Intravascular Large Cell Lymphoma
Intravascular Histiocytosis Variant
- Very rare cases are described with a true histiocytic phenotype
- Clinical
- One case restricted to skin, remained localized but recurred following electron beam therapy
- One case generalized including skin, responded to chemotherapy
- Histology
- Intravascular with few extravascular atypical cells
- Granular foamy cytoplasm with occasional phagocytosis of erythrocytes and neutrophils
- Nuclei oval, no grooves, minimal pleomorphism
- Immunohistology
- Histiocytic markers positive
- CD68, Mac387 in paraffin sections
- CD11b, CD14, HLADR in frozen tissue
- Other markers negative
- CD20, MB1, kappa, lambda, heavy chains
- CD3, CD45RO, CD43
- CD15, CD30, S100, CD34, elastase, FactorVIIIRA
- Genotypic studies negative for B and T receptor rearrangement
- Lacks high grade nuclear pleomorphism of true histiocytic lymphoma
