Intralymphatic Cutaneous Anaplastic Large Cell Lymphoma/Lymphomatoid Papulosis

Abstract

Intravascular large B-cell lymphomas and EBV NK/T-cell lymphomas commonly follow an aggressive clinical course. We recently reported an entirely intravascular anaplastic large cell lymphoma (ALCL) in the skin with a surprisingly indolent clinical course; interestingly, this lymphoma involved the lymphatic rather than the blood vasculature. We hypothesized that intravascular skin-limited ALCL is distinct from aggressive systemic intravascular lymphomas in its intralymphatic localization and clinical course. We now describe 18 cases of cutaneous intravascular large cell lymphoproliferations from 4 institutions. All 12 intravascular large T-cell lesions were intralymphatic; the majority (9) were CD30 T-cell lymphoproliferative disorders (TLPDs), 5 further classified as intravascular ALK ALCL. One ALK ALCL and 2 benign microscopic intravascular T-cell proliferations were also intralymphatic. A single case of otherwise typical cutaneous follicle center lymphoma contained intralymphatic centroblasts. The clinical and pathologic characteristics of the CD30 TLPDs were similar to those of their extravascular counterparts, including extralymphatic dermal involvement in a subset, DUSP22-IRF4 translocations in half of tested ALK ALCLs, and associated mycosis fungoides in 1; most were skin-limited at baseline and remained so at relapse. All 5 cases of intravascular large B-cell lymphoma involved the blood vasculature and behaved in a clinically aggressive manner; the ALK ALCL, although intralymphatic, was systemic and clinically aggressive. We propose that cutaneous ALK ALCL and related CD30 ALK TLPDs involving the lymphatics are part of an expanding spectrum of CD30 TLPDs. The identification of intralymphatic as distinct from blood vascular localization may provide critical prognostic and therapeutic information.

Asymptomatic Cutaneous Plaques on the Scalp and Face in an Older Adult Woman

“…5,6 The DUSP22 gene is not specific for primary cutaneous anaplastic large cell lymphoma, but some cases of primary cutaneous anaplastic large cell lymphoma with DUSP22 translocation display prominent intravascular involvement. 7,8 Of note, it has been postulated that most of the so-called IALCLs have intralymphatic origin and demonstrate a tropism to lymphatic channels, like the case reported here that was both D2-40 positive and CD31 positive. 7 Because lymphovascular channels were involved with this patient, it was challenging to distinguish IALCL fromsystemicanaplasticlargecelllymphoma.Nevertheless,thelesions in the patient presented here were located exclusively on the skin during a 2-year period and lacked ALK and EMA expression, which supported categorization of this case at one end of the spectrum of the primary cutaneous disease process with propensity to lymphovascular involvement.…”

“…7 Because lymphovascular channels were involved with this patient, it was challenging to distinguish IALCL fromsystemicanaplasticlargecelllymphoma.Nevertheless,thelesions in the patient presented here were located exclusively on the skin during a 2-year period and lacked ALK and EMA expression, which supported categorization of this case at one end of the spectrum of the primary cutaneous disease process with propensity to lymphovascular involvement. 8 The main differential diagnoses of IALCL are large B-cell lymphoma and NKTCL. Large B-cell lymphoma expresses B-cellassociated antigens (CD20, CD79a, Bcl-2, MUM-1), and NKTCL is positive for CD3ε, CD56, EBER, and cytotoxic markers.…”

A case of exuberant cutaneous lymphomagenesis in the setting of chronic patch mycosis fungoides

“…With the exception of this index case, previous descriptions of intravascular cutaneous ALCL do not show a consistent association with pre-existing MF and more prototypically develop de novo as solitary or progressive papulonodular eruptions. 5 Attempts in this case to clonally link the prior MF with the subsequent intravascular cutaneous ALCL were not successful, as there was inadequate amplification of both T-cell receptor target DNA and an internal control despite the use of laser microdissection. The clinical presentation and histopathologic features were also not consistent with large cell transformation of MF.…”

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.…”

“…lymphomatoid papulosis. [11][12][13] 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. All these considerations make the case we describe unique, the first one to describe an entity with appears to be very rare or unrecognized.…”