Aggressive NK-cell leukemia: Difference between revisions

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	Aggressive NK-cell leukemia;
ICD-O:	9948/3

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Revision as of 19:57, 18 September 2012

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

Synonyms and related key words: Aggressive natural killer cell leukemia; aggressive NK-cell leukemia; aggressive NK-cell lymphoma; large granular lymphocyte leukemia, NK-cell type

Morphology

Peripheral blood

The leukemic cells have a diameter mildly greater than a large granular lymphocyte (LGL) and have azurophilic granules and nucleoli of varying prominence. Nuclei may be irregular and hyperchromatic.^[1]

Bone marrow

Bone marrow involvement runs the spectrum between an inconspicuous infiltrate to extensive marrow replacement by leukemic cells. Reactive histiocytes displaying hemophagocytosis can been seen interspersed in the neoplastic infiltrate.^[1]

Other organs

Leukemic involvement of organs is typically destructive on tissue sections with necrosis and possibly angioinvasion, and the monotonous infiltrate may be diffuse or patchy.^[1]

Molecular findings

Immunophenotype

The immunophenotype of this disease is the same as extranodal NK/T-cell lymphoma, nasal type and is shown in the table below. CD11b and CD16 show variable expression.^[2]^[3]

Status	Antigens
Positive	CD2, CD3ε, CD56, perforin, granzyme B, TIA-1
Negative	CD57

Genetic findings

Due to the myeloid lineage, clonal rearrangements of lymphoid (T cell receptor; B cell receptor) genes are not seen.^[1] The genome of the Epstein Barr virus (EBV) is detected in many cases^[4], along with a variety of chromosomal abnormalities.^[5]

Current articles

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References

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↑ [1] Wong KF, Zhang YM, Chan JK. "Cytogenetic abnormalities in natural killer cell lymphoma/leukaemia--is there a consistent pattern?" Leuk Lymphoma. 1999 Jul;34(3-4):241-50. PMID: 10439361

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[won1-5] [1] Wong KF, Zhang YM, Chan JK. "Cytogenetic abnormalities in natural killer cell lymphoma/leukaemia--is there a consistent pattern?" Leuk Lymphoma. 1999 Jul;34(3-4):241-50. PMID: 10439361

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@@ Line 17: / Line 17: @@
 '''Synonyms and related key words''': Aggressive natural killer cell leukemia; aggressive NK-cell leukemia; aggressive NK-cell lymphoma; large granular lymphocyte leukemia, NK-cell type
-==Clinical features==
-===Etiology===
-This disease has a strong association with the [[Epstein-Barr virus]] (EBV)<ref name="gel1"/>, but the true pathogenesis of this disease has yet to be described. The cell of origin is believed to be an [[NK cell]].<ref name="who1"/> Blastoid NK cell lymphoma appears to be a different entity and shows no association with EBV.<ref name="cha1"/>
-===Presentation===
-Patients usually present with constitutional symptoms ([[malaise]], [[weight loss]], [[fatigue (medical)|fatigue]]), and [[hepatosplenomegaly]] is commonly found on physical exam. [[Lymphadenopathy]] is also found to a lesser extent. Due to the aggressive nature of the disease, patients may initially present at a more advanced stage, with [[coagulopathies]], hemophagocytic syndrome, and multi-organ failure.<ref name="cha1"/><ref name="ima1"/><ref name="kwo1"/><ref name="kob1">
-[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8854571&query_hl=29&itool=pubmed_ExternalLink]
-Kobayashi Y, Uehara S, Inamori K, Shirato R, Ozawa K, Sklar J, Asano S. "Hemophagocytosis as a para-neoplastic syndrome in NK cell leukemia." '''Int J Hematol'''. 1996 Aug;64(2):135-42. PMID: 8854571</ref><ref name="oku1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=1746541&query_hl=31&itool=pubmed_ExternalLink]
-Okuda T, Sakamoto S, Deguchi T, Misawa S, Kashima K, Yoshihara T, Ikushima S, Hibi S, Imashuku S. "Hemophagocytic syndrome associated with aggressive natural killer cell leukemia." '''Am J Hematol'''. 1991 Dec;38(4):321-3. PMID: 1746541</ref>
-===Laboratory findings===
-Leukemic cells are invariably present in samples of peripheral [[blood]] to a variable extent. [[Pancytopenia]] ([[anemia]], [[neutropenia]], [[thrombocytopenia]]) is commonly seen as well.<ref name="who1"/>
-===Sites of involvement===
-This disease is typically found and diagnosed in peripheral [[blood]], and while it can involve any organ, it is usually found in the [[spleen]], [[liver]], and [[bone marrow]].<ref name="who1"/>
 ==Morphology==

v t e Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208)
CFU-GM/ and other granulocytes	Template:Navbox subgroup
MEP	Template:Navbox subgroup
CFU-Mast	Mastocytoma (Mast cell leukemia, Mast cell sarcoma, Systemic mastocytosis)
Multiple/unknown	AML (Acute panmyelosis with myelofibrosis, Myeloid sarcoma) · MP (Myelofibrosis) · Acute biphenotypic leukaemia
See also hematology, lymphoid malignancy