancyl 20 mg [Sanofi Aventis, France], imatinib mesylate [Glivec 400 mg; Novartis Europharm], interferon alpha 2A [Roferon-A 3 MUI/0.5 ml; Roche]) and other lines of therapy including imatinib mesylate treatment, an oral necrotic lesion developed, and finally progressed into a peripheral CD30+ T-cell lymphoma. CHOP chemotherapy (cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone), interferon-α, and mepolizumab were ineffective. Although progression into peripheral T-cell lymphoma is documented as a rare complication of HES-L, severe oral extension of HES-L is described for the first time.
authors.affiliation: Department of Dentistry and Oral Health, University Hospital of Bordeaux, Bordeaux, France. jmdelbee64@gmail.com
authors.forename: J M
authors.initials: JM
authors.lastname: d'Elbée
authors.forename: M
authors.initials: M
authors.lastname: Parrens
authors.forename: P
authors.initials: P
authors.lastname: Mercié
authors.forename: M
authors.initials: M
authors.lastname: Longy Boursier
authors.forename: C
authors.initials: C
authors.lastname: Dieval
authors.forename: A
authors.initials: A
authors.lastname: de Mascarel
authors.forename: P
authors.initials: P
authors.lastname: Duffau
authors.forename: P
authors.initials: P
authors.lastname: Dubus
authors.forename: J C
authors.initials: JC
authors.lastname: Fricain
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D017809:Fatal Outcome; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications* / Q000473:pathology; D016411:Lymphoma, T-Cell, Peripheral / Q000209:etiology* / Q000473:pathology; D008297:Male; D009061:Mouth Mucosa / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D000911:Antibodies, Monoclonal