Department of Hematology and Hemotherapy, Federal University of Sao Paulo, São Paulo, Brazil.
Department of Hematology and Hemotherapy, Federal University of Sao Paulo, São Paulo, Brazil.
Transfus Apher Sci. 2021 Aug;60(4):103136. doi: 10.1016/j.transci.2021.103136. Epub 2021 Apr 8.
Autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), and autoimmune neutropenia (AIN) are reported in the literature after liver, intestinal, heart, pancreas, and kidney transplants. We report a case of autoimmune pancytopenia (AIHA, AIN and ITP) 9 years after liver transplantation with confirmed erythrocyte and neutrophil auto-antibodies.
A 49 years old man was admitted to our hospital presented with dysentery and fever, with history of liver transplantation in 2008. Laboratory evaluation demonstrated hemoglobin: 7.2 g/dL, granulocytes: 0.10 × 10/L and platelets: 15 × 10/mm³; indirect bilirubin: 3.62 mg/dL; lactate dehydrogenase: 603 U/L. Direct antiglobulin test revealed a monospecific anti-IgG plus C3 and the acid eluate was reactive to all panel red cells, consistent with an AIHA. Granulocyte immunofluorescence test (GIFT) and agglutination test (GAT) were reactive for granulocytes. Test with Luminex technology for human neutrophil antigen (HNA) antibody detection was strong reactive with beads expressing HNA-1a, -1b, -1c, -2, -4a and -5a antigens. HNA genotyping revealed the presence of the corresponding antigens, confirming the autoantibodies. Test with Luminex technology for human leucocyte antigen (HLA) antibody detection was negative. Monoclonal antibody immobilization of platelet antigens (MAIPA) assay was negative. Viral causes were excluded. The condition was compatible with clinical onset of autoimmune pancytopenia. Prednisone was administered at an initial dose of 1 mg/kg/day and immunosuppressive therapy was adjusted. This treatment resulted in rapid resolution of pancytopenia.
Combined autoimmune pancytopenia (AIHA, AIN and ITP) is a rare condition that may occur after liver transplantation. Early recognition of this phenomenon permits appropriate treatment.
在肝、肠、心脏、胰腺和肾脏移植后,文献中报道了自身免疫性溶血性贫血(AIHA)、免疫性血小板减少症(ITP)和自身免疫性中性粒细胞减少症(AIN)。我们报告了一例肝移植 9 年后发生的自身免疫性全血细胞减少症(AIHA、AIN 和 ITP)病例,伴有红细胞和中性粒细胞自身抗体的确诊。
一名 49 岁男性因痢疾和发热入院,该患者于 2008 年接受了肝移植。实验室评估显示血红蛋白:7.2g/dL,粒细胞:0.10×10/L 和血小板:15×10/mm³;间接胆红素:3.62mg/dL;乳酸脱氢酶:603 U/L。直接抗球蛋白试验显示单特异性抗 IgG 加 C3,酸洗脱液与所有面板红细胞反应,符合自身免疫性溶血性贫血。粒细胞免疫荧光试验(GIFT)和凝集试验(GAT)对粒细胞反应阳性。采用 Luminex 技术检测人类中性粒细胞抗原(HNA)抗体检测呈强反应性,与表达 HNA-1a、-1b、-1c、-2、-4a 和-5a 抗原的珠子反应。HNA 基因分型显示存在相应的抗原,证实了自身抗体的存在。采用 Luminex 技术检测人类白细胞抗原(HLA)抗体检测呈阴性。血小板抗原单克隆抗体固定(MAIPA)检测呈阴性。排除了病毒原因。病情符合自身免疫性全血细胞减少症的临床发作。初始剂量为 1mg/kg/天给予泼尼松,并调整免疫抑制治疗。这种治疗导致全血细胞减少迅速缓解。
联合自身免疫性全血细胞减少症(AIHA、AIN 和 ITP)是肝移植后罕见的疾病。早期识别这种现象可以进行适当的治疗。