Hypereosinophilic syndrome – lymphocytic variant transforming into peripheral T-cell lymphoma with severe oral manifestations

Autor: Pierre Dubus, M. Longy Boursier, A. de Mascarel, Pierre Duffau, Patrick Mercié, Jean-Marie d’Elbée, Marie Parrens, Céline Dieval, Jean-Christophe Fricain
Rok vydání: 2013
Předmět:
Zdroj: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology. 116:e185-e190
ISSN: 2212-4403
DOI: 10.1016/j.oooo.2013.03.017
Popis: 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.
Databáze: OpenAIRE