Uncommon Late Relapse of Angioimmunoblastic T-Cell Lymphoma after 16-Year Remission Period Edit Páyer & Zsófia Miltényi & Zsófia Simon & Lajos Szabados & Katalin Hegyi & Gábor Méhes & Árpád Illés Received: 29 July 2010 / Accepted: 3 November 2011 / Published online: 30 November 2011 # Arányi Lajos Foundation 2011 Abbreviations AITL Angioimmunoblastic T-cell lymphoma EBV Epstein-Barr virus 18 FDG-PET/ CT 18 Fluoro-deoxy-glucose Positron Emission Tomography/Computed Tomography LDH Lactate dehydrogenase CHOP Cyclophosphamide, doxorubicine, vincristine, prednisone COPBLAM Cyclophosphamide, vincristine, prednisone, bleomycin, adriamycin and procarbazin CEVP Cyclophosphamide, vinblastine, epirubicin and prednisone DHAP Dexamethasone, cytarabin, and cisplatin TNF Tumor necrosis factor EBER Epstein-Barr early RNA Introduction Angioimmunoblastic T-cell lymphoma is rare and aggres- sive disorder representing approximately 2% of all non- Hodgkin lyphomas [1], that affects mainly elderly patients [2]. Allergic reactions, infections and chemicals can all play role in the etiology [3], in most of the cases EBV genome can be detected in the environmental B-cells [4–6]. It is suggested that the EBV-related B-cell proliferation is secondary effect due to immunosupression [7]. Most patients present with fever, weight loss, lymphadenop- athy, splenomegaly and skin rushes. Laboratory findings may include anaemia (often autoimmune hemolytic type), thrombo- cytopenia, elevated level of LDH and polyclonal hypergamma- globulinaemia [2]. Nevertheless, several autoimmune phenomena have been reported in association with AITL [8– 15]. Characteristic histological features are effaced nodal architecture, increased vascular proliferation, dilated sinuses, and polymorphous T-cell infiltration with plasmacytes, macro- phages, eosinophils and rarely Sternberg-Reed-like cells [16]. The neoplastic cells commonly express CD3, CD4, CD5, Bcl6, CD10 and CXCL13 on their surfaces [17–20]. Treatment usually includes anthracyclin based combina- tions, like CHOP. The clinical outcome of AITL may be varied with a median survival of less than 3 years and 5-year survival of around 30–35% [21–23]. The currently available staging systems and prognostic scores proved to be inadequate when applied to AITL cases [24]. Although a complete remission rate of 50% can be achieved with polychemotherapy, relapse rates still remain high [13]. Our patient’ s case is highly special as a 16-year-term complete remission and an association with B-cell lymphoma is reported. We would like to highlight the difficulties of treatment of AITL and the necessity of standardized therapeutic protocols. Case Report In 1990 a 53-year-old female patient presented with fever, skin rush and enlarged lymph nodes on the neck. E. Páyer (*) : Z. Miltényi : Z. Simon : Á. Illés 3rd Department of Internal Medicine, University of Debrecen, Medical and Health Science Center, Móricz Zs. krt. 22, 4032 Debrecen, Hungary e-mail: payeredit@vipmail.hu L. Szabados Scanomed Ltd, Nagyerdei krt. 98, Debrecen 4032, Hungary K. Hegyi : G. Méhes University of Debrecen, Medical and Health Science Center, Institute of Pathology, Nagyerdei krt. 98, Debrecen 4032, Hungary Pathol. Oncol. Res. (2012) 18:737–741 DOI 10.1007/s12253-011-9475-7