Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of diffuse large B-cell lymphoma (see this term) characterized by the selective growth of lymphoma cells within the lumina of small blood vessels (especially the capillaries) that most often presents with a wide range of clinical manifestations (as potentially any tissue can be involved), with patients from Western countries more frequently manifesting with neurological and cutaneous symptoms while patients from Asian countries more frequently displaying hepatosplenomegaly and thrombocytopenia. IVLBCL is characterized by an absence of lymphadenopathy, an aggressive clinical course and a poor prognosis. Disease data Classification Disease Synonyms Angioendotheliomatosis proliferans systemisata Angiotropowy chłoniak wielkokomórkowy Choroba Tappeinera i Pflegera Limfomatoza wewnątrznaczyniowa Złośliwa angioendoteliomatoza Angiotropic large cell lymphoma Intravascular lymphomatosis Malignant angioendotheliomatosis Tappeiner-Pfleger disease ORPHA code 98839 OMIM code - ICD10 code C83.3 ICD11 code 2A81.1 *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