Hematology and Stem Cell Transplantation Unit, AOUC Policlinico, Bari, Italy.
Pathology Unit, AOUC Policlinico, Bari, Italy.
Clin Lymphoma Myeloma Leuk. 2022 Aug;22(8):e745-e750. doi: 10.1016/j.clml.2022.04.002. Epub 2022 Apr 8.
Lymphoproliferative diseases arise when the physiological mechanisms that control the proliferation of T and B lymphocytes are disrupted, resulting in an uncontrolled and autonomous increase in immune cells leading to lymphocytosis and lymphadenopathy, and often to the involvement of extranodal sites. The differential diagnosis of malignant T cell tumors involves other neoplasms and non-clonal T cell proliferations. Immunological markers are essential, as a first step, to distinguish between T-cell and non-T-cell disorders. It must be established based on the configuration of the genes of the TCR chain to rule out that the picture is not reactive to other underlying diseases. This clinical review and accompanying case reports highlight the diagnostic challenges associated with indolent lymphoproliferative T-cell disorders, which in many cases may represent the clinical manifestation of a single disease. Particularly we focus on gastrointestinal manifestations that could be expression either of lymphoproliferative disorder either of autoimmune disease either of both. The correct interpretation of the different clinical situations can help in the diagnostic and therapeutic process.
当控制 T 和 B 淋巴细胞增殖的生理机制被破坏时，就会出现淋巴增殖性疾病，导致免疫细胞不受控制和自主增加，导致淋巴细胞增多和淋巴结病，并且常常涉及淋巴结外部位。恶性 T 细胞肿瘤的鉴别诊断涉及其他肿瘤和非克隆性 T 细胞增殖。免疫标志物是必不可少的，作为第一步，以区分 T 细胞和非 T 细胞疾病。必须根据 TCR 链基因的构型来确定，以排除该图像不是对其他潜在疾病的反应。本临床综述和随附的病例报告强调了与惰性淋巴增殖性 T 细胞疾病相关的诊断挑战，在许多情况下，这些疾病可能代表单一疾病的临床表现。特别是，我们专注于胃肠道表现，这些表现可能是淋巴增殖性疾病的表现，也可能是自身免疫性疾病的表现，或者两者兼有。正确解释不同的临床情况有助于诊断和治疗过程。
