Changes

==Cancer Category/Type==

==Cancer Category/Type==

[[Myeloproliferative Neoplasms (MPN)|Myeloproliferative Neoplasms]]

[[HAEM4:Myeloproliferative Neoplasms (MPN)|Myeloproliferative Neoplasms]]

==Cancer Sub-Classification / Subtype==

==Cancer Sub-Classification / Subtype==

Chronic neutrophilic leukemia (CNL) is a rare heterogeneous BCR/ABL1-negative member of the WHO myeloproliferative neoplasm (MPN) category, which presents with neutrophilia lacking dysplastic features.  Diagnostic criteria include sustained increased white blood cells in the peripheral blood for more than 3 months, with a predominance of band and segmented forms equal to or in excess of 25 x10⁹/L.<ref name=":0" />  The bone marrow is hypercellular with elevated myeloid to erythroid ratio, due to increase in neutrophilic granulocyte proliferation, with normal maturation (>80% mature forms of neutrophils and <10% neutrophil precursors) and rare (<5%) of nucleated cells represented by myeloblasts.  This disease is also associated with hepatosplenomegaly, but can affect other tissues.

Chronic neutrophilic leukemia (CNL) is a rare heterogeneous BCR/ABL1-negative member of the WHO myeloproliferative neoplasm (MPN) category, which presents with neutrophilia lacking dysplastic features.  Diagnostic criteria include sustained increased white blood cells in the peripheral blood for more than 3 months, with a predominance of band and segmented forms equal to or in excess of 25 x10⁹/L.<ref name=":0" />  The bone marrow is hypercellular with elevated myeloid to erythroid ratio, due to increase in neutrophilic granulocyte proliferation, with normal maturation (>80% mature forms of neutrophils and <10% neutrophil precursors) and rare (<5%) of nucleated cells represented by myeloblasts.  This disease is also associated with hepatosplenomegaly, but can affect other tissues.

Diagnosis is made after exclusion of reactive neutrophilia (especially due to plasma cell neoplasms), atypical CML and of other myeloproliferative neoplasms ([[Chronic Myeloid Leukemia (CML), BCR-ABL1 Positive|BCR-ABL1- positive Chronic Myeloid Leukemia]], [[Polycythemia Vera (PV)|Polycythemia Vera]], [[Essential Thrombocythemia (ET)|Essential Thrombocythemia]] and [[Primary Myelofibrosis (PMF)|Primary Myelofibrosis]]) and and myelodysplastic syndrome/myeloproliferative neoplasms.<ref name=":0" /><ref name=":3">{{Cite journal|last=Szuber|first=Natasha|last2=Elliott|first2=Michelle|last3=Tefferi|first3=Ayalew|date=02 2020|title=Chronic neutrophilic leukemia: 2020 update on diagnosis, molecular genetics, prognosis, and management|url=https://pubmed.ncbi.nlm.nih.gov/31769070|journal=American Journal of Hematology|volume=95|issue=2|pages=212–224|doi=10.1002/ajh.25688|issn=1096-8652|pmid=31769070}}</ref><ref name=":4" />  Previous use of G-CSF and leukaemogenic drugs (busulphan, melphalan) must also be excluded.

Diagnosis is made after exclusion of reactive neutrophilia (especially due to plasma cell neoplasms), atypical CML and of other myeloproliferative neoplasms ([[HAEM5:Chronic myeloid leukaemia|BCR-ABL1- positive Chronic Myeloid Leukemia]], [[HAEM5:Polycythaemia vera|Polycythemia Vera]], [[HAEM5:Essential thrombocythaemia|Essential Thrombocythemia]] and [[HAEM5:Primary myelofibrosis|Primary Myelofibrosis]]) and and myelodysplastic syndrome/myeloproliferative neoplasms.<ref name=":0" /><ref name=":3">{{Cite journal|last=Szuber|first=Natasha|last2=Elliott|first2=Michelle|last3=Tefferi|first3=Ayalew|date=02 2020|title=Chronic neutrophilic leukemia: 2020 update on diagnosis, molecular genetics, prognosis, and management|url=https://pubmed.ncbi.nlm.nih.gov/31769070|journal=American Journal of Hematology|volume=95|issue=2|pages=212–224|doi=10.1002/ajh.25688|issn=1096-8652|pmid=31769070}}</ref><ref name=":4" />  Previous use of G-CSF and leukaemogenic drugs (busulphan, melphalan) must also be excluded.

Mutations in ''CSF3R'' (colony-stimulating factor 3 receptor) are present in the majority of CNL cases and are part of diagnostic criteria. Genetic rearrangement for ''PDGFRA'', ''PDGFRB'', ''FGFR1'', and ''PCM1''-''JAK2'' fusion must be absent.<ref name=":0">Bain BJ, et al., (2017). Chronic Neutrophilic Leukaemia, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p37-38.</ref>

Mutations in ''CSF3R'' (colony-stimulating factor 3 receptor) are present in the majority of CNL cases and are part of diagnostic criteria. Genetic rearrangement for ''PDGFRA'', ''PDGFRB'', ''FGFR1'', and ''PCM1''-''JAK2'' fusion must be absent.<ref name=":0">Bain BJ, et al., (2017). Chronic Neutrophilic Leukaemia, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p37-38.</ref>