Division of Internal Medicine, Mayo Clinic, Jacksonville, Florida, USA.
Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, Florida, USA.
Transfusion. 2024 Apr;64(4):755-760. doi: 10.1111/trf.17748. Epub 2024 Mar 1.
This case describes passenger lymphocyte syndrome (PLS) generating human platelet antigen 1a (HPA-1a) alloantibodies against the recipient's platelets after liver transplant. Given the rarity of PLS, especially in liver transplant with HPA-1a alloantibodies, disease course and management options are poorly described.
The patient had cirrhosis secondary to nonalcoholic steatohepatitis complicated by hepatocellular carcinoma, encephalopathy, and severe ascites. The model for end-stage liver disease (MELD) score was 15 at presentation. The patient developed hepatic artery thrombosis after an orthotopic liver transplant and was relisted for transplant with a MELD score of 40. The patient received a hepatitis C virus antibody positive, hepatitis C virus nucleic amplification test positive donor liver on postoperative day (POD) 7 after first transplant. On POD 7 after the second transplant, the patient developed profound thrombocytopenia refractory to platelet infusion. They were found to have serum antibody to HPA-1a based upon serum platelet alloantibody testing. The donor was later found to be negative for HPA-1a by genetic testing. However, the patient's native platelets were HPA-1a positive. The patient was diagnosed with PLS.
The patient's treatment course included 57 units of platelets transfused, emergency splenectomy, rituximab, plasma exchange, intravenous immunoglobulin (IVIG), eltrombopag, romiplostim, and efgartigimod.
The synergistic effect of efgartigimod with eltrombopag and romiplostim most likely resolved the patient's thrombocytopenia. This case represents a novel use of efgartigimod in the treatment of passenger lymphocyte syndrome following liver transplant.
本病例描述了肝移植后供者淋巴细胞综合征（PLS）产生针对受者血小板的人类血小板抗原 1a（HPA-1a）同种异体抗体。鉴于 PLS 尤其在伴有 HPA-1a 同种异体抗体的肝移植中罕见，因此疾病过程和治疗选择描述甚少。
患者患有非酒精性脂肪性肝炎继发的肝硬化，合并肝细胞癌、肝性脑病和严重腹水。就诊时终末期肝病模型（MELD）评分为 15。肝移植后发生肝动脉血栓形成，再次因 MELD 评分为 40 而被列入移植名单。患者在第一次移植后第 7 天接受了丙型肝炎病毒抗体阳性、丙型肝炎病毒核酸扩增试验阳性供者的肝脏。第二次移植后第 7 天，患者出现严重的血小板减少症，对血小板输注无效。基于血清血小板同种异体抗体检测，发现患者存在针对 HPA-1a 的血清抗体。后来发现供者的 HPA-1a 基因检测为阴性。然而，患者的自身血小板 HPA-1a 阳性。患者被诊断为 PLS。
患者的治疗过程包括输注 57 单位血小板、紧急脾切除术、利妥昔单抗、血浆置换、静脉注射免疫球蛋白（IVIG）、艾曲泊帕、罗米司亭和 efgartigimod。
efgartigimod 与艾曲泊帕和罗米司亭的协同作用可能使患者的血小板减少症得到缓解。本病例代表了在肝移植后治疗供者淋巴细胞综合征中使用 efgartigimod 的一种新方法。
