This article's lead sectionmay be too short to adequately summarize the key points. Please consider expanding the lead to provide an accessible overview of all important aspects of the article.(December 2015)
The cause of CNL is currently unknown. An association between CNL and multiple myeloma has been suggested based on the observation of myeloma in 20% of CNL cases.[5] However, a clonal genetic abnormality has not been detected in these myeloma-associated cases of CNL, raising the possibility that the neutrophilia is a reaction due to the neoplastic myeloma cells.[2] The postulated cell of origin is a limited-potential, marrow-derived stem cell.[6]
Geneticsedit
The majority (90%) of cases have not had detectable cytogenetic abnormalities. Most importantly, the Philadelphia chromosome and other BCR/ABL fusion genes are not detected.[2]
On both the bone marrow aspirate and the core biopsy, a hypercellular marrow with an increased myeloid:erythroid ratio of 20:1 or greater. Myelocytes and neutrophils are increased, and blasts and promyelocytes are not increased. Due to the myeloproliferative nature of the disease, an increase in megakaryocytes and erythroid precursors may be observed, but dyspoiesis in not seen in any cell lineage. Also, reticulinfibrosis is rare.[3][4] There is a reported association between CNL and multiple myeloma, so the bone marrow biopsy may show evidence of a plasma cell dyscrasia with increased numbers of atypical plasma cells.[2]
No distinct immunophenotype abnormality for CNL has been described.[2] See OHSU 2013 findings of gene CSF3R, mutation p. T6181.
Epidemiologyedit
This is a rare disease, with less than 100 cases reported. Of these cases, an equal male:female ratio was observed,[3] with cases typically seen in older adults.[4]
Referencesedit
^. rarediseases.info.nih.gov. Archived from the original on 17 November 2019. Retrieved 17 November 2019.
^ abcdeElaine Sarkin Jaffe; Nancy Lee Harris; Harald Stein; J.W. Vardiman, eds. (2001). Pathology & Genetics: Tumours of haematopoietic and lymphoid tissues. World Health Organization Classification of Tumors. Lyon: IARC Press. ISBN92-832-2411-6.
^ abcdefgYou W, Weisbrot IM (August 1979). "Chronic neutrophilic leukemia. Report of two cases and review of the literature". American Journal of Clinical Pathology. 72 (2): 233–42. doi:10.1093/ajcp/72.2.233. PMID 289288.
^ abcdefgZittoun R, Réa D, Ngoc LH, Ramond S (February 1994). "Chronic neutrophilic leukemia. A study of four cases". Ann. Hematol. 68 (2): 55–60. doi:10.1007/BF01715131. PMID 8148416. S2CID 19097693.
^Standen GR, Steers FJ, Jones L (April 1993). "Clonality of chronic neutrophilic leukaemia associated with myeloma: analysis using the X-linked probe M27 beta". J. Clin. Pathol. 46 (4): 297–8. doi:10.1136/jcp.46.4.297. PMC501206. PMID 8098719.
^Yanagisawa K, Ohminami H, Sato M, et al. (March 1998). "Neoplastic involvement of granulocytic lineage, not granulocytic-monocytic, monocytic, or erythrocytic lineage, in a patient with chronic neutrophilic leukemia". Am. J. Hematol. 57 (3): 221–4. doi:10.1002/(SICI)1096-8652(199803)57:3<221::AID-AJH7>3.0.CO;2-X. PMID 9495373.
