Intravascular <scp>NK</scp>/T‐cell lymphoma: a report of five cases with cutaneous manifestation from China

Wang, Lei; Chen, Siyuan; Ma, Han; Shi, Dongmei; Huang, Changzheng; Lu, Chun; Gao, Tianwen; Wang, Gang

doi:10.1111/cup.12515

Cited by 16 publications

(18 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In our cohort, 2 Caucasian patients (7.7%) presented “Asian variant” IVL; this observation is not unusual, as cases of “classical” IVL have been reported in Asians and “Asian variant” IVL has been reported in Europeans and Africans. [ 3 , 39 – 44 ] This leads us to believe that IVL nomenclature should use “classical IVL” and “IVL associated with hemophagocytic syndrome,” instead of “European IVL” and “Asian variant” IVL.…”

Section: Discussionmentioning

confidence: 99%

Retrospective study of intravascular large B-cell lymphoma cases diagnosed in Quebec

et al. 2017

View full text Add to dashboard Cite

Introduction:Intravascular large B-cell lymphoma (IVL) is an extremely rare malignancy, mainly studied through European and Asian series. Due to the low incidence of this condition, our understanding of the clinical presentation as well as the management of IVL relies on a limited number of patients.We report the largest North American study to date on IVL with 29 cases from Quebec hospital diagnosed between 1990 and 2016. The aim of our study is to describe the clinical presentations, diagnostic and staging procedures, therapeutic management and clinical outcomes of IVL patients in our population and compare the disease phenotype to European and Asian series reported.In our cohort, all patients had stage IV IVL at diagnosis, with a median age of 66.7 years (range 47.2–90.8). Clinical presentation was characterized by constitutional symptoms (100%), poor ECOG-PS (100% ≥ 2), cytopenias (93% anemia), and elevated lactate dehydrogenase (97%) and C-reactive protein (96%). Our cohort presented with mainly cutaneous and neurological symptoms. However, neurological involvement (75.9%) was predominant and no “cutaneous variant” was observed; this differs from European literature, where “classical” IVL is reported with mainly cutaneous involvement. Two of our Caucasian patients presented “Asian variant” IVL; this observation is not unusual, as cases of “classical” IVL have been reported in Asians and “Asian variant” IVL has been reported in Europeans. All patients were classified according to their immunophenotypic features in 3 different subgroups (CD5+ or CD5−CD10+, CD5−CD10−, CD5+CD10−) with no difference in outcome. Finally, 62% of our cohort received anthracycline-based chemotherapy and 53% of them achieved a complete response. After a median follow-up of 328 days, OS at 3 years was 42.7% for the entire cohort and 47.4% for the cases with in vivo diagnosis.Conclusion:Unlike European studies on “classical” IVL, our study showed that the French Canadian presentation of this subtype of IVL is more frequently observed with neurological rather than cutaneous involvement. Finally, an early diagnosis is of primary importance since almost a quarter of patients receive a post-mortem diagnosis. A prompt diagnosis allows the introduction of an early treatment, associated with a CR in 53% of patients.

show abstract

Section: Discussionmentioning

confidence: 99%

Retrospective study of intravascular large B-cell lymphoma cases diagnosed in Quebec

et al. 2017

View full text Add to dashboard Cite

show abstract

“…Wang et al [ 13 ] reported 5 cases of NK/T-cell IVL, but, as commented by Alegria-Landa et al [ 8 ] in 2017, the possibility of an extranodal NK/T-cell lymphoma nasal-type in their case number 2 cannot be excluded. As well in their case number 5, the presence of circulating atypical CD56 positive lymphocytes, makes aggressive NK leukemia the most likely diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…NK/T-cell IVL often follows an aggressive clinical course, however, the prognosis may vary with the extent of disease, being more favorable in patients with exclusive skin lesions. In particular 2 cases with the disease limited to the skin had a really favorable outcome, being in complete remission after 1.5 and 3 years [ 13 , 15 ]. However, the limited number of cases makes difficult to draw any conclusion.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2018

View full text Add to dashboard Cite

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.

show abstract

“…It is worth remembering that CD30 could be expressed by any type of EBV-related lymphoproliferative disease and, in fact, it was positive in our case and in four other published cases of NK/T IVLs. 13,22,28,29 A few cases of primary cutaneous anaplastic CD30 + T cell lymphoma with exclusive intravascular localization have also been described. [35][36][37][38] However, in contrast with NK/T IVLs, in cutaneous anaplastic CD30 + IVLs neoplastic cells are confined within the D2-40-positive dermal lymphatic vessels, EBER-1 is negative and the course of this cutaneous anaplastic CD30 + IVL is mainly indolent.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype

et al. 2017

View full text Add to dashboard Cite

Intravascular lymphoma (IVL) is a rare entity. Most cases are a variant of extranodal diffuse large B cell lymphoma, and fewer than 10% of the published cases are of T cell origin. Only intravascular B cell lymphoma is recognized as a distinct entity in the most recent World Health Organization (WHO) classification of lymphoproliferative disorders. We describe a case of cutaneous natural killer (NK)/T IVL, with a cytotoxic immunophenotype and Epstein-Barr virus (EBV) positivity. However, our case was immunohistochemically negative not only for T cell receptor (TCR)-βF1 and TCR-γ (TCR-silent), but also for CD56, making it the first triple-negative NK/T IVL case to be described. We urge recognition of this NK/T cell lineage intravascular lymphoma due to its particular immunophenotypical profile and its unvarying relationship with EBV. Its occurrence should not be considered a coincidence, but rather a key aspect of the pathogenic background of this haematological neoplasm.

show abstract

Intravascular NK/T‐cell lymphoma: a report of five cases with cutaneous manifestation from China

Abstract: Intravascular NK/T-cell lymphoma is a rare highly aggressive and EBV-associated lymphoma that is prone to develop in Chinese patients. The relationship between intravascular NK/T-cell lymphoma and extranodal NK/T-cell lymphoma, nasal type, requires clarification.

Cited by 16 publications

References 37 publications

Retrospective study of intravascular large B-cell lymphoma cases diagnosed in Quebec

Retrospective study of intravascular large B-cell lymphoma cases diagnosed in Quebec

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

Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype

Contact Info

Product

Resources

About