Intravascular Large Cell Lymphoma
Definition
- A rare subtype of diffuse large cell lymphoma characterized by the presence of lymphoma cells in the lumina of small vessels
Alternate/Historical Names
- Angioendotheliomatosis proliferans systemisata
- Angio-endotheliotropic lymphoma (Kiel)
- Diffuse large B cell lymphoma (REAL)
- Intravascular lymphomatosis
- Malignant angioendotheliomatosis
- Intravascular histiocytosis (variant)
Diagnostic Criteria
- Predominantly Intravascular Involvement
- Brain and skin most common sites of involvement
- Capillaries to medium sized vessels
- Occasional features
- Endothelial proliferation
- Thrombosis with recanalization
- Regional infarction
- Minor extravascular involvement
- Infiltrate may be free floating or subendothelial
- Atypical cells may appear to be adherent to wall
- Cells may be trapped in clots
- Large Noncohesive Cells
- Cytologically malignant nuclei
- Scant to moderate cytoplasm
- Erythrophagocytosis in Asian Variant and Histiocytic Variant
- Predominantly B Lineage
- Originally thought to be a neoplasm of endothelial cells, now clearly shown to be a lymphoma
- Leukocyte common antigen (LCA) positive
- Most cases B lineage, occasionally T
- Rare reports of true histiocytic lineage cases
- Occasionally Associated with Conventional Lymphomas
Yasodha Natkunam MD PhD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting:: May 6, 2006
