Search Database ICD-O-3 Code Lists

Name

Myelodysplastic/myeloproliferative neoplasm with neutrophilia (MDS/MPN-N)

ICD-O-3 Morphology

9876/3: Atypical chronic myeloid leukemia, BCR-ABL1 negative
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Myelodysplastic/myeloproliferative neoplasm with neutrophilia (MDS/MPN-N) is part of the Myelodysplastic/myeloproliferative (MDS/MPN) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B5)

MDS/MPN-N is uncommon.

Peripheral blood and bone marrow are always involved; splenic and hepatic involvement is common.

The diagnosis of MDS/MPN-N requires exclusion of the BCR::ABL1 fusion.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Atypical chronic myeloid (aCML) leukemia, BCR::ABL1-negative
Atypical chronic myeloid leukemia, Philadelphia chromosome (Ph1) negative

Definition

Myelodysplastic/myeloproliferative neoplasm with neutrophilia (MDS/MPN-N), formerly known as atypical chronic myeloid leukemia, is a myeloid neoplasm with myelodysplastic and myeloproliferative features characterized by sustained peripheral blood neutrophilia and neutrophilic left shift. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation

Genetics Data

Chromosomal separation regulators (STAG2)
Enzymes (ETNK1)
Epigenetic regulators (SETBPI, EZH2)
Mutations associated with clonal hematopoiesis (ASXL1, TET2, DNMT3A)
Proliferative signaling pathways (CBL, JAK2, NF1, NRAS)
Splicing machinery (SRSF2, U2AF1, SF3B1, ZRSR2)
Transcription factors (CUX1, GATA2, RUNX1)

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C92.1 Chronic myeloid leukemia

Corresponding ICD-10-CM Codes (U.S. only)

C92.2 Atypical chronic myeloid leukemia, BCR/ABL-negative (effective October 01, 2015 - September 30, 2024)
C92.20 Atypical chronic myeloid leukemia, BCR/ABL-negative not having achieved remission (effective October 01, 2024)
C92.21 Atypical chronic myeloid leukemia, BCR/ABL-negative, in remission (effective October 01, 2024)
C92.22 Atypical chronic myeloid leukemia, BCR/ABL-negative, in relapse (effective October 01, 2024)

Signs and Symptoms

Anemia
Easy bruising or bleeding
Fatigue
Fever
Frequent infections
Pain or a feeling of fullness below the ribs
Pale skin
Petechiae
Shortness of breath
Splenomegaly
Thrombocytopenia
Weakness

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) Scan
Cytogenetic analysis
Flow cytometry
Immunophenotyping
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

15-40% of patients evolve to acute myeloid leukemia

Epidemiology and Mortality

Age: 70-80 years median age
Incidence: 1-2 cases for every 100 cases of BCR-ABL1 positive (9875/3)
Sex: no male or female predominance
Survival: 14-29 months median survival time

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Myelodysplastic/myeloproliferative neoplasms
Pages: Part A: 102-104

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Myelodysplastic/Myeloproliferative Neoplasms Treatment. Bethesda, MD: National Cancer Institute. Updated <06/14/2024>. Available at: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389321]
Section: Myelodysplastic/Myeloproliferative Neoplasms Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq#section/_1
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