Multicentric dermatofibrosarcoma protuberans in a child with severe combined immunodeficiency due to adenosine deaminase deficiency

Autor:	Peter H. Hoeger, Heinz Kutzner, Matthias Bleeke, Tatjana D. Wahjudi
Rok vydání:	2021
Předmět:	Transplantation Severe combined immunodeficiency business.industry Pediatrics Perinatology and Child Health Dermatofibrosarcoma protuberans medicine Cancer research Lymphocyte activation Dermatology Stem cell medicine.disease business Adenosine deaminase deficiency
Zdroj:	Pediatric Dermatology. 38:875-878
ISSN:	1525-1470 0736-8046
Popis:	We report the case of a 4-year-old boy, post-human stem cell transplantation for severe combined immunodeficiency (SCID) due to adenosine deaminase deficiency (ADA), who developed multiple dermatofibrosarcoma protuberans (DFSP). We hypothesize a role for chimerism leading to accumulation of toxic metabolites which can cause DNA strand breaks and inhibit lymphocyte activation. Patients with ADA-SCID should remain under lifelong dermatologic surveillance as DFSP lesions can be quite inconspicuous.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_________::555447d76a077885c8d7251d073bdae6 https://doi.org/10.1111/pde.14597 Zobrazit plný text záznamu Plný text