Diz Lopes Mariana, Marques Gomes Carlos, Santos Inês, Martins-Rocha Teresa, Bernardes Miguel, Pinto José, Costa Lúcia
Centro Hospitalar Universitário de São João, Porto, Portugal.
Centro Hospitalar Tondela-Viseu, Viseu, Portugal.
ARP Rheumatol. 2024 Oct-Dec;3(4):320-323. doi: 10.63032/DNPZ5424.
Large Granular Lymphocytic (LGL) leukemia is a rare lymphoproliferative disorder with a peculiar association with Rheumatoid Arthritis (RA). The most common feature is neutropenia and patients can have splenomegaly, resembling Felty's Syndrome. These diseases have similar clinical and laboratory abnormalities, but the diagnosis of T-cell LGL (T-LGL) leukemia requires evidence of clonality. Even though T-LGL leukemia is indolent in most cases, inadequate treatment when it is indicated can lead to significant morbidity and mortality, mainly associated with recurrent infections. We present two clinical cases that emphasize the emerging role of Rituximab as an effective therapeutic option in patients with T-LGL and RA.
大颗粒淋巴细胞（LGL）白血病是一种罕见的淋巴细胞增殖性疾病，与类风湿性关节炎（RA）有着特殊关联。最常见的特征是中性粒细胞减少，患者可能出现脾肿大，类似费尔蒂综合征。这些疾病具有相似的临床和实验室异常表现，但T细胞大颗粒淋巴细胞（T-LGL）白血病的诊断需要克隆性证据。尽管大多数情况下T-LGL白血病进展缓慢，但在需要治疗时若治疗不当可导致严重的发病率和死亡率，主要与反复感染有关。我们展示两例临床病例，强调利妥昔单抗作为T-LGL和RA患者有效治疗选择的新作用。
