Living-Donor Lung Transplantation After Bone Marrow Transplantation for Chediak-Higashi Syndrome.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 28219570.
- Also identified by DOI 10.1016/j.athoracsur.2016.08.049.
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Abstract
An 8-year-old girl with Chediak-Higashi syndrome (CHS) had pulmonary complications after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) and eventually underwent single living-donor lobar lung transplantation (LDLLT). Electron micrographic findings showed vagus nerve tissue in extracted lung having granular inclusions, which are pathognomonic for CHS. Because her mother was the donor for both hematopoietic stem cell and lung transplantations, she was weaned from immunosuppression and is doing well 3 years after lung transplantation. Furthermore, an induced pluripotent stem (iPS) cell line was established from her skin fibroblasts for investigation and potential future treatment for CHS.
Medical subject headings
- Chediak-Higashi Syndrome
- Hematopoietic Stem Cell Transplantation
- Living Donors
- Lung Transplantation