Intravascular large T-cell lymphoma: a case report of CD30-positive and ALK-negative anaplastic type with cytotoxic molecule expression

Abstract

We reported a case of intravascular T-cell lymphoma (T-IVL) with anaplastic large cell morphology, the hemophagocytic syndrome, and an aggressive clinical course. Phenotypic analysis of the tumor cells revealed CD2+, CD3-, CD4+, CD5-, CD8-, CD30+, CD56-, T-cell receptor alpha/beta-, ALK-, TIA1+, granzyme B+, and perforin+. No association with Epstein-Barr virus was found by in situ hybridization. A review revealed that 25 cases of T-IVL have been reported in the available literature, only two of which were of CD30+ anaplastic large cell or cytotoxic T-cell type. The findings in the present case may highlight the unique clinicopathologic aspects of a subset of CD30-positive T-IVLs with an ALK-negative cytotoxic phenotype.

Intravascular cytotoxic T-cell lymphoma: A case report and review of the literature

“…14,15 EBV is generally absent in IVL of B-cell origin, having been reported in only 3 of 39 cases, two of which occurred in immunosuppressed patients. 10,[16][17][18][19][20] In contrast, EBV was detected in 3 of 6 T-cell IVL and 2 of 3 NK-cell IVL analyzed to date 3,7,[12][13][14]20 ; only one of these 5 patients was immunosuppressed. The relatively high prevalence of EBV in NK-and T-cell IVL is intriguing, but further studies are needed to establish an etiologic role for the virus.…”

“…The first case, which arose in an HIV-positive patient, had a cytotoxic/suppressor T-cell phenotype (CD4 À /CD8 1 , CD56 À , and TIA-1 1 ) and was positive for EBV; immunohistochemical analysis for TCRbF1 and gTCR1 was not performed. 14 The second case was CD4 1 /CD8 À , CD30 1 , CD56 À , TCRbF1 À , and negative for EBV 12,14 ; given the CD4 1 /CD8 À phenotype, this case is more likely of ab T-cell origin rather than gd T-cell origin despite the lack of TCRbF1 expression. Both patients had visceral involvement and an aggressive clinical course, leading to death at 2 months and 1 week, respectively.…”

“…[11][12][13] Given the overlapping phenotypic profile for NK cells and cytotoxic T cells, some of these cases may actually represent NK cell rather than T cell neoplasms. Definitive characterization would require molecular studies.…”

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

“…Prognosis of ilALCL is good, but rare cases of genuine CD30 + NK/T-cell IVL with poor prognosis have been described. 12,13 Rare cases of intralymphatic lymphomatoid papulosis (ilLyP) may represent another potential pitfall, but the clinical setting of waxing and waning lesions that regress without treatment is different from that of the inflammatory conditions associated with IPTCLB. Unlike IPTCLB, analysis of the rearrangement of TCR genes shows a monoclonal pattern in most cases of LyP.…”

Diagnostic Difficulties of Pure Intrasinusoidal Bone Marrow Infiltration of Non-Hodgkin's Lymphoma: A Report of Eight Cases from India

“…Immunohistochemistry is essential to detect intrasinusoidal medullary infiltrates and should be performed in patients with splenomegaly and peripheral lymphocytosis (2). Uncommon entities that need to be considered in the differential diagnosis of T-cell lymphomas are 'intravascular large T-cell lymphoma' and nasal-type NK/T-cell lymphoma (15). These two entities present with different clinical findings.…”