Cutaneous Intravascular NK/T-cell lymphoma mimic panniculitis clinically, case report and literature brief review.

Ahmed Alhumidi

doi:10.1186/s13000-015-0330-0

Abstract

Intravascular large cell lymphoma is a rare subtype of extranodal large cell lymphoma characterized by the presence of neoplastic cells within the lumina of small vessels. Most cases of intravascular large cell lymphoma have a B-cell phenotype. To date, 12 cases of intravascular natural killer (NK/)/T–cell lymphoma (IVNKL) have been reported. Our case is A 47-year-old female presented with erythematous patches and plaques on the lower extremities mimicking panniculitis clinically. A skin biopsy revealed intravascular lymphoma (IVL) with a NK/T cell phenotype (positive for CD3, and granzyme B and negative for CD20, CD4, CD8, CD5). The lymphoma cells were also positive for Epstein-Barr virus by Epstein-Barr virus-encoded RNA in situ hybridization test. Because this type of lymphoma is extremely rare, our case is documented and compared with the previously reported cases.

Highlights

Intravascular lymphoma (IVL) is a rare form of non-Hodgkin lymphoma characterized by massive proliferation of large, neoplastic cells in small- and medium-sized blood vessels with a predilection for the skin and the central nervous system
IVL is a very rare neoplasm characterized by proliferation of lymphoma cells almost exclusively within the blood vessels
Described in 1959 as “angioendotheliomatosis proliferans systemisata” and considered an endothelial neoplasm, IVL has subsequently been shown to be a lymphoid malignancy based on immunohistochemical techniques [1,2,3]

Summary

Introduction

Background Intravascular lymphoma (IVL) is a rare form of non-Hodgkin lymphoma characterized by massive proliferation of large, neoplastic cells in small- and medium-sized blood vessels with a predilection for the skin and the central nervous system. Most cases of IVL are of B-cell immunophenotype; rare cases of NK/T-cell IVL have been reported. Because this variant is extremely rare, our case is documented and compared with the previously reported cases The histological study revealed many distended vessels filled with atypical large lymphoid cells in the subcutaneous tissue (Fig. 1).

Results

Conclusion