Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A severe form of alpha-thalassemia that is mostly lethal, and associated with severe long-term outcome and lifelong transfusions in survivors. It is characterized by fetal onset of generalized edema, pleural and pericardial effusions, and severe hypochromic anemia. Disease data Classification Clinical subtype Synonyms Alpha-thalassemia hydrops fetalis Alfa-talasemia ciężka Alfa-talasemia homozygotyczna Alfa-talasemia obrzęk płodu Hemoglobina Barta obrzęk płodu Alpha-thalassemia major Hemoglobin Bart's hydrops fetalis Homozygous alpha0-thalassemia ORPHA code 163596 OMIM code 236750 ICD10 code D56.0 ICD11 code 3A50.03 *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