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Chronic Myeloid Neoplasms

Myeloproliferative neoplasms (MPN)

Chronic neutrophilic leukemia

Reviewer: Nikhil Sangle, M.D., University of Utah & ARUP Laboratories (see Reviewers page)
Revised: 4 August 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Rare
● Associated with splenomegaly (Orv Hetil 2006;147:827)
● Usually chronic course with poor prognosis, often death from cerebral hemorrhage (J Clin Pathol 2002;55:862)

Bone marrow:
● Hypercellular with granulocyte hyperplasia, predominantly segmented and band neutrophils
● Variable erythroid and megakaryocyte hyperplasia; no/minimal dysplastic changes
● No increased myeloblasts

Laboratory
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● Increased WBC count > 25 x 10⁹/L with neutrophilia, increased serum vitamin B12

Diagnosis
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WHO 2008:
● ≥25 x 10⁹ WBC in peripheral blood, with >80% segmented neutrophils or bands, <10% immature granulocytes and <1% myeloblasts (<5% blasts in the bone marrow)
● No cause for reactive neutrophilia
● Demonstration of clonal status of the granulocytes
● Hepatosplenomegaly
● No rearrangement of PDGFRA, PDGFRB or FGFR1
● No BCR-ABL
● No dyserythropoiesis, no granulocyte dysplasia, no monocytosis
● No other myeloproliferative disorder (Ann Hematol 2008;87:1)

Case reports
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● 74 year old man with Sweet’s syndrome (Singapore Med J 2007;48:e74)
● 72 year old man with transformation to AML (Jpn J Clin Oncol 2000;30:362)

Treatment
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● Optimal treatment unknown

Micro description
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● Bone marrow: hypercellular with granulocyte hyperplasia, predominantly segmented and band neutrophils; variable erythroid and megakaryocyte hyperplasia; no/minimal dysplastic changes; no increased myeloblasts

Micro images
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Peripheral blood:

Neutrophilia without left shift

Bone marrow biopsy:

Marked granulocytic proliferation

Bone marrow smear:

Neutrophils at various maturational states

Cytogenetics description
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● 90% normal, also +8, 20q-

Molecular description
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● Monoclonal (J Clin Pathol 2003;56:292)
● Occasionally has JAK2 V617F mutation (Blood 2005;106:1207), no BCR-ABL

Differential diagnosis
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● CML with p230 variant BCR-ABL fusion transcript and mature peripheral blood neutrophils should be considered as CML (Cancer 2002;94:2416)
● Myeloma or other neoplasms that secrete G-CSF or other cytokines may be associated with chronic neutrophilic leukemia, but must demonstrate clonal granulocytes to establish the diagnosis (Am J Hematol 2005;80:243)

End of Chronic Myeloid Neoplasms > Myeloproliferative neoplasms (MPN) > Chronic neutrophilic leukemia

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