Intravascular large B-cell lymphoma

Orpha code: 98839OMIM code:

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

No additional description.

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