Angioimmunoblastic t-cell lymphoma involving the skin and peripheral lymph nodes: case report

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive form of T-cell lymphoma and accounts 1–2% of non-Hodgkin lymphomas. Malignant transformation has been associated with mutations in the epigenetic regulators (TET2, IDH2 and DNMT3A), the Ras homolog gene family member A (RHOA) and T-cell receptor pathway (CD28, FYN, PLCG1, CARD11, P13K elements, CTNNB1, and GTF2I). Infectious diseases associated with AITL include Epstein-Barr virus (EBV), human herpesvirus 6 (HHV-6), human herpesvirus 8 (HHV-8), human immunodeficiency virus (HIV), bacterial and fungal infections [1]. More than 70% of patients with AITL present with constitutional symptoms, and in 50% with a rash: papules, nodules, ulcers, petechiae, and less often - erythroderma. Lymphadenopathy and hepatosplenomegaly are typically present on examination. Pleural effusions, ascites, neurological signs and gastrointestinal symptoms are less common [2]. The study aims to evaluate the clinical, diagnostic, and treatment particularities in a case of AITL. Presentation of the case We present a prospective case involving a 66-year-old woman with angioimmunoblastic T-cell lymphoma involving the skin and peripheral lymph nodes. She had a 7-year history of the disease, its onset being with alopecia and erythematous, pruritic skin lesions. Being established the diagnosis of allergic dermatitis, she administered systemic and topical treatment with corticosteroids, without a positive therapeutic effect. It is important to note that during 10 years she was exposed to toxic substances at work. Concomitant diseases hypertension, autoimmune thyroiditis. The clinical examination revealed generalized alopecia, erythematous plaques with a tendency towards erythroderma, covered with fine, furfuraceous scales, milia and cysts on the face, ear lobes and trunk, as well as poikiloderma, palmoplantar keratoderma. Also, a generalized lymphadenopathy was detected with mobile lymph nodes, painless, hard on palpation, with a diameter of approx. 2 cm. Para-clinical tests showed thrombocytosis and low ESR. The histopathological examination of the lymph nodes revealed lymphoid proliferation consisting of monomorphic cells, with moderate atypia, predominantly around the microvascular bed, and in the skin - discrete subepithelial lymphocytic inflammatory infiltrate. Immunohistochemistry (IHC) showed: positive pan-lymphocytic reaction for CD43, CD45, positive nuclear reaction in lymphoid cells for BCL2 and Ki67, positive membrane reaction in B lymphocytes for CD20, CD19 and in stellate cells of the lymphoid follicles, positive reaction in T lymphocytes for CD5. The chemotherapeutic treatment according to the CHOP scheme (cyclophosphamide, doxorubicin, vincristine, prednisolone), administered in 3 courses, led to the partial involution of the erythematous-scaly lesions and palmoplantar keratoderma, as well as to the decrease in the size of the lymph nodes. On alopecia, however, it had an insignificant effect. Conclusions We note that in the described case, AITL mimicked initially an allergic dermatitis, which delayed the establishment of a correct diagnosis and the initiation of specific treatment. We believe that the professional activity in an environment with toxic substances had an important impact in triggering this pathology. The histopathological and IHC tests were decisive in establishing the diagnosis. The initiated chemotherapeutic treatment according to the CHOP scheme determined a partial involution of both skin lesions and lymphadenopathy, but for alopecia it had an insignificant effect.