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WHO Classification of Tumours
Natural killer (NK) cell lymphoblastic leukaemia/lymphoma
Tumours of haematopoietic and lymphoid tissues


Definition

This neoplasm has been very difficult to define, and there is considerable confusion in the literature. Contributing to the confusion is that many cases reported as NK leukaemia because of the expression of N-CAM (CD56) are now recognized to represent cases of plasmacytoid dendritic cell leukaemia
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Petrella T, Bagot M, Willemze R, Beylot-Barry M, Vergier B, Delaunay M, Meijer CJ, Courville P, Joly P, Grange F, De Muret A, Machet L, Dompmartin A, Bosq J, Durlach A, Bernard P, Dalac S, Dechelotte P, D'Incan M, Wechsler J, Teitell MA (2005)
Blastic NK-cell lymphomas (agranular CD4+CD56+ hematodermic neoplasms): a review.
Am J Clin Pathol 123: 662-75




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Petrella T, Comeau MR, Maynadiť M, Couillault G, De Muret A, Maliszewski CR, Dalac S, Durlach A, Galibert L (2002)
'Agranular CD4+ CD56+ hematodermic neoplasm' (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes.
Am J Surg Pathol 26: 852-62



. Similarly, the entity of myeloid/NK-cell acute leukaemia
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Scott AA, Head DR, Kopecky KJ, Appelbaum FR, Theil KS, Grever MR, Chen IM, Whittaker MH, Griffith BB, Licht JD (1994)
HLA-DR-, CD33+, CD56+, CD16- myeloid/natural killer cell acute leukemia: a previously unrecognized form of acute leukemia potentially misdiagnosed as French-American-British acute myeloid leukemia-M3.
Blood 84: 244-55




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Suzuki R, Yamamoto K, Seto M, Kagami Y, Ogura M, Yatabe Y, Suchi T, Kodera Y, Morishima Y, Takahashi T, Saito H, Ueda R, Nakamura S (1997)
CD7+ and CD56+ myeloid/natural killer cell precursor acute leukemia: a distinct hematolymphoid disease entity.
Blood 90: 2417-28



, which has been suggested to be of precursor NK origin
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Oshimi K (2007)
Progress in understanding and managing natural killer-cell malignancies.
Br J Haematol 139: 532-44



has a primitive immunophenotype that cannot be distinguished from acute myeloid leukaemia with minimal differentiation, and until further evidence emerges these should be considered as cases of AML.
Early in development, NK-cell progenitors express no specific markers

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Freud AG, Caligiuri MA (2006)
Human natural killer cell development.
Immunol Rev 214: 56-72



or express markers that overlap with those seen in T-cell ALL, including CD7, CD2 and even CD5 and cytoplasmic CD3ε
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Spits H, Lanier LL, Phillips JH (1995)
Development of human T and natural killer cells.
Blood 85: 2654-70



, so that distinguishing between T-ALL and NK-cell tumours may be difficult. More mature but more specific markers such as CD16 are rarely expressed in any acute leukaemia, while some markers that might be considered relatively more specific, but still expressed on NK progenitors, such as CD94 or CD161
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Freud AG, Caligiuri MA (2006)
Human natural killer cell development.
Immunol Rev 214: 56-72



are not commonly tested. Some well characterized cases of NK precursor tumours with lymphomatous presentations that expressed NK-specific CD94 1A transcripts have been described
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Lin CW, Liu TY, Chen SU, Wang KT, Medeiros LJ, Hsu SM (2005)
CD94 1A transcripts characterize lymphoblastic lymphoma/leukemia of immature natural killer cell origin with distinct clinical features.
Blood 106: 3567-74



. It is hoped that wider availability of more specific NK markers including panels of antibodies against killer immunoglobulin-≠like receptors (KIRs) will help clarify this disease, but until then this is best considered a provisional entity. The diagnosis of precursor NK lymphoblastic leukaemia/ lymphoma may be considered in a case that expresses CD56 along with immature T-associated markers such as CD7 and CD2 and even including cCD3, provided that it lacks B-cell and myeloid markers, T-cell and IG receptor genes are in the germline configuration
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Kawano S, Tatsumi E, Yoneda N, Yamaguchi N, Goji J, Ito H, Nagai T, Nishikori M, Okamura A, Koiwai O (1995)
Novel leukemic lymphoma with probable derivation from immature stage of natural killer (NK) lineage in an aged patient.
Hematol Oncol 13: 1-11




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Koita H, Suzumiya J, Ohshima K, Takeshita M, Kimura N, Kikuchi M, Koono M (1997)
Lymphoblastic lymphoma expressing natural killer cell phenotype with involvement of the mediastinum and nasal cavity.
Am J Surg Pathol 21: 242-8




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Oshimi K (2007)
Progress in understanding and managing natural killer-cell malignancies.
Br J Haematol 139: 532-44



and blastic plasmacytoid dendritic cell leukaemia has been excluded.

> Related Topics
Overview: Acute leukaemias of ambiguous lineage