AUTOINFLAMMATORY DISEASES IN HEMATOLOGY FROM DIAGNOSIS TO TREATMENT

AUTOINFLAMMATORY_DISEASES_IN_HEMATOLOGY_FROM_DIAGNOSIS_TO_TREATMENTkapak

ANGIOIMMUNOBLASTIC T CELL LYMPHOMA (AITL)

Fatma Keklik Karadağ

İzmir City Hospital, Department of Hematology, İzmir, Türkiye

Keklik Karadağ F. Angioimmunoblastic T Cell Lymphoma (AITL). In: Kurt Yüksel M, editor. Autoinflammatory Diseases in Hematology from Diagnosis to Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.197-209.

ABSTRACT

Angioimmunoblastic T cell Lymphoma (AITL) which is a rare disease represents approximately 15– 30% of peripheral T cell lymphoma (PTCL). Elderly patients and male gender are commonly affected by AITL. It is known as many faces’ disease due to its association of numerous immunological and inflammatory diseases. AITL can present with a wide range of clinical symptoms, including fever, skin rashes, anemia, and polyarthritis, as well as non-specific symptoms that may include lymphoid organs. One of three patients have polyclonal hypergammaglobulinemia or monoclonal gammopathy. Most of the patients have advanced stage disease with bone marrow involvement at the time of diagnosis. For diagnosis of AITL which is derived from mature T follicular helper cells (TFH), polymorphous infiltration of neoplastic T cells and a significant proliferation of follicular dendritic cells are seen in the lymph node biopsy. Pan-T cell antigens CD2, CD3, CD5, and CD7 are expressed by neoplastic T cells; one or more of these markers is frequently abnormally lost or downregulated. Additionally, the expression of TFH antigens such as CD10, PD-1, ICOS, CD200, and CXCL13 are found in immunohistochemical staining. The prognosis for AITL is typically regarded as poor, with 5-year overall survival (OS) ranging from 32-41%. Anthracycline based regimen ± etoposide is widely preferred treatment option for the frontline therapy. The use of autologous stem cell transplantation (ASCT) for PTCL in the frontline context is still debatable in the absence of data from randomized clinical trials. For the majority of PTCL subtypes, the current recommendation to take ASCT into account in complete remission is primarily based on the findings of a limited number of phase 2, single-arm studies. Besides multiagent salvage chemotherapy followed by ASCT, new drugs such as brentuximab vedotin, histone deacetylase inhibitors (romidepsin, belinostat), bendamustine, pralatrexate, lenalidomide and azacytidine have been used for relapsed refractory disease so far. Although reported OS and progression free survival rates were promising with these novel agents, allogeneic stem cell transplantation is still the only curative option for eligible patients.

Keywords: Angioimmunoblastic T cell lymphoma; Hypergammaglobulinemia; Peripheral T cell lymphoma; T follicular helper cell

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