WordNet

being long-lasting and recurrent or characterized by long suffering; "chronic indigestion"; "a chronic shortage of funds"; "a chronic invalid"
of long duration; "chronic money problems" (同)continuing
habitual; "a chronic smoker" (同)inveterate
malignant neoplasm of blood-forming tissues; characterized by abnormal proliferation of leukocytes; one of the four major types of cancer (同)leukaemia, leucaemia, cancer of the blood
the chief phagocytic leukocyte; stains with either basic or acid dyes (同)neutrophile

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(病気が)長期にわたる,慢性の / 《名詞の前にのみ用いて》常習の,癖になった
白血病

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/12/15 11:04:36」(JST)

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Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative disorder 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.^[1]

Epidemiology

This is a rare disease, with less than 100 cases reported. Of these cases, an equal male:female ratio was observed,^[2] with cases typically seen in older adults.^[3]

Clinical Features

Etiology

The etiology 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.^[4] 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.^[1] The postulated cell of origin is a limited-potential, marrow-derived stem cell.^[5]

Clinical Presentation

The most common clinical finding is hepatosplenomegaly. Pruritus, gout, and mucocutaneous bleeding are occasionally seen.^[2]^[3]

Laboratory findings

Peripheral blood neutrophilia (> 25 x 10⁹/L) with myeloid precursors (promyelocytes, myelocytes, metamyelocytes) comprising less than 5% of leukocytes.^[2] ^[3]

Sites of Involvement

Peripheral blood, bone marrow, spleen, and liver are most common, but any organ or tissue can be infiltrated by neutrophils.^[2] ^[3]

Morphology

Bone Marrow Biopsy

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.^[2]^[3] 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.^[1]

Spleen

Splenic infiltrates are typically found only in the red pulp.^[2] ^[3]

Liver

Hepatic infiltrates can be found in either the sinusoids, portal triad regions, or both.^[2] ^[3]

Molecular Findings

Immunophenotype

No distinct immunophenotype abnormality for CNL has been described.^[1]

Genetics

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.^[1]

Current Articles

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References

^ ^a ^b ^c ^d ^e ^f Elaine Sarkin Jaffe, Nancy Lee Harris, World Health Organization, International Agency for Research on Cancer, Harald Stein, J.W. Vardiman (2001). Pathology and genetics of tumours of haematopoietic and lymphoid tissues. World Health Organization Classification of Tumors. 3. Lyon: IARC Press. ISBN 92-832-2411-6. http://books.google.com/?id=XSKqcy7TUZUC.
^ ^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". Am. J. Clin. Pathol. 72 (2): 233–42. PMID 289288.
^ ^a ^b ^c ^d ^e ^f ^g Zittoun 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.
^ 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. http://jcp.bmj.com/cgi/pmidlookup?view=long&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.

UpToDate Contents

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1. 慢性骨髄性白血病の臨床症状および診断 clinical manifestations and diagnosis of chronic myeloid leukemia
2. 好中球増多症の原因 causes of neutrophilia
3. 骨髄増殖性腫瘍の概要 overview of the myeloproliferative neoplasms
4. 造血腫瘍の分類 classification of the hematopoietic neoplasms
5. 血液悪性腫瘍およびリンパ悪性腫瘍の遺伝的異常 genetic abnormalities in hematologic and lymphoid malignancies

English Journal

Chronic myelomonocytic leukemia mimicking hematologic systemic lupus erythematosus.

Moura CA1, Santiago M2, Neto J3, Gomes de Souza VH4, Moura CG5.
Lupus.Lupus.2016 Jun 30. pii: 0961203316657436. [Epub ahead of print]
The relationship between primary hematologic disease and rheumatologic manifestations is well known, especially acute lymphocytic leukemia, lymphomas, plasma cell dyscrasias and myelodysplastic syndrome (MDS). Currently, more has been described about autoimmune manifestations in chronic myelomonocyt
PMID 27365372

Clinical response to ruxolitinib in CSF3R T618-mutated chronic neutrophilic leukemia.

Stahl M1, Xu ML2, Steensma DP3, Rampal R4, Much M2, Zeidan AM2.
Annals of hematology.Ann Hematol.2016 Jun;95(7):1197-200. doi: 10.1007/s00277-016-2664-4. Epub 2016 Apr 11.
PMID 27068405

"Unusual Cause Of Tophi With Renal Thrombotic Microangiopathy".

Sahu KK1, Law AD1, Kumar G2, Dhir V1, Naseem S2, Nada R3, Varma SC1, Malhotra P1.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion.Indian J Hematol Blood Transfus.2016 Jun;32(Suppl 1):100-3. doi: 10.1007/s12288-015-0539-9. Epub 2015 Apr 16.
Chronic neutrophilic leukemia (CNL) is a rare entity amongst myeloproliferative neoplasms (MPNs). The classical presentation of CNL is with splenomegaly, mature neutrophilic leucocytosis and hyperuricemia. We herein report a case who presented with symptoms of acute gouty arthritis. Physical examina
PMID 27408367