Haemophagocytic syndrome in a liver transplant patient during treatment with Telaprevir.

Autores de IIS La Fe

• Beatriz Rodriguez Medina

  Autor

• Marino Blanes Juliá

  Autor

• Carmen Vinaixa Aunés

  Autor

• Victoria Aguilera Sancho-Tello

  Autor

• Ángel Rubín Suárez

  Autor

• Martín Prieto Castillo

  Autor

• 

  Marina Berenguer Haym

  Autor

Grupos

• Hepatología experimental y Trasplante hepático

• Hepatología, Cirugia Hepatobiliopancreática y Trasplantes

Abstract

Haemophagocytic syndrome (HS) is a rare disease that is often fatal despite treatment. HS is characterized by fevers, lymphadenopathy, hepatosplenomegaly, cytopenias and hyperferritinaemia due to deregulated activation and proliferation of macrophages, leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes, and their hematopoietic precursors throughout the reticuloendothelial system. Mycobacterium tuberculosis-associated HS is a rare and underdiagnosed association with only 39 cases reported. We describe a case of HS associated with disseminated Mycobacterium tuberculosis in the setting of post-liver transplantation anti-hepatitis C therapy with pegylated interferon (pegIFN), ribavirin (RBV) and telaprevir (TVR). Despite the delay in the etiologic diagnosis, the patient was treated properly with corticosteroids, cyclosporine and tuberculostatic agents. It is unknown whether telaprevir, a drug that only recently has been started off-label in liver transplant recipients, may have contributed to the development of the HS. Unfortunately, as in many reported cases of HS, the outcome was unfavourable resulting in the death of the patient.