Bone marrow neoplastic
Bone marrow - neoplastic myeloid
Myeloproliferative neoplasms (MPN)
Chronic neutrophilic leukemia
Author: Nikhil Sangle, M.D.
Topic Completed: 1 August 2011
Minor changes: 13 January 2021
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: Chronic neutrophilic leukemia [title]
Table of Contents
Definition / general | Diagnosis | Laboratory | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Molecular / cytogenetics description | Differential diagnosisCite this page: Sangle N. Chronic neutrophilic leukemia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/myeloproliferativeCNL.html. Accessed March 7th, 2021.
Definition / general
- 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
Diagnosis
WHO 2008:
- ≥ 25 × 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)
Laboratory
- Increased WBC count > 25 × 109/L with neutrophilia, increased serum vitamin B12
Case reports
- 72 year old man with transformation to AML (Jpn J Clin Oncol 2000;30:362)
- 74 year old man with Sweet’s syndrome (Singapore Med J 2007;48:e74)
Treatment
- Optimal treatment unknown
Microscopic (histologic) description
- Bone marrow: hypercellular with granulocyte hyperplasia, predominantly segmented and band neutrophils; variable erythroid and megakaryocyte hyperplasia; no / minimal dysplastic changes; no increased myeloblasts
Microscopic (histologic) images
Images hosted on other servers:
Bone marrow smear:
Neutrophils at various maturational states
Molecular / cytogenetics description
Molecular:
Cytogenetics
- Monoclonal (J Clin Pathol 2003;56:292)
- Occasionally has JAK2 V617F mutation (Blood 2005;106:1207), no BCR-ABL
Cytogenetics
- 90% normal, also +8, 20q-
Differential diagnosis
- 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)
