Name

Acute basophilic leukemia

ICD-O-3 Morphology

9870/3: Acute basophilic leukemia
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Abstractor Notes

Acute basophilic leukemia is part of the Acute myeloid leukemia (AML) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B6)

Bone marrow and peripheral blood are always involved. Cutaneous involvement, hepatosplenomegaly, and lytic bone lesions may also be present.

If this leukemia and myeloid sarcoma (9930/3) occur during the same clinical workup, this is one primary, the leukemia.

If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.

See Multiple Primary Rule M3

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

None

Definition

Acute basophilic leukemia is an acute myeloid leukemia (AML) with basophilic differentiation that lacks defining genetic abnormalities. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

t(X;6)(p11.2;q23.3) resulting in MYB-GATA1

Immunophenotyping

CD11b+ (expression/positive)
CD13+ (expression/positive)
CD33+ (expression/positive)
CD34+ (expression/positive)
CD38+ (expression/positive)
CD123+ (expression/positive)
CD203c+ (expression/positive)
HLA-DR+ (expression/positive)
KIT (CD117)- (no expression/negative)
TdT+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

None

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

C92.0 Acute myeloid leukemia

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

C94.8 Other specified leukemias (effective October 01, 2015 - September 30, 2024)
C94.80 Other specified leukemias not having achieved remission (effective October 01, 2024)
C94.81 Other specified leukemias, in remission (effective October 01, 2024)
C94.82 Other specified leukemias, in relapse (effective October 01, 2024)

Signs and Symptoms

Bone marrow failure
Cutaneous involvement
Easy bruising or bleeding
Fatigue
Fever
Hyperhistaminemia
Lytic lesions
Organomegaly
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite

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

Incidence: <1% of all AML cases
Survival: limited information, known cases had poor prognosis

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: Acute myeloid leukemia
Pages: Part A: 153-154

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 Acute Myeloid Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <03/06/2024>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389432]
Section: Acute Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary