Gastric Non-Hodgkin's Lymphoma Associated with Behcet's Disease.

Abstract

The first case of primary gastric lymphoma associated with Beh^et's disease is reported. A 43year-old woman, who had been treated for Beh^et's disease for 15 years, was diagnosed as lowgrade and small lymphocytic malignant lymphomaof the stomach. Although the patient had been treated for Beh^et's disease, chemotherapy was performed and a complete remission of gastric lymphomawas achieved. The literature regarding the association of malignant diseases with Beh^et's disease is briefly reviewed.

Malignant lymphoma associated with Behçet’s disease: A report of 2 cases

“…Nevertheless, the worldwide prevalence of BD and the possible association between malignant diseases and BD remain unclear. Only 96 cases of malignancy associated with BD have been reported; 63 cases had solid organ tumors, 16 cases had hematological malignancies, and 17 cases had lymphoid malignancies [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] (Table 1).…”

“…The major immunologic features of BD are increased T-cell and B-cell response to heat-shock proteins, increased neutrophil activity, and alteration in cytokine levels [4]. Additionally, helper T-cells and macrophages are known to exhibit immunological dysfunction, as well as neutrophils [10]. Development of malignancy in autoimmune diseases and vasculitides could be related to immune regulation abnormality, T-cell deficiency, or over proliferation of B-cells, which may trigger the malignant transformation of cells [20].…”

“…The treatment of BD is empirical and usually includes systemic corticosteroids, and immunosuppressive and/or cytotoxic agents [4]. The literature contains few reports of the coexistence of malignant disease in BD patients-to date only 96 cases (excluding myelodysplastic syndromes) have been reported, of which 17 are lymphoid malignancy [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. Malignant transformation in BD might be due to the autoimmune nature of BD, triggering infectious agents, or the drugs used for the management of BD.…”

Ulcerações orais e genitais como manifestação inicial de leucemia em criança

“…Dentre os fatores que podem predispor ao aparecimento de neoplasias, inclui-se o uso de agentes imunossupressores como clorambucil, ciclofosfamida ou colchicina (12,13,26,27) . Entretanto, drogas imunossupressoras nem sempre foram utilizadas nos casos associados a neoplasias (14,15) .…”

“…A doença é descrita na literatura associada a tumores sólidos e a malignidades hematológicas, principalmente à leucemia mielóide crônica, linfomas e síndro-me mielodisplásica, algumas vezes precedendo o quadro e, outras vezes, aparecendo posteriormente ao mesmo (9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) . A associação com leucemia mielóide aguda é raramente descrita e não foram encontrados trabalhos na literatura associando tais doenças na faixa etária pediátrica.…”