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Aggressive NK-cell leukemia

MedGen UID:
266235
•Concept ID:
C1292777
•
Neoplastic Process
Synonym: Aggressive natural killer cell leukemia
SNOMED CT: Aggressive NK-cell leukemia (128833001); Aggressive NK cell leukemia (128833001); Aggressive natural killer-cell leukemia (128833001); Aggressive natural killer-cell leukemia (721310007)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019470
Orphanet: ORPHA86873

Definition

An extremely rare and highly aggressive neoplasm, usually manifesting in the third to fourth decade of life, affecting males and females equally, and characterized by the onset of high fever, weight loss, jaundice, skin infiltration, lymphadenopathy, hepatosplenomegaly, and severe anemia. It has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAggressive NK-cell leukemia
Follow this link to review classifications for Aggressive NK-cell leukemia in Orphanet.

Professional guidelines

PubMed

Recent advances in the diagnosis and treatment of natural killer/T-cell lymphomas.
Tse E, Au-Yeung R, Kwong YL
Expert Rev Hematol 2019 Nov;12(11):927-935. Epub 2019 Sep 5 doi: 10.1080/17474086.2019.1660640. PMID: 31487202
NK/T-cell lymphomas: pathobiology, prognosis and treatment paradigm.
Suzuki R
Curr Oncol Rep 2012 Oct;14(5):395-402. doi: 10.1007/s11912-012-0245-9. PMID: 22639106
Diagnosis and management of natural killer-cell malignancies.
Ishida F, Kwong YL
Expert Rev Hematol 2010 Oct;3(5):593-602. doi: 10.1586/ehm.10.51. PMID: 21083476

Recent clinical studies

Etiology

Allogeneic stem cell transplantation for patients with aggressive NK-cell leukemia.
Fujimoto A, Ishida F, Izutsu K, Yamasaki S, Chihara D, Suzumiya J, Mitsui T, Doki N, Sakai H, Kobayashi H, Kanda J, Fukuda T, Atsuta Y, Suzuki R
Bone Marrow Transplant 2021 Feb;56(2):347-356. Epub 2020 Aug 10 doi: 10.1038/s41409-020-01009-8. PMID: 32778688
Genomic and Immunophenotypic Landscape of Aggressive NK-Cell Leukemia.
El Hussein S, Patel KP, Fang H, Thakral B, Loghavi S, Kanagal-Shamanna R, Konoplev S, Jabbour EJ, Medeiros LJ, Khoury JD
Am J Surg Pathol 2020 Sep;44(9):1235-1243. doi: 10.1097/PAS.0000000000001518. PMID: 32590457
Epstein - Barr virus positive T and NK-cell lymphoproliferations: Morphological features and differential diagnosis.
Montes-Mojarro IA, Kim WY, Fend F, Quintanilla-Martinez L
Semin Diagn Pathol 2020 Jan;37(1):32-46. Epub 2019 Dec 17 doi: 10.1053/j.semdp.2019.12.004. PMID: 31889602
Natural killer cell neoplasms.
Liang X, Graham DK
Cancer 2008 Apr 1;112(7):1425-36. doi: 10.1002/cncr.23316. PMID: 18286525
Mature T-cell and NK-cell lymphomas in the pediatric age group.
Jaffe ES
Am J Clin Pathol 2004 Dec;122 Suppl:S110-21. doi: 10.1309/0DJW8GX2EKF7U1UG. PMID: 15690647

Diagnosis

EBV associated T- and NK-cell lymphoproliferative diseases: A comprehensive overview of clinical manifestations and novel therapeutic insights.
Shafiee A, Shamsi S, Kohandel Gargari O, Beiky M, Allahkarami MM, Miyanaji AB, Aghajanian S, Mozhgani SH
Rev Med Virol 2022 Jul;32(4):e2328. Epub 2022 Feb 4 doi: 10.1002/rmv.2328. PMID: 35122349
Genomic and Immunophenotypic Landscape of Aggressive NK-Cell Leukemia.
El Hussein S, Patel KP, Fang H, Thakral B, Loghavi S, Kanagal-Shamanna R, Konoplev S, Jabbour EJ, Medeiros LJ, Khoury JD
Am J Surg Pathol 2020 Sep;44(9):1235-1243. doi: 10.1097/PAS.0000000000001518. PMID: 32590457
Epstein - Barr virus positive T and NK-cell lymphoproliferations: Morphological features and differential diagnosis.
Montes-Mojarro IA, Kim WY, Fend F, Quintanilla-Martinez L
Semin Diagn Pathol 2020 Jan;37(1):32-46. Epub 2019 Dec 17 doi: 10.1053/j.semdp.2019.12.004. PMID: 31889602
Recent advances in the diagnosis and treatment of natural killer/T-cell lymphomas.
Tse E, Au-Yeung R, Kwong YL
Expert Rev Hematol 2019 Nov;12(11):927-935. Epub 2019 Sep 5 doi: 10.1080/17474086.2019.1660640. PMID: 31487202
Hepatic Lymphoma Diagnosis.
Choi WT, Gill RM
Surg Pathol Clin 2018 Jun;11(2):389-402. Epub 2018 Mar 21 doi: 10.1016/j.path.2018.02.003. PMID: 29751882

