Cutaneous Intravascular Hematolymphoid Entities: A Review

Abstract

Intravascular lymphomas are rare disease conditions that exhibit neoplastic lymphoid cells that are confined mainly to the lumens of small capillaries and medium-sized vessels. The majority of the intravascular lymphomas are of B-cell origin, but they can include NK/T-cell and CD30+ immunophenotypes. In the histologic differential diagnosis are benign proliferations such as intralymphatic histiocytosis and intravascular atypical CD30+ T-cell proliferation. In this review, we discuss the clinical, histopathologic, and molecular findings of intravascular B-cell lymphoma, intravascular NK/T-cell lymphoma, intralymphatic histiocytosis, and benign atypical intravascular CD30+ T-cell proliferation.

Keywords: B-cell; CD30; T/NK-cell; histiocytosis; intralymphatic; intravascular; lymphoma.

Figure 1

Intravascular B-cell lymphoma involving the skin. Atypical lymphoid cells are seen within the vascular lumens of a cutaneous hemangioma ((A), ×40). At higher magnification, the tumor cells are large and hyperchromatic, and they fill the vascular spaces ((B), ×200). The tumor cells express CD20, a B-cell marker ((C), ×200), while they are negative for CD3, a T-cell marker ((D), ×200).

Figure 2

Intravascular lymphoma involving the brain. The vasculature of the brain parenchyma is dilated by atypical lymphoid cells ((A), ×200). At higher magnification, the tumor cells are large and hyperchromatic ((B), ×400). The tumor cells express CD20, a B-cell marker, ((C), ×400) and they exhibit a high Ki-67 proliferation index ((D), ×400).

Figure 3

Intravascular histiocytosis. Skin biopsy shows the dermal vascular spaces expanded by lymphoid cells (arrows) ((A), ×40). The infiltrate is comprised mainly of histiocytes ((B), ×400) that express CD68, a macrophage marker, ((C), ×400) and lymphocytes. D2-40, a lymphatic marker, highlights the dermal vascular channels ((D), ×400).