How we treat NK/T-cell lymphomas

Tse, Eric; Zhao, Wei‐Li; Xiong, Jie; Kwong, Yok–Lam

doi:10.1186/s13045-022-01293-5

Cited by 66 publications

(59 citation statements)

References 99 publications

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“…However, when MFCI was added, the diagnostic rate of primary PPL increased to 100%[ 18 ]. Presently, in the course of NKTCL treatment, asparaginase-containing chemotherapeutic schemes are the standard treatment regimen[ 19 ]. Based on previous experience, SMILE is the most popular regimen for the treatment of NKTCL[ 20 ].…”

Section: Discussionmentioning

confidence: 99%

Intestinal natural killer/T-cell lymphoma presenting as a pancreatic head space-occupying lesion: A case report

Wang¹,

Zhu²,

Lei³

et al. 2023

World J Gastrointest Oncol

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BACKGROUND Intestinal natural killer/T-cell lymphoma (NKTCL) is a rare and aggressive non-Hodgkin’s lymphoma, and its occurrence is closely related to Epstein-Barr virus infection. In addition, the clinical symptoms of NKTCL are not obvious, and the specific pathogenesis is still uncertain. While NKTCL may occur in any segment of the intestinal tract, its distinct location in the periampullary region, which leads clinicians to consider mimics of a pancreatic head mass, should also be addressed. Therefore, there remain huge challenges in the diagnosis and treatment of intestinal NKTCL. CASE SUMMARY In this case, we introduce a male who presented to the clinic with edema of both lower limbs, accompanied by diarrhea, and abdominal pain. Endoscopic ultrasound (EUS) showed well-defined homogeneous hypoechoic lesions with abundant blood flow signals and compression signs in the head of the pancreas. Under the guidance of EUS- fine needle biopsy (FNB) with 19 gauge or 22 gauge needles, combined with multicolor flow cytometry immunophenotyping (MFCI) helped us diagnose NKTCL. During treatments, the patient was prescribed the steroid (dexamethasone), methotrexate, ifosfamide, L-asparaginase, and etoposide chemotherapy regimen. Unfortunately, he died of leukopenia and severe septic shock in a local hospital. CONCLUSION Clinicians should enhance their understanding of NKTCL. Some key factors, including EUS characteristics, the right choice of FNB needle, and combination with MFCI, are crucial for improving the diagnostic rate and reducing the misdiagnosis rate.

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Section: Discussionmentioning

confidence: 99%

Intestinal natural killer/T-cell lymphoma presenting as a pancreatic head space-occupying lesion: A case report

Wang¹,

Zhu²,

Lei³

et al. 2023

World J Gastrointest Oncol

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“…The role of HSCT in the treatment of ENKTCL is controversial. Auto-HSCT did not lead to better outcomes compared to Asp-based CT (176,177). Several small cohort retrospective studies have evaluated the efficacy of allo-HSCT in patients with advanced-stage ENKTCL.…”