Therapy

Recent advances in the diagnosis and treatment of natural killer/T-cell lymphomas.
Tse E, Au-Yeung R, Kwong YL
Expert Rev Hematol 2019 Nov;12(11):927-935. Epub 2019 Sep 5 doi: 10.1080/17474086.2019.1660640. PMID: 31487202
NK/T-cell lymphomas: pathobiology, prognosis and treatment paradigm.
Suzuki R
Curr Oncol Rep 2012 Oct;14(5):395-402. doi: 10.1007/s11912-012-0245-9. PMID: 22639106
The diagnosis and management of extranodal NK/T-cell lymphoma, nasal-type and aggressive NK-cell leukemia.
Kwong YL
J Clin Exp Hematop 2011;51(1):21-8. doi: 10.3960/jslrt.51.21. PMID: 21628857
Treatment of advanced extranodal NK/T cell lymphoma, nasal-type and aggressive NK-cell leukemia.
Suzuki R
Int J Hematol 2010 Dec;92(5):697-701. Epub 2010 Dec 1 doi: 10.1007/s12185-010-0726-2. PMID: 21116747
Natural killer-cell neoplasms.
Greer JP, Mosse CA
Curr Hematol Malig Rep 2009 Oct;4(4):245-52. doi: 10.1007/s11899-009-0032-3. PMID: 20425414

Prognosis

Allogeneic stem cell transplantation for patients with aggressive NK-cell leukemia.
Fujimoto A, Ishida F, Izutsu K, Yamasaki S, Chihara D, Suzumiya J, Mitsui T, Doki N, Sakai H, Kobayashi H, Kanda J, Fukuda T, Atsuta Y, Suzuki R
Bone Marrow Transplant 2021 Feb;56(2):347-356. Epub 2020 Aug 10 doi: 10.1038/s41409-020-01009-8. PMID: 32778688
EBV in T-/NK-Cell Tumorigenesis.
Kimura H
Adv Exp Med Biol 2018;1045:459-475. doi: 10.1007/978-981-10-7230-7_21. PMID: 29896680
NK/T-cell lymphomas: pathobiology, prognosis and treatment paradigm.
Suzuki R
Curr Oncol Rep 2012 Oct;14(5):395-402. doi: 10.1007/s11912-012-0245-9. PMID: 22639106
Natural killer-cell neoplasms.
Greer JP, Mosse CA
Curr Hematol Malig Rep 2009 Oct;4(4):245-52. doi: 10.1007/s11899-009-0032-3. PMID: 20425414
Mature T-cell and NK-cell lymphomas in the pediatric age group.
Jaffe ES
Am J Clin Pathol 2004 Dec;122 Suppl:S110-21. doi: 10.1309/0DJW8GX2EKF7U1UG. PMID: 15690647

Clinical prediction guides

Clinical features and prognosis of chronic natural killer cell lymphoproliferative disorders.
Liu DL, Wang YJ, Qian SY, Ma SS, Ding MJ, Dong M, Zhang JM, Zhang MZ, Chen QJ, Zhang XD
Hematology 2024 Dec;29(1):2307817. Epub 2024 Feb 6 doi: 10.1080/16078454.2024.2307817. PMID: 38319083
Elevated serum granulysin and its clinical relevance in mature NK-cell neoplasms.
Sekiguchi N, Asano N, Ito T, Momose K, Momose M, Ishida F
Int J Hematol 2012 Oct;96(4):461-8. Epub 2012 Aug 14 doi: 10.1007/s12185-012-1159-x. PMID: 22890551
Chemokine system and tissue infiltration in aggressive NK-cell leukemia.
Makishima H, Ito T, Momose K, Nakazawa H, Shimodaira S, Kamijo Y, Nakazawa Y, Ichikawa N, Ueno M, Kobayashi H, Kitano K, Saito H, Kiyosawa K, Ishida F
Leuk Res 2007 Sep;31(9):1237-45. Epub 2006 Nov 22 doi: 10.1016/j.leukres.2006.10.020. PMID: 17123604
Natural killer cell neoplasms.
Tse E, Liang RH
Clin Lymphoma 2004 Dec;5(3):197-201. doi: 10.3816/clm.2004.n.027. PMID: 15636697
Splenic involvement by peripheral T-cell and NK-cell neoplasms.
Chan JK
Semin Diagn Pathol 2003 May;20(2):105-20. doi: 10.1016/s0740-2570(03)00013-3. PMID: 12945934

Recent systematic reviews

Diseases of large granular lymphocytes.
Alekshun TJ, Sokol L
Cancer Control 2007 Apr;14(2):141-50. doi: 10.1177/107327480701400207. PMID: 17387299

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