Hepatocellular Carcinoma in ADA-SCID Patient After Hematopoietic Stem Cell Transplantation
Küçük Resim Yok
Tarih
2023
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Lippincott Williams & Wilkins
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Adenosine deaminase (ADA) deficiency is one of the most prevalent forms of severe combined immunodeficiency and results in the accumulation of toxic substrates which creates a systemic metabolic disease. It predisposes patients to the development of malignancies, most commonly lymphoma. We report an 8-month-old infant with ADA deficient severe combined immunodeficiency who developed progressive liver dysfunction and hepatocellular carcinoma after successful hematopoietic stem cell transplantation. This is the first case report of an ADA-deficient patient who presented with hepatocellular carcinoma and gives an insight into the complex etiology that can lie behind liver dysfunction in these patients.
Açıklama
Anahtar Kelimeler
Adenosine Deaminase, Severe Combined Immunodeficiency, Hematopoietic Stem Cell Transplantation, Hepatocellular Carcinoma, Liver Transplantation
Kaynak
Journal of Pediatric Hematology Oncology
WoS Q Değeri
N/A
Scopus Q Değeri
Q3
Cilt
45
Sayı
5
