Intravascular large B-cell lymphoma

Definition

Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of diffuse large B-cell lymphoma 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.

Also known as angioendotheliomatosis proliferans systemisata, angiotropic large cell lymphoma, angiotropic lymphoma, intravascular B-cell lymphoma, intravascular large B-cell lymphoma, intravascular lymphomatosis, malignant angioendotheliomatosis, Tappeiner-Pfleger disease — per MONDO

Also identified as

• DOID 0081311 per MONDO
• ICD9 202.80 per MONDO
• NCIT C4342 per MONDO
• Orphanet 98839 per MONDO
• SCTID 255102004 per MONDO
• UMLS C0334660 per MONDO

Affected Organs