Name

Chronic myeloid leukemia (CML), NOS

ICD-O-3 Morphology

Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421

Abstractor Notes

Chronic myeloid leukemia, NOS is not included in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B23)

Chronic myeloid leukemia, NOS is a generic disease description. DCO cases or path report only cases may stay in this classification. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease. Further review of the medical record should be done to look for the tests listed as definitive diagnosis.

The more specific chronic myeloid leukemias are
1. Chronic myelogenous leukemia, BCR-ABL1 positive (9875/3)
2. Atypical chronic myeloid leukemia, BCR-ABL1 negative (9876/3)
3. Chronic myelomonocytic leukemia (9945/3)
4. Juvenile myelomonocytic leukemia (9946/3)

If the characteristics of a specific subtype of chronic myeloid leukemia develop later in the course of the disease, change the histology code to the more specific diagnosis.

Treatment:

Chronic phase: Tyrosine kinase inhibitor. High-dose chemotherapy with donor stem cell transplant; BRM (interferon). OR Chemotherapy; splenectomy; clinical trial of lower-dose chemotherapy with donor stem cell transplant.

Accelerated phase: Donor stem cell transplant;; tyrosine kinase inhibitor; BRM, (interferon (with or without chemotherapy). Chemotherapy may be a single or multi-drug regimen.

Blast phase: Tyrosine kinase inhibitor; chemotherapy (single or multi-drug); bi=BRM (interferon); Donor stem cell transplant

Diagnostic Confirmation

This is a histology for which the Definitive Diagnostic Method does not include Genetics Data or Immunophenotyping, thus Diagnostic Confirmation should never be 3. If genetics and/or immunophenotyping are available, re-review to see if a more specific neoplasm can be coded.

Module Rule

None

Alternate Names

Chronic granulocytic leukemia, NOS
Chronic myelocytic leukemia, NOS
Chronic myelogenous leukemia, NOS

Definition

No delineation of translocation, BCR-ABL1, or Phil chromosome noted to qualify for ICD-O-3 9863.

Specific term as defined by WHO, codes as 9875/3 and indicates the process has Ph+, BCR-ABL1 fusion and/or t(9;22)(q34;q11) demonstrated, as defined by ICD-O-3 for 9875 as well. Presumably myelogenous leukemia without genetic studies done would be coded to 9863.

Definitive Diagnostic Methods

Clinical diagnosis
Histologic confirmation

Genetics Data

None

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

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

C92.1 Chronic myeloid leukemia
C95.1 Chronic leukemia of unspecified cell type

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

C95.1 Chronic leukemia of unspecified cell type (effective October 01, 2015 - September 30, 2024)
C95.10 Chronic leukemia of unspecified cell type not having achieved remission (effective October 01, 2024)
C95.11 Chronic leukemia of unspecified cell type, in remission (effective October 01, 2024)
C95.12 Chronic leukemia of unspecified cell type, in relapse (effective October 01, 2024)

Signs and Symptoms

Bone or joint pain
Easy bruising or bleeding
Eczema-like skin rash
Fatigue
Fever
Night sweats
Pain or feeling of fullness below the ribs
Painless lumps
Petechiae
Shortness of breath
Weakness

Diagnostic Exams

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

Progression and Transformation

None

Epidemiology and Mortality

None

Sources

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 Chronic Myeloid Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <12/13/2024>. Available at: https://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389354]
Section: Chronic Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq
Glossary