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

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. Copyright © 2013 Elsevier Inc. All rights reserved.

Citation

J M d'Elbée, M Parrens, P Mercié, M Longy Boursier, C Dieval, A de Mascarel, P Duffau, P Dubus, J C Fricain. Hypereosinophilic syndrome - lymphocytic variant transforming into peripheral T-cell lymphoma with severe oral manifestations. Oral surgery, oral medicine, oral pathology and oral radiology. 2013 Sep;116(3):e185-90

PMID: 23669204

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