Haematology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
Haematology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
BMJ Case Rep. 2020 Sep 7;13(9):e235851. doi: 10.1136/bcr-2020-235851.
A 35-year-old woman presented with a widespread petechial rash and pancytopenia. She underwent simultaneous pancreas and kidney transplantation for type 1 diabetes 8 years previously followed by a renal transplant 1 year prior to presentation, and was taking tacrolimus as long-term immunosuppression. The full blood count showed haemoglobin 97 g/L, platelet count 2×10/L and neutrophil count 0.22×10/L. Peripheral blood film examination confirmed genuine thrombocytopenia in the absence of any haemolytic or malignant features. Serological testing identified autoantibodies against all three blood lineages, consistent with a diagnosis of autoimmune pancytopenia. Treatment with steroids, intravenous immunoglobulins, romiplostim and mycophenolate mofetil achieved only fleeting remissions. Blood counts eventually normalised following the administration of rituximab and a change from tacrolimus to ciclosporin immunosuppression. Cytopenias are a well-recognised complication of post-transplantation care but we believe this to be the first reported case of autoimmune pancytopenia following solid organ transplantation. In this case report, we discuss the approach to investigation of haematological abnormalities post-transplant and the rationale for, and outcome of, the management of this rare case.
一位 35 岁女性因广泛出现瘀点疹和全血细胞减少症就诊。她 8 年前因 1 型糖尿病接受了胰肾联合移植,1 年前又进行了肾移植,并长期接受他克莫司免疫抑制治疗。全血细胞计数显示血红蛋白 97g/L,血小板计数 2×10/L,中性粒细胞计数 0.22×10/L。外周血涂片检查证实存在真正的血小板减少症,且无任何溶血性或恶性特征。血清学检测发现针对所有三种血细胞谱系的自身抗体,符合自身免疫性全血细胞减少症的诊断。尽管使用了皮质类固醇、静脉注射免疫球蛋白、罗米司亭和霉酚酸酯等治疗,但仅短暂缓解。在给予利妥昔单抗并将他克莫司免疫抑制改为环孢素后,血液计数最终恢复正常。细胞减少症是移植后护理中常见的并发症,但我们认为这是首例实体器官移植后发生自身免疫性全血细胞减少症的报告病例。在本病例报告中,我们讨论了移植后血液学异常的调查方法,以及这种罕见病例的治疗原则和结果。