External linksedit
December 15, 2023
chronic, neutrophilic, leukemia, this, article, lead, section, short, adequately, summarize, points, please, consider, expanding, lead, provide, accessible, overview, important, aspects, article, december, 2015, rare, myeloproliferative, neoplasm, that, featur. This article s lead section may be too short to adequately summarize the key points Please consider expanding the lead to provide an accessible overview of all important aspects of the article December 2015 Chronic neutrophilic leukemia CNL is a rare myeloproliferative neoplasm that features a persistent neutrophilia in peripheral blood myeloid hyperplasia in bone marrow hepatosplenomegaly and the absence of the Philadelphia chromosome or a BCR ABL fusion gene 2 Chronic neutrophilic leukemiaOther namesCNL 1 SpecialtyHematology and oncology Contents 1 Signs and symptoms 2 Cause 3 Genetics 4 Diagnosis 4 1 Laboratory findings 4 2 Sites of involvement 4 2 1 Bone marrow biopsy 4 2 2 Spleen 4 2 3 Liver 4 3 Immunophenotype 5 Epidemiology 6 References 7 External linksSigns and symptoms editThe most common clinical finding is hepatosplenomegaly Pruritus gout and mucocutaneous bleeding are occasionally seen 3 4 Cause editThe cause of CNL is currently unknown An association between CNL and multiple myeloma has been suggested based on the observation of myeloma in 20 of CNL cases 5 However a clonal genetic abnormality has not been detected in these myeloma associated cases of CNL raising the possibility that the neutrophilia is a reaction due to the neoplastic myeloma cells 2 The postulated cell of origin is a limited potential marrow derived stem cell 6 Genetics editThe majority 90 of cases have not had detectable cytogenetic abnormalities Most importantly the Philadelphia chromosome and other BCR ABL fusion genes are not detected 2 Diagnosis editLaboratory findings edit Peripheral blood neutrophilia gt 25 x 109 L with myeloid precursors promyelocytes myelocytes metamyelocytes comprising less than 5 of leukocytes 3 4 Sites of involvement edit Peripheral blood bone marrow spleen and liver are most common but any organ or tissue can be infiltrated by neutrophils 3 4 Bone marrow biopsy edit On both the bone marrow aspirate and the core biopsy a hypercellular marrow with an increased myeloid erythroid ratio of 20 1 or greater Myelocytes and neutrophils are increased and blasts and promyelocytes are not increased Due to the myeloproliferative nature of the disease an increase in megakaryocytes and erythroid precursors may be observed but dyspoiesis in not seen in any cell lineage Also reticulin fibrosis is rare 3 4 There is a reported association between CNL and multiple myeloma so the bone marrow biopsy may show evidence of a plasma cell dyscrasia with increased numbers of atypical plasma cells 2 Spleen edit Splenic infiltrates are typically found only in the red pulp 3 4 Liver edit Hepatic infiltrates can be found in either the sinusoids portal triad regions or both 3 4 Immunophenotype edit No distinct immunophenotype abnormality for CNL has been described 2 See OHSU 2013 findings of gene CSF3R mutation p T6181 Epidemiology editThis is a rare disease with less than 100 cases reported Of these cases an equal male female ratio was observed 3 with cases typically seen in older adults 4 References edit Chronic neutrophilic leukemia Genetic and Rare Diseases Information Center GARD an NCATS Program rarediseases info nih gov Archived from the original on 17 November 2019 Retrieved 17 November 2019 a b c d e Elaine Sarkin Jaffe Nancy Lee Harris Harald Stein J W Vardiman eds 2001 Pathology amp Genetics Tumours of haematopoietic and lymphoid tissues World Health Organization Classification of Tumors Lyon IARC Press ISBN 92 832 2411 6 a b c d e f g You W Weisbrot IM August 1979 Chronic neutrophilic leukemia Report of two cases and review of the literature American Journal of Clinical Pathology 72 2 233 42 doi 10 1093 ajcp 72 2 233 PMID 289288 a b c d e f g Zittoun R Rea D Ngoc LH Ramond S February 1994 Chronic neutrophilic leukemia A study of four cases Ann Hematol 68 2 55 60 doi 10 1007 BF01715131 PMID 8148416 S2CID 19097693 Standen GR Steers FJ Jones L April 1993 Clonality of chronic neutrophilic leukaemia associated with myeloma analysis using the X linked probe M27 beta J Clin Pathol 46 4 297 8 doi 10 1136 jcp 46 4 297 PMC 501206 PMID 8098719 Yanagisawa K Ohminami H Sato M et al March 1998 Neoplastic involvement of granulocytic lineage not granulocytic monocytic monocytic or erythrocytic lineage in a patient with chronic neutrophilic leukemia Am J Hematol 57 3 221 4 doi 10 1002 SICI 1096 8652 199803 57 3 lt 221 AID AJH7 gt 3 0 CO 2 X PMID 9495373 External links edit Retrieved from https en wikipedia org w index php title Chronic neutrophilic leukemia amp oldid 1186820540, wikipedia, wiki, book, books, library,
