Molecular Classification of Tumor Cells in a Patient with Intravascular Large B-Cell Lymphoma

Abstract

Background

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma. It is characterized by the proliferation of tumour cells exclusively intraluminally in small blood vessels of different organs. The clinical manifestation depends on the type of organ affected, additionally, a haemophagocytic syndrome can be observed in some patients.

Objective

Due to the rarity of this lymphoma and in spite of detailed immunohistochemical investigations the exact nosology of this cancer is only incompletely understood.

Methods

We used microarray-based analysis of gene expression of tumour cells isolated from a patient with primary manifestation of the lymphoma in the skin and compared it to various other diffuse large B-cell lymphomas (DLBCLs) as well as a previously published DLBCL classifier.

Results

In unsupervised analyses, the tumour cells cluster together with non-GCB DLBCL samples but are clearly distinct from GCB-DLBCL. Analogous to non-GCB DLBCL, molecular cell-of-origin classification revealed similarity to bone-marrow derived plasma cells.

Conclusion

The IVLBCL of this patient shows molecular similarity to non-GCB DLBCL. Due to the prognostic, and increasingly also therapeutic relevance of molecular subtyping in DLBCL, this method, in addition to immunohistochemistry, should also be considered for the diagnosis of IVLBCL in the future.

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