Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype

Alegría-Landa, Victoria; Manzarbeitia, Félix; Calderón, Maria G Salvatierra; Requena, Luis; Rodríguez‐Pinilla, Socorro María

doi:10.1111/his.13332

Cited by 19 publications

(24 citation statements)

References 37 publications

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“…The present study thoroughly investigated the transcriptomic landscape of two rare IVNKTCL cases. Twenty cases of IVNKTCL have been reported to date 6–10. Our cases comprise the 21st and 22nd, and this is the first study focusing on transcriptomic modifications in IVNKTCL patients.…”

Section: Discussionmentioning

confidence: 97%

“…Intravascular lymphoma (IVL) is a rare form of non-Hodgkin’s lymphoma characterised by almost-exclusive growth of neoplastic cells within the lumina of small-sized or medium-sized blood vessels 1. Most cases of IVL have a B-cell phenotype (intravascular large B-cell lymphoma, IVLBCL),2–5 but extremely rare cases with an NK/T-cell phenotype (i.e., intravascular NK/T-cell lymphoma, IVNKTCL) have also been identified 6–11. IVNKTCL has been first described in 2003, and only 20 cases have been reported in the literature.…”

Section: Introductionmentioning

confidence: 99%

“…IVNKTCL is aggressive and has a poor overall prognosis, especially in patients with multisystem involvement (e.g., skin, liver and central nervous system). The pathobiology of IVNKTCL has been linked to infection with the Epstein–Barr virus (EBV), which is considered to be a cause of tumourigenesis and responsible for the aggressive features of this lymphoma 6–11…”

Section: Introductionmentioning

confidence: 99%

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Transcriptome complexity in intravascular NK/T-cell lymphoma

Fujikura¹,

Yoshida²,

Uesaka

2020

J Clin Pathol

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AimsIntravascular NK/T-cell lymphoma (IVNKTCL) is a rare disease, which is characterised by exclusive growth of large cells within the lumen of small vessels, Epstein–Barr virus infection and somatic mutations in epigenetic regulator genes. Here, we elucidate the transcriptomic complexity of IVNKTCL.MethodsIVNKTCL cases were retrieved from a single-centre cohort of 25 intravascular lymphomas. RNA-seq and whole exome sequencing (WES) were performed to analyse transcriptomic abnormalities and mutations in splicing factors.ResultsApproximately 88% of the total reads from the RNA-seq were considered exonic, while the remaining reads (12%) were mapped to intronic or intergenic regions. We detected 28,941 alternative splicing events, some of which would produce abnormal proteins rarely found in normal cells. The detected events also included tumour-specific splicing alterations in oncogenes and tumour suppressors (e.g., HRAS, MDM2 and VEGFA). WES identified premature termination mutations or copy number losses in a total of 15 splicing regulator genes, including SF3B5, SRSF12 and TNPO3.ConclusionsThis study raises the possibility that IVNKTCL may be driven by multiple complex regulatory loops, including non-exonic expression and aberrant splicing, in addition to defects in epigenetic regulation.

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Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Transcriptome complexity in intravascular NK/T-cell lymphoma

Fujikura¹,

Yoshida²,

Uesaka

2020

J Clin Pathol

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“…IVL of T and NK-cell lineage are exceedingly rare and very few cases have been reported so far [ 2 – 18 ]. NK/T-cell IVL is still not recognized as a specific entity by the last WHO classification of lymphoid neoplasms, probably due to its rarity.…”

Section: Discussionmentioning

confidence: 99%

“…Skin and central nervous system (CNS) are most commonly involved, although virtually any organ can be affected [ 1 ]. The majority of cases are of B-cell origin [ 1 ], however, rare cases of T and natural killer (NK) immunophenotype have been reported [ 2 – 18 ]. IVL of B-cell phenotype are rarely Epstein-Barr virus (EBV) positive [ 8 ], whereas the frequent detection of EBV in NK/T cell IVL supports its possible pathogenic role.…”

Section: Introductionmentioning

confidence: 99%

Intravascular NK/T-cell lymphoma, Epstein–Barr virus positive with multiorgan involvement: a clinical dilemma

et al. 2018

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BackgroundIntravascular lymphoma is a rare type of non-Hodgkin lymphoma mostly of B-cell lineage. A few cases of intravascular lymphoma have been found to be of NK/T-cell origin, mainly affecting the skin and central nervous system.Case presentationA 54-year-old Caucasian man sought care because of a 2 weeks history of jaundice and intermittent fever, not responsive to antibiotics and antipyretics. Laboratory tests showed low blood oxygen concentration and pancytopenia. Serum microbiological tests were negative. Computerized tomography (CT) scan revealed hepatosplenomegaly and diffuse ground-glass opacities in both lungs without interlobular septal thickening. Despite oxygen therapy, the clinical conditions rapidly deteriorated leading to death 3 days after admission. Autopsy revealed a multiorgan involvement by an Epstein-Barr virus positive NK/T-cell lymphoma, strikingly growing within the blood vessel lumina, in absence of skin lesions.ConclusionsThe current case highlights the pathological features of this rare entity, the protean clinical presentation of which is often misleading, resulting in delayed diagnosis and treatment.

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Painful Cutaneous Plaques on the Lower Legs in a Middle-aged Woman

2020

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A 47-year-old woman presented with a 7-month history of multiple painful erythematous plaques on her lower legs. The lesions appeared as erythematous nodules on the left leg with tenderness and pruritus and rapidly progressed to both legs. Marked lower-leg edema was noted 20 days prior to the visit. She also had an intermittent fever during the past 3 months that did not respond to antibiotic therapy. The patient's medical history was unremarkable.Physical examination revealed ill-defined, indurated erythematous-violaceous plaques on the lower extremities with substantial edema (Figure , A and B). Complete blood cell count revealed a normal white blood cell count with moderate anemia (hemoglobin level, 87 g/L; normal range 115-150 g/L). Additional laboratory tests demonstrated an elevated level of serum lactate dehydrogenase (749 U/L; normal range 100-240 U/L) (to convert to μkat/L, multiply by 0.0167). Quantification of Epstein-Barr virus (EBV) DNA in sera showed a high viral load of EBV in peripheral blood (1 800 000 copies/mL, normal range <500 copies/mL). A skin biopsy specimen was obtained from a violaceous plaque (Figure , C).

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Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype

Cited by 19 publications

References 37 publications

Transcriptome complexity in intravascular NK/T-cell lymphoma

Transcriptome complexity in intravascular NK/T-cell lymphoma

Intravascular NK/T-cell lymphoma, Epstein–Barr virus positive with multiorgan involvement: a clinical dilemma

Painful Cutaneous Plaques on the Lower Legs in a Middle-aged Woman

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