Severe atherosclerosis of the aorta and development of peripheral T‐cell lymphoma in an adolescent with angiolymphoid hyperplasia with eosinophilia

• Published in: British journal of dermatology (1951) Vol. 152; no. 5; pp. 1033 - 1038
• Main Authors: Andreae, J., Galle, C., Magdorf, K., Staab, D., Meyer, L., Goldman, M., Querfeld, U.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Science Ltd 01-05-2005; Blackwell; Oxford University Press
• Subjects: Adolescent; angiolymphoid hyperplasia with eosinophilia; Angiolymphoid Hyperplasia with Eosinophilia - complications; Angiolymphoid Hyperplasia with Eosinophilia - immunology; Aorta, Abdominal; Aortic Valve Stenosis - etiology; Aortic Valve Stenosis - immunology; Arteriosclerosis - etiology; Arteriosclerosis - immunology; atherosclerosis; Atherosclerosis (general aspects, experimental research); Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; cytokines; Cytokines - blood; Dermatology; Female; Hematologic and hematopoietic diseases; Humans; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; lymphoma; Lymphoma, T-Cell, Peripheral - etiology; Lymphoma, T-Cell, Peripheral - immunology; Medical sciences; T-Lymphocyte Subsets - immunology; Human; Hypertension; Skin disease; Dermatology; Cytokine; lymphoma; Herpes; Cardiovascular disease; Malignant hemopathy; angiolymphoid hyperplasia with eosinophilia; Non Hodgkin lymphoma; Eosinophilia; Peripheral T cell lymphoma; Infection; Vascular disease; Case study; Association; Lymphoproliferative syndrome; Viral disease; Adolescent; Atherosclerosis; Aorta; cytokines; Angiolymphoid hyperplasia
• ISSN: 0007-0963; 1365-2133
• DOI: 10.1111/j.1365-2133.2005.06421.x

Summary
We report an adolescent girl with a history of angiolymphoid hyperplasia with eosinophilia (ALHE) diagnosed at the age of 10 years. The patient also suffered from chronic persistent multiresistant herpes simplex virus infection. Atherosclerotic occlusive disease of the abdominal aorta and its major branches was observed at the age of 17 years, necessitating vascular surgical intervention 1 year later because of disease progression. Histological examination of the aorta disclosed widespread atherosclerosis and high levels of gene expression of both T‐helper cell type (Th) 1‐ and Th2‐derived cytokines. This suggests that a highly stimulated systemic immune response including increased production of both Th1‐ and Th2‐derived cytokines such as interferon‐γ and interleukin‐4 may result in severe atherosclerotic lesions at a very young age. In addition, the patient developed a peripheral T‐cell lymphoma at the age of 18 years. Neither systemic atherosclerosis nor T‐cell lymphoma has been reported in association with ALHE. It is suggested that a highly stimulated dysfunctional immune response may play a key role in persistent inflammatory disease and premature development of atherosclerosis as well as malignant transformation of T cells.