Alfano Gaetano, Ferrari Annachiara, Fontana Francesco, Damiano Francesca, Solazzo Andrea, Mori Giacomo, Cappelli Gianni
Surgical, Medical and Dental Department of Morphologic Sciences, Section of Nephrology, University of Modena and Reggio Emilia, Modena, Italy; Nephrology Dialysis and Transplant Unit, University Hospital of Modena, Modena, Italy.
Surgical, Medical and Dental Department of Morphologic Sciences, Section of Nephrology, University of Modena and Reggio Emilia, Modena, Italy.
Transplant Proc. 2020 Jun;52(5):1617-1618. doi: 10.1016/j.transproceed.2020.02.183. Epub 2020 Jun 4.
T-cell large granular lymphocytic (T-LGL) leukemia is a rare clonal proliferation presenting with cytopenia, splenomegaly, and autoimmune manifestations. It has rarely been described in recipients of solid organ transplants. We report the clinical case of a young kidney transplant recipient that developed T-LGL leukemia 3 years after kidney transplantation. The disorder manifested with a severe form of autoimmune hemolytic anemia in the absence of other laboratory abnormalities. The anemia was successfully treated with an intense course of corticosteroids ands witch of immunosuppressive therapy from a calcineurin inhibitor to sirolimus, a mammalian target of rapamycin inhibitor. Our case shows that autoimmune hemolytic anemia can be a life-threatening manifestation of T-LGL disease. The antiproliferative effects of sirolimus may be useful in the treatment of symptoms of T-LGL leukemia in kidney transplantation.
T细胞大颗粒淋巴细胞(T-LGL)白血病是一种罕见的克隆性增殖疾病,表现为血细胞减少、脾肿大和自身免疫表现。实体器官移植受者中很少有相关描述。我们报告了一例年轻肾移植受者在肾移植3年后发生T-LGL白血病的临床病例。该疾病表现为严重的自身免疫性溶血性贫血,且无其他实验室异常。通过强化使用皮质类固醇疗程以及将免疫抑制治疗从钙调神经磷酸酶抑制剂转换为雷帕霉素抑制剂西罗莫司,成功治疗了贫血。我们的病例表明,自身免疫性溶血性贫血可能是T-LGL疾病的危及生命的表现。西罗莫司的抗增殖作用可能有助于治疗肾移植中T-LGL白血病的症状。