Combined lung and liver transplant for cirrhosis, idiopathic pulmonary fibrosis, and hemophilia A: Case report

• First documented case of combined liver and lung transplant (CLLT) in a hemophilia A patient. • CLLT may be feasible while dealing with coagulopathy from hemophilia and liver disease. • An individualized, case-by-case approach is crucial in such a complex scenario. • FVIII target levels were 60 % on ECMO and 100–120 % during and after transplantation. • Hemophilia a phenotype was cured after the liver transplantation. Combined lung and liver transplantation (CLLT) is a rare intervention for end-stage lung and liver diseases. It poses a challenge for patients with increased bleeding risk due to Hemophilia A and liver coagulopathy. We present the first documented case of CLLT in an elderly male with Hemophilia A, HCV-associated cirrhosis, and Idiopathic Pulmonary Fibrosis (IPF). Despite the patient exhibiting stable liver function and hemophilia, his lung condition rapidly deteriorated, prompting the listing for transplant. The patient underwent a successful CLLT with perioperative management coordinated by the multidisciplinary team to address the unique challenges of Hemophilia A, resulting in intra-operative correction of coagulopathy. The patient exhibited a favorable recovery, with no requirement for Factor FVIII replacement therapy postoperatively. This case demonstrates that CLLT can address three diseases with distinct pathophysiologies: end-stage lung and liver disease, and correct the hemophilia A phenotype. Our report contributes to the limited literature on the suitability of CLLT in patients with hemophilia A.

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Publication Details

Journal
Transplantation Reports
Published
2024-11-19
DOI
https://doi.org/10.1016/j.tpr.2024.100163
Primary Topic
Hemophilia Treatment and Research
Type
article
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article

Combined lung and liver transplant for cirrhosis, idiopathic pulmonary fibrosis, and hemophilia A: Case report

Ian A. Makey, Jorge Sinclair, Tathagat Narula, Lorenzo J. Olivero et al.
Transplantation Reports
Hemophilia Treatment and Research
article

Combined lung and liver transplant for cirrhosis, idiopathic pulmonary fibrosis, and hemophilia A: Case report

Ian A. Makey, Jorge Sinclair, Tathagat Narula, Lorenzo J. Olivero, Si M. Pham, Wesley L. Allen, Stephen Aniskevich, Pablo Moreno Franco, Candido E. Rivera, Nathan H. Waldron, Pramod Kumar Guru, Hong Liang, Sadia Shah, Liu Yang, Dana K. Perry
article en

Abstract

• First documented case of combined liver and lung transplant (CLLT) in a hemophilia A patient. • CLLT may be feasible while dealing with coagulopathy from hemophilia and liver disease. • An individualized, case-by-case approach is crucial in such a complex scenario. • FVIII target levels were 60 % on ECMO and 100–120 % during and after transplantation. • Hemophilia a phenotype was cured after the liver transplantation. Combined lung and liver transplantation (CLLT) is a rare intervention for end-stage lung and liver diseases. It poses a challenge for patients with increased bleeding risk due to Hemophilia A and liver coagulopathy. We present the first documented case of CLLT in an elderly male with Hemophilia A, HCV-associated cirrhosis, and Idiopathic Pulmonary Fibrosis (IPF). Despite the patient exhibiting stable liver function and hemophilia, his lung condition rapidly deteriorated, prompting the listing for transplant. The patient underwent a successful CLLT with perioperative management coordinated by the multidisciplinary team to address the unique challenges of Hemophilia A, resulting in intra-operative correction of coagulopathy. The patient exhibited a favorable recovery, with no requirement for Factor FVIII replacement therapy postoperatively. This case demonstrates that CLLT can address three diseases with distinct pathophysiologies: end-stage lung and liver disease, and correct the hemophilia A phenotype. Our report contributes to the limited literature on the suitability of CLLT in patients with hemophilia A.

Transplantation ReportsVol. 9(4)
Mount Sinai Hospital (US), Jacobi Medical Center (US), WinnMed (US), Mayo Clinic in Florida (US), Icahn School of Medicine at Mount Sinai (US)
Good health and well-being
Openalex Percentile: Top 66%
Hemophilia Treatment and Research
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