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.
Authors
- Ian A. Makey (ORCID: https://orcid.org/0000-0002-8380-0331)
- Jorge Sinclair (ORCID: https://orcid.org/0000-0001-9716-4252)
- Tathagat Narula (ORCID: https://orcid.org/0000-0003-4631-9888)
- Lorenzo J. Olivero (ORCID: https://orcid.org/0000-0003-2358-9527)
- Si M. Pham (ORCID: https://orcid.org/0000-0001-7396-8344)
- Wesley L. Allen (ORCID: https://orcid.org/0000-0003-1562-1208)
- Stephen Aniskevich (ORCID: https://orcid.org/0000-0001-9571-234X)
- Pablo Moreno Franco (ORCID: https://orcid.org/0000-0001-9146-5599)
- Candido E. Rivera
- Nathan H. Waldron (ORCID: https://orcid.org/0000-0002-1430-8204)
- Pramod Kumar Guru (ORCID: https://orcid.org/0000-0001-7695-4407)
- Hong Liang (ORCID: https://orcid.org/0000-0002-5631-4266)
- Sadia Shah (ORCID: https://orcid.org/0000-0002-5934-0013)
- Liu Yang (ORCID: https://orcid.org/0000-0003-1613-8146)
- Dana K. Perry (ORCID: https://orcid.org/0009-0007-8791-7522)
Institutions
- Mount Sinai Hospital (US)
- Jacobi Medical Center (US)
- WinnMed (US)
- Mayo Clinic in Florida (US)
- Icahn School of Medicine at Mount Sinai (US)
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
- Field-Weighted Citation Impact
- 0.00