Successful treatment of severe passenger lymphocyte syndrome with efgartigimod synergy.
Transfusion
; 64(4): 755-760, 2024 Apr.
Article
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| MEDLINE
| ID: mdl-38425280
ABSTRACT
INTRODUCTION:
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.METHODS:
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.RESULTS:
The patient's treatment course included 57 units of platelets transfused, emergency splenectomy, rituximab, plasma exchange, intravenous immunoglobulin (IVIG), eltrombopag, romiplostim, and efgartigimod.DISCUSSION:
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.Palabras clave
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Banco de datos:
MEDLINE
Asunto principal:
Pirazoles
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Trombocitopenia
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Benzoatos
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Trasplante de Hígado
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Antígenos de Plaqueta Humana
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Enfermedad Hepática en Estado Terminal
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Hidrazinas
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Anemia
Límite:
Humans
Idioma:
En
Año:
2024
Tipo del documento:
Article