Gernert Michael, Schwaneck Eva Christina, Schmalzing Marc
Medizinische Klinik 2, Schwerpunkt Rheumatologie/Klinische Immunologie, Universitätsklinikum Würzburg, Oberdürrbacher Str. 6, 97080, Würzburg, Deutschland.
Medizinisches Versorgungszentrum Rheumatologie und Autoimmunmedizin Hamburg GmbH, Mönckebergstr. 27, 20095, Hamburg, Deutschland.
Z Rheumatol. 2025 Feb;84(1):48-56. doi: 10.1007/s00393-024-01611-x. Epub 2025 Jan 24.
Neutropenia in rheumatoid arthritis (RA) is a problem that often needs to be addressed. Side effects of basic antirheumatic treatment, infections or substrate deficiencies are common causes; however, T‑cell large granular lymphocytic (T-LGL) leukemia, a mature T‑cell neoplasm, can also lead to autoimmune cytopenia. The T‑LGL leukemia can be associated not only with RA but also with other autoimmune diseases or neoplasms. Correspondingly, increases in clonal T cells, natural killer T (NKT) cells and LGL cells are found in the peripheral blood. A T‑cell receptor PCR and flow cytometry (or at least a blood smear) are therefore necessary to diagnose T‑LGL leukemia. The presence of clonal T cells alone is usually not pathological. A distinction must be made from Felty's syndrome (consisting of the clinical triad of arthritis, leukopenia, splenomegaly), which does not require the two T‑LGL leukemia criteria mentioned. The treatment for both entities (with underlying RA) is methotrexate and, if insufficiently effective, rituximab.
类风湿关节炎（RA）中的中性粒细胞减少是一个常常需要解决的问题。基础抗风湿治疗的副作用、感染或底物缺乏是常见原因；然而，T细胞大颗粒淋巴细胞（T-LGL）白血病，一种成熟的T细胞肿瘤，也可导致自身免疫性血细胞减少。T-LGL白血病不仅可与RA相关，还可与其他自身免疫性疾病或肿瘤相关。相应地，在外周血中可发现克隆性T细胞、自然杀伤T（NKT）细胞和LGL细胞增多。因此，诊断T-LGL白血病需要进行T细胞受体PCR和流式细胞术（或至少进行血涂片检查）。仅克隆性T细胞的存在通常并无病理学意义。必须与费尔蒂综合征（由关节炎、白细胞减少、脾肿大的临床三联征组成）相鉴别，费尔蒂综合征并不需要满足上述提到的T-LGL白血病的两条标准。这两种情况（伴有潜在RA）的治疗方法均为甲氨蝶呤，若疗效欠佳，则使用利妥昔单抗。