Section: Hsctmentioning

confidence: 93%

Treatment of extranodal NK/T-cell lymphoma: From past to future

et al. 2023

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Extranodal NK/T-cell lymphoma (ENKTCL) is the most common subtype of T/NK-cell lymphoma in Asia and Latin America, but very rare in North American and Europe. Patient survival has improved significantly over the past two decades. However, standard treatment has not yet been established, although dozens of prospective trials have been conducted. To help understand how the treatment of ENKTCL has evolved in the past and what trends lie ahead, we have comprehensively reviewed the treatment of this aggressive malignancy, with a particular focus on neglected or unanswered issues, such as the optimal staging method, the best partner of asparaginase (Asp), the individualized administration of Asp, the preferred sequence of CT and RT and so on. Overall, the 5-year overall survival (OS) of patients with Ann Arbor stage I/II disease increased from < 50% in the early 20th century to > 80% in recent years, and the median OS of patients with Ann Arbor stage III/IV disease increased from < 1 year to more than 3 years. The improvement in patient survival is largely attributable to advances in radiation technology and the introduction of Asp and anti-PD-1/PD-L1 immunotherapy into practice. Radiotherapy is essential for patients with early-stage disease, while Asp-based chemotherapy (CT) and PD-1/PD-L1 inhibitors significantly improved the prognosis of patients with advanced-stage disease. ENKTCL management is trending toward simpler regimens, less toxicity, and higher efficacy. Novel drugs, such as manufactured T cells, monoclonal antibodies, and small molecule inhibitors, are being intensively investigated. Based on the fact that ENKTCL is highly resistant to cytotoxic drugs except Asp, and aggressive CT leads to higher toxicity rather than better outcomes, we recommend it is unnecessary to expend additional resources to compare different combinations of Asp with cytotoxic agents. Instead, more efforts should be made to optimize the use of Asp and immunotherapy to maximize efficacy and minimize toxicity, explore ways to overcome resistance to Asp and immunotherapy, identify novel treatment targets, and define subpopulations who may benefit more from specific treatments.

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“…ENKTL is notoriously resistant to anthracycline-based chemotherapy, whereas the use of L-asparaginase-based chemotherapy combined with radiotherapy has improved the outcome for this aggressive lymphoma [ 78 ]. However, as chemotherapy is often unable to achieve long-term remissions in advanced stages of the disease, it is extremely important that novel therapies targeting different molecular pathways are currently available [ 79 , 80 ].…”

Section: Differential Diagnoses Of Ivnktlmentioning

confidence: 99%

“…Despite still lacking a standardized expression cutoff for predicting the response to PD-1/PD-L1 inhibitors, several clinical trials are ongoing, and immune checkpoint inhibitor therapy is used in relapsed or refractory disease [ 81 ]. Due to the relevant role played by the activation of both JAK/STAT and NF-kB pathways in ENKTL pathogenesis, these pathways are potentially targetable for therapy [ 74 , 75 , 76 , 79 , 80 ]. Further studies are needed to evaluate the efficacy of the anti-CD30 antibody brentuximab vedotin, particularly in combination with chemotherapy, for the treatment of ENKTL [ 82 ].…”

Section: Differential Diagnoses Of Ivnktlmentioning

confidence: 99%

Intravascular NK/T-Cell Lymphoma: What We Know about This Diagnostically Challenging, Aggressive Disease

Zanelli

Parente

Sanguedolce

et al. 2022

Cancers

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Intravascular lymphoma is a form of lymphoid malignancy characterized by neoplastic cells growing almost exclusively within the lumina of small- to medium-sized blood vessels. Most cases are of B-cell origin with rare cases of natural killer or T-cell lineage. Extranodal sites are affected, mainly the skin and central nervous system, although any organ may be involved. Intravascular NK/T-cell lymphoma deserves special attention because of its clinicopathologic features and the need for adequate immunophenotyping combined with clonality test for a proper diagnosis. Moreover, intravascular NK/T-cell lymphoma is strongly linked to Epstein–Barr virus (EBV), which is considered to play a role in tumorigenesis and to be responsible for the aggressive behavior of the disease. In this paper, we review the current knowledge on this rare lymphoma and, in particular, the most recent advances about its molecular landscape. The main distinguishing features with other EBV-related entities, such as extranodal NK/T-cell lymphoma, EBV-positive primary nodal T/NK-cell lymphoma, and aggressive NK-cell leukemia, are discussed to help pathologists obtain the correct diagnosis and consequently develop an adequate and prompt therapy response.

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How we treat NK/T-cell lymphomas

Cited by 66 publications

References 99 publications

Intestinal natural killer/T-cell lymphoma presenting as a pancreatic head space-occupying lesion: A case report

Intestinal natural killer/T-cell lymphoma presenting as a pancreatic head space-occupying lesion: A case report

Treatment of extranodal NK/T-cell lymphoma: From past to future

Intravascular NK/T-Cell Lymphoma: What We Know about This Diagnostically Challenging, Aggressive Disease

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