Search Database ICD-O-3 Code Lists

Name

Atypical chronic myeloid leukemia, BCR-ABL1-negative

ICD-O-1 Morphology

9863/3: Chronic myeloid leukemia, NOS
Effective 1978 - 1991

ICD-O-2 Morphology

9863/3: Chronic myeloid leukemia, NOS
Effective 1992 - 2000

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

Atypical CML BCR-ABL1 negative is a rare disease. The peripheral blood and bone marrow are always involved; spleen and hepatic involvement are common.

The physician will run tests to rule out BCR-ABL1 involvement.

For more information, see the NCI website: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq#section/_50

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.

Grade

Not Applicable

Module Rule

None

Alternate Names

aCML
Atypical chronic myeloid leukemia
Atypical chronic myeloid leukemia, BCR/ABL1-negative
Atypical chronic myeloid leukemia, Philadelphia chromosome (Ph1) negative
Philadelphia chromosome (PH1) negative

Definition

aCML is a leukemic disorder with myelodysplastic as well as myeloproliferative features. It is characterized by principal involvement of the neutrophil series with leukocytosis comprised of immature and mature neutrophils that are dysplastic. Mutilineage dysplasia; however, is common and reflects the stem cell origin.

Definitive Diagnostic Methods

Bone marrow biopsy
Peripheral blood smear

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

Same Primaries

Corresponding ICD-9 Codes

205.1 Chronic myeloid leukemia

Corresponding ICD-10 Codes

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)

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

Blood chemistry studies
Bone marrow aspiration and biopsy
Cytogenetic analysis
Immunocytochemistry
Peripheral blood smear

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

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myelodysplastic/myeloproliferative neoplasms
Pages: 87-89

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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

National Cancer Institute
Section: General Information About Myelodysplastic/ Myeloproliferative Neoplasms (MDS/MPN)
Pages: https://www.cancer.gov/types/myeloproliferative/hp/mds-mpd-treatment-pdq#section/_1
Glossary