Benign Atypical Intravascular CD30+ T-Cell Proliferation: A Recently Described Reactive Lymphoproliferative Process and Simulator of Intravascular Lymphoma

Abstract

Objectives: Intravascular accumulations of atypical large lymphoid cells are a rare finding in skin biopsy specimens and raise the suspicion for intravascular lymphoma. The intravascular accumulation of atypical large CD30+ T cells, however, as a reactive process is very uncommon in the skin, with only four cases documented so far in the literature. This condition, referred to as benign intravascular atypical CD30+ T-cell proliferation, has been associated with chronic inflammation after trauma. Methods: We report on a case of atypical intravascular CD30+ T-cell proliferation in a patient with ulcerated lichen sclerosus on the foreskin, discuss the differential diagnoses, propose diagnostic criteria, and review the literature on this uncommon reactive intralymphatic CD30+ T-cell lymphoproliferation. Results: The atypical intravascular CD30+ T-cell proliferation is characterized by the accumulation of large CD30+ polyclonal T cells within lymphatics in close vicinity to ulceration or an inflammatory skin disease. There is no association with Epstein-Barr virus infection. Conclusions: This benign cutaneous lymphoproliferation needs to be distinguished from intravascular T-cell lymphoma, particularly from the intravascular variant of anaplastic large cell lymphoma. Obstruction of lymphatics due to lichen sclerosus with disrupted immune cell trafficking may result in the accumulation of activated CD30+ lymphocytes.

Intravascular T‐cell lymphoma: A rare, poorly characterized entity with cytotoxic phenotype

“…All cases of ALCL have in common the large, anaplastic morphology of the neoplastic cells and the positive stains for CD30. Recently, an indolent CD30 positive entity has been described in the lymphatics in the skin, probably an entity sharing many characteristics with lymphomatoid papulosis . Cases of intravascular T‐cell lymphoma, not belonging to the described categories, are very rare, even case reports are lacking, and there are no reliable morphologic or immunophenotypic studies characterizing these entities.…”

“…Unlike intravascular B‐cell lymphoma, a well‐defined entity, intravascular T‐cell lymphoma is not included in the current classification of lymphomas and its revision, probably due to the very small number of cases thoroughly characterized and reported. Moreover, most cases reported as intravascular T‐cell or natural killer (NK)‐cell lymphomas actually are leukemic presentation or variants of other types of lymphoma, mainly NK/T‐cell lymphoma, anaplastic large cell lymphoma (both anaplastic large cell kinase positive and negative types), or a recently described CD30 positive intravascular T‐lymphoproliferative disorder involving the skin . An additional level of complexity is added by the wide geographical distribution of the cases and by the lack of a homogenous work‐up.…”

Intralymphatic Proliferation of T-cell Lymphoid Blasts in the Setting of Hidradenitis Suppurativa

“…Although the histopathologic features may arise the suspicion of intravascular large cell lymphoma, all reported cases occurred in the background of an inflammatory disorder, without clinical signs of lymphoma and with a favorable outcome. [2][3][4][5][6][7] So far, only 7 cases of IPTCLB have been reported, 6 of them with cutaneous localization, and 1 arising within an endometrial polyp (Table 1). [2][3][4][5][6][7] Although the first description is considered to be that by Bryant et al in 2007, 4 Ackerman and Tanski 8 described a similar case in 1977 under the term "pseudoleukemia cutis."…”

“…1 To date, only few cases of IPTCLB have been reported in the literature, associated with different inflammatory conditions. [2][3][4][5][6][7] We present a new additional case occurring in a young male patient in the setting of hidradenitis suppurativa.…”

Intravascular (blood vessel) histiocytosis with haemophagocytosis

“…In most cases the cause is intralymphatic histiocytosis (ILH), that may be either primary (idiopathic) or secondary to neoplastic diseases or inflammatory conditions, and is particularly frequent in relation to rheumatoid arthritis . In addition, different types of CD30 + lymphoproliferative disorders may also show lymphotrophism, including anaplastic large cell lymphomas, lymphomatoid papulosis and the incidental finding of benign clusters of T lymphoid blasts in the setting of inflammation . It is noteworthy, that the involvement of blood vessel lumina has been described only in relation to malignant lymphomas, usually of the large B cell type .…”