Aggressive NK Cell Leukemia: Current State of the Art

Hussein, Siba El; Medeiros, L. Jeffrey; Khoury, Joseph D.

doi:10.3390/cancers12102900

Cited by 27 publications

(17 citation statements)

References 85 publications

(193 reference statements)

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“…Current therapy for ANKL is chemotherapy 118 in defined combinations such as SMILE (dexamethasone, MTX, ifosfamide, L-asparaginase and etoposide), AspaMetDex (L-asparaginase, MTX, and dexamethasone), VIDL (etoposide, ifosfamide, dexamethasone and L-asparaginase) [119][120][121] or anthracycline-containing regimens 103 . Patients that are EBV negative after treatment have better prognosis 119 .…”

Section: New Therapeutic Approachesmentioning

confidence: 99%

Recent advances in T-cell lymphoid neoplasms

Bigas

Rodríguez‐Sevilla

Espinosa

et al. 2022

Experimental Hematology

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This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Section: New Therapeutic Approachesmentioning

confidence: 99%

Recent advances in T-cell lymphoid neoplasms

Bigas

Rodríguez‐Sevilla

Espinosa

et al. 2022

Experimental Hematology

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“…The polycomb repressive complex 2 (PRC2) receptor is a multiprotein complex that is involved in targeting genes for epigenetic silencing [ 35 ]. JAK/STAT pathways may play a role in the overexpression of MYC, which then interacts with PRC2 signaling histone modification [ 36 ]. This finding may suggest a novel therapeutic target.…”

Section: Reviewmentioning

confidence: 99%

Aggressive Natural Killer Cell Leukemia: A Brief Overview of Its Genomic Landscape, Histological Features, and Current Management

Sumbly¹,

Vest²,

Landry³

2022

Cureus

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Aggressive natural killer-cell leukemia (ANKL) is a rare hematological malignancy characterized by the abnormal proliferation of natural killer (NK) cells. There are currently no therapies approved by the US Food and Drug Administration (FDA) for the treatment of ANKL, but advancements in genomics are assisting in the unraveling of this rare malignancy. We selected 37 articles that contained information on genomics, immunohistochemistry, and/or current clinical trials relating to the treatment and survival of ANKL. Current therapeutic strategies have been subdivided into (1) concurrent chemoradiation, (2) sequential chemoradiation, and (3) sandwich chemoradiation. These methods have been developed to reduce toxicity while still producing a pathologic response. Concurrent chemoradiation with VIDL (etoposide, ifosfamide, dexamethasone, and L-asparaginase) produced an excellent clinical response, while sequential chemoradiation with SMILE (steroid dexamethasone, methotrexate, ifosfamide, L-asparaginase, and etoposide) showed an adequate response, but with severe hematologic toxicity. The efficacy of Lasparaginase in chemotherapeutic regimens and its association with NK-cell apoptosis have led to its inclusion in all standard regimens. Future studies are focusing on the addition of a programmed deathligand 1 (PD-L1) inhibitor and hematopoietic stem cell transplant (HSCT).

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“…Its course is indolent with an overall 10-year survival rate of 70% (1). Massive hepatosplenic and bone marrow infiltration of NK-LGLs and rapidly progressive NK cell blood lymphocytosis, is related to aggressive NK cell leukemia, a rare and distinct entity with a poor prognosis (42). Symptoms are mainly due to infections (mouth ulcers, ENT or lung infections, severe sepsis) secondary to severe neutropenia which is the most common cytopenia.…”

Section: Clinical Characteristics Of Nk-lgl Leukemiamentioning

confidence: 99%

Toward a Better Classification System for NK-LGL Disorders

et al. 2022

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Large granular lymphocytic leukemia is a rare lymphoproliferative disorder characterized by a clonal expansion of T-lineage lymphocyte or natural killer (NK) cells in 85 and 15% of cases respectively. T and NK large granular leukemia share common pathophysiology, clinical and biological presentation. The disease is characterized by cytopenia and a frequent association with autoimmune manifestations. Despite an indolent course allowing a watch and wait attitude in the majority of patients at diagnosis, two third of the patient will eventually need a treatment during the course of the disease. Unlike T lymphocyte, NK cells do not express T cell receptor making the proof of clonality difficult. Indeed, the distinction between clonal and reactive NK-cell expansion observed in several situations such as autoimmune diseases and viral infections is challenging. Advances in our understanding of the pathogenesis with the recent identification of recurrent mutations provide new tools to prove the clonality. In this review, we will discuss the pathophysiology of NK large granular leukemia, the recent advances in the diagnosis and therapeutic strategies.

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Aggressive NK Cell Leukemia: Current State of the Art

Cited by 27 publications

References 85 publications

Recent advances in T-cell lymphoid neoplasms

Recent advances in T-cell lymphoid neoplasms

Aggressive Natural Killer Cell Leukemia: A Brief Overview of Its Genomic Landscape, Histological Features, and Current Management

Toward a Better Classification System for NK-LGL Disorders

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