Chronic neutrophilic leukemia

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Chronic neutrophilic leukemia - Wikipedia

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Chronic neutrophilic leukemia
Other names	CNL^[1]
Specialty	Hematology and oncology

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]

Signs and symptoms

The most common clinical finding is hepatosplenomegaly. Pruritus, gout, and mucocutaneous bleeding are occasionally seen.^[3]^[4]

Cause

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]

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.^[2]

Diagnosis

Laboratory findings

Peripheral blood neutrophilia (> 25 x 10⁹/L) with myeloid precursors (promyelocytes, myelocytes, metamyelocytes) comprising less than 5% of leukocytes.^[3]^[4]

Sites of involvement

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

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

Splenic infiltrates are typically found only in the red pulp.^[3]^[4]

Liver

Hepatic infiltrates can be found in either the sinusoids, portal triad regions, or both.^[3]^[4]

Immunophenotype

No distinct immunophenotype abnormality for CNL has been described.^[2] See OHSU 2013 findings of gene CSF3R, mutation p. T6181.

Epidemiology

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]

References

^ "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 & 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, 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. 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<221::AID-AJH7>3.0.CO;2-X. PMID 9495373.

External links

CFU-GM/
and other granulocytes

CFU-GM

Myelocyte

AML	Acute myeloblastic leukemia M0 M1 M2 APL/M3
MP	Chronic neutrophilic leukemia

Monocyte

AML	AMoL/M5 Myeloid dendritic cell leukemia
CML	Philadelphia chromosome Accelerated phase chronic myelogenous leukemia

Myelomonocyte

AML	M4
MD-MP	Juvenile myelomonocytic leukemia Chronic myelomonocytic leukemia

Other

Histiocytosis

CFU-Baso

AML	Acute basophilic

CFU-Eos

AML	Acute eosinophilic
MP	Chronic eosinophilic leukemia/Hypereosinophilic syndrome

MEP

CFU-Meg

MP	Essential thrombocythemia Acute megakaryoblastic leukemia

CFU-E

AML	Erythroleukemia/M6
MP	Polycythemia vera
MD	Refractory anemia Refractory anemia with excess of blasts Chromosome 5q deletion syndrome Sideroblastic anemia Paroxysmal nocturnal hemoglobinuria Refractory cytopenia with multilineage dysplasia

CFU-Mast

Mastocytoma	Mast cell leukemia Mast cell sarcoma Systemic mastocytosis
Mastocytosis	Diffuse cutaneous mastocytosis Erythrodermic mastocytosis Adult type of generalized eruption of cutaneous mastocytosis Urticaria pigmentosa Mast cell sarcoma Solitary mastocytoma
Systemic mastocytosis	Xanthelasmoidal mastocytosis

Multiple/unknown

AML	Acute panmyelosis with myelofibrosis Myeloid sarcoma
MP	Primary myelofibrosis Biphenotypic acute leukaemia

Categories:

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Classification	D ICD-10: D47.1 ICD-9-CM: 205.1 ICD-O: M9963/3 MeSH: D015467
External resources	Orphanet: 86829