Intravascular NK/T-cell lymphoma: a case report and literature review

Abstract

Intravascular lymphoma is characterized by an exclusively intravascular distribution of tumor cells. Intravascular natural killer/T-cell lymphoma (IVNKTL) is extremely rare, highly aggressive, commonly Epstein-Barr virus (EBV)–positive, and predominantly affects the skin and central nervous system. Here we report a case of IVNKTL diagnosed in a 67-year-old female, presenting with persistent intermittent fever and skin rashes throughout the body. Incisional biopsy of an erythematous lesion on the chest exhibited aggregation of medium to large-sized atypical lymphoid cells confined to the lumen of small vessels that were positive for CD3, granzyme B, and CD56 on immunohistochemistry and EBV-encoded RNA in situ hybridization. EBV DNA was also detected in serum after diagnosis. With a review of 26 cases of IVNKTL to date, we suggest that active biopsy based on EBV DNA detection may facilitate early diagnosis of IVNKTL.

Cutaneous Intravascular Hematolymphoid Entities: A Review

“…Given its rarity, optimal disease classification remains unclear; IVNKTL was formerly considered to be a variant of extranodal NK/T-cell lymphoma [43,44]; however, the most recent edition of the WHO classification of lymphoid neoplasms categorizes IVNKTL as a subtype of aggressive NKcell leukemia instead [45]. IVNKTL has predominantly been reported in adult patients without a clear age or sex predilection [40,42].…”

“…The clinical features of IVNKTL are highly variable, contributing to challenges in making the diagnosis. The vast majority of patients present with skin findings, though the rash characteristics follow no consistent pattern [42]; erythema [46] and erythematous plaques or nodules [47][48][49], sometimes with overlying telangiectasias [50], are frequently described in the literature. In addition to the cutaneous manifestations, other organs and organ systems including the CNS, bone marrow, lungs, and kidneys may be affected, with CNS manifestations being the most common [46,47].…”

“…Due to the potential for many different organ systems to be involved, presentation is highly variable; symptoms may include fever and other B-symptoms, malaise, jaundice, neurologic symptoms, arthralgias and other musculoskeletal symptoms, or rash [40,46,47,51]. To further complicate matters, some patients present only with cutaneous manifestations with no symptomatic evidence of systemic disease [40,42,48,52]. Hematologic findings including anemia, thrombocytopenia, and leukopenia are sometimes observed upon workup due to involvement of the bone marrow [33,48].…”

“…Skin biopsy is the most effective means of diagnosis [42]. In cases without evidence of skin involvement, patients may alternatively be diagnosed via bone marrow [33] or brain [42] biopsy.…”