Elsevier

Human Pathology

Volume 31, Issue 2, February 2000, Pages 220-226
Human Pathology

Original Contribution
Lack of CD 29 (β1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis*

https://doi.org/10.1016/S0046-8177(00)80223-3Get rights and content

Intravascular Lymphomatosis (IL) is a rare and usually aggressive form of non-Hodgkin's lymphoma characterized by the growth of neoplastic cells within vascular lumina that usually presents with skin or central nervous system (CNS) involvement. The mechanism(s) for the selective intravascular growth of this neoplasm remain(s) unexplained. We now report clinical and immunohistologic data on surgical material from 6 cases of IL; in 4 of 6 cases, autopsies were performed. Our IL cases shared the following features: (1) B-cell lineage; (2) lack of skin involvement at presentation; (3) aggressive behavior; and (4) lack of extravascular lymphomatous masses; in addition, 1 case had an associated gastric low-grade MALT lymphoma. We studied by immunohistochemistry formalin-fixed, paraffin-embedded sections with monoclonal antibodies to molecules known to be involved in lymphocyte and endothelial adhesion phenomena, that is, CD29 (β1 integrin subunit), CD43 (leukosialin), CD44 (H-CAM), CD54 (ICAM-1), embryonal N-CAM (e-NCAM), and EMA (episialin). In all cases, the surfaces of IL aggregates reacted for CD44 but were consistently negative for CD29; also absent was CD54. Conversely, the integrity of the endothelial cells was underscored by their even reactivity for CD29, CD44, and CD54. Given that CD29 is currently regarded as critical for lymphocyte trafficking in general and for transvascular migration in particular, and CD54 is also involved in transvascular lymphocyte migration, we conclude that their consistent absence in IL may contribute to its intravascular and disseminated distribution pattern. The rather frequent association of IL with various conventional lymphomas is known; yet, one of our cases appears to be the first report of IL associated with a low-grade MALT lymphoma.

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      Treatment with R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone) combined with an intrathecal infusion of methotrexate (MTX) is recommended for IVLBCL with central nervous system involvement. Although the reasons for intravascular localization of IVLBCL are unknown, it has been reported that the absence of intercellular adhesion molecule 1 (ICAM1) and β1 integrin (CD29) surface ligands may disable lymphoma cells from diapedesis across the endothelium.2,3 IVLBCL with pituitary involvement is rare.

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    *

    Presented in part at the XXII Congress of the International Academy of Pathology and 13th World Congress of Academic and Environmental Pathology, Nice (France), October 18–23, 1998.

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