Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare autosomal recessive primary immunodeficiency characterized by Epstein-Barr virus (EBV)-triggered lymphoprolipherative disorders such as malignant B-cell proliferation, Hodgkin lymphoma, B-cell lymphoma and EBV-driven hemophagocytic lymphohistiocytosis (HLH). Aplastic anemia and inflammatory disorders such as uveitis and oral ulcers are also observed. Disease data Classification Disease Synonyms Autosomal recessive lymphoproliferative disease due to CD27 deficiency CD27 deficiency Autosomal recessive lymphoproliferative disease due to CD27 deficiency CD27 deficiency ORPHA code 238505 OMIM code 615122 ICD10 code D47.9 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl