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

Abstract

Intralymphatic proliferation of T-cell lymphoid blasts (IPTCLBs) is a rare, recently described entity, associated with cutaneous inflammatory conditions and characterized by intralymphatic proliferation of highly proliferating, blastoid T lymphocytes expressing CD30, thus mimicking an intravascular lymphoma. In all reported cases, the intralymphatic proliferation was associated with an underlying inflammatory condition, with no clonal T-cell receptor rearrangement, no signs of systemic or cutaneous lymphoma, and excellent prognosis. The authors present a new case of IPTCLB arising in a patient with hidradenitis suppurativa. Histological examination revealed a dilated follicle embedded within a fibrotic stroma surrounded by a dense lymphoid infiltrate characterized by the presence of dilated small vessels filled with atypical medium-to-large sized blastoid lymphocytes expressing a CD4 T phenotype. There was also expression of CD30, but negativity for cytotoxic markers and Epstein-Barr virus. The proliferation index was high and the vessels showed expression of D2-40, confirming their lymphatic nature. No signs of systemic lymphoma could be detected after routine investigations, and the patient is alive and in good general health. IPTCLB is a rare benign entity that presents with worrying, potentially misleading histopathological features that mimic those observed in intravascular lymphoma. Careful histological and phenotypic investigations and correlation with the clinical features are necessary for a proper diagnosis.

Benign atypical intralymphatic CD30+ lymphoid proliferation with activated regulatory T‐cell phenotype in the oral cavity

“…Excluding the current report, to date, 12 cases of benign atypical intralymphatic T‐cell proliferation, mostly CD30+, all but one affecting the skin and often associated with reactive/benign lesion, have been reported in the English literature . Interestingly, 7 (58%) of them showed oligoclonal or polyclonal T‐cell populations by PCR analysis . These cases affected six males and six females, with a range of 17 to 77 years (mean age, 44 years).…”

“…To date, only 12 cases of atypical intralymphatic T‐cell proliferations, mostly CD30+, associated with cutaneous reactive/benign lesion, and following a benign clinical course, have been previously reported in the English literature . To the best of our knowledge, we report the first oral case of atypical intralymphatic CD30+ T‐cell lymphoid proliferation, initially suspicious for IVL, which showed spontaneous resolution after biopsy.…”

“…We report here the first oral case of benign atypical intralymphatic CD30+ T‐cell proliferation, also known as intralymphatic proliferation of T‐cell lymphoid blasts, which describes a subset of reactive atypical intralymphatic T‐cell proliferations, following a benign course, and it should be careful differentiated from IVL, an aggressive disease with poor prognosis …”

“…These cases affected six males and six females, with a range of 17 to 77 years (mean age, 44 years). The associated skin lesions were molluscum contagiosum, hemangioma, pyogenic granuloma, lichen sclerosus, hidradenitis suppurativa and prurigo nodule, as well as endometrial polyp and trauma . Recently, a case associated with drug rash with eosinophilia and systemic symptoms has been reported .…”

Cutaneous intravascular CD30+ T‐cell pseudolymphoma occurring in a regressing keratoacanthoma

“…The incidental finding of intravascular or intralymphatic large atypical lymphocytes on skin biopsies should raise the suspicion of intravascular lymphoproliferative disorders including intravascular T‐cell lymphoma, intravascular CD30+ anaplastic large cell lymphoma and the recently described benign atypical CD30+ T‐cell intravascular proliferation, termed “CD30+ T‐cell pseudolymphoma.” There are only a few cases reported in the literature associated with chronic cutaneous inflammatory conditions, lymphatic disruption and trauma. In all reported cases, clonal T‐cell receptor rearrangement was absent, no sign of systemic or cutaneous lymphoma was present, and none progressed to more serious disease . We describe a patient with benign cutaneous intravascular CD30+ T‐cell pseudolymphoma associated with a regressing keratoacanthoma and review the morphological, immunophenotypic and molecular features of intravascular/intralymphatic atypical lymphoid proliferations.…”