Name

Acute basophilic leukemia

ICD-O-1 Morphology

Effective 1978 - 1991

ICD-O-2 Morphology

Effective 1992 - 2000

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

Cutaneous involvement, organomegaly, lytic lesion, and symptoms relating to hyperhistaminemia may be present. Basophilic leukemia is differentiated from other AML subtypes with basophilia using basophilia immunological markers, light microscopic cytochemistry for myeloperoxidase and electron microscopy.

If the leukemia occurs before or simultaneously with Myeloid Sarcoma (9930/3), see M3 and Module 5: PH10

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

Basophilic leukemia

Definition

Acute basophilic leukemia is an acute myeloid leukemia (AML) in which the primary differentiation is to basophils.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear

Genetics Data

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

Immunophenotyping

CD11b positive
CD13 expression
CD22 positive
CD33 expression
CD34 expression
CD123 positive
CD203c positive
HLA-DR positive
KIT (CD117) negative
TdT+ positive

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

None

Corresponding ICD-9 Codes

205.0 Acute myeloid leukemia

Corresponding ICD-10 Codes

C92.0 Acute myeloid leukemia

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

C94.8 Other specified leukemias (effective October 01, 2015)

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

CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Physical exam and history
Reverse transcription-polymerase chain reaction test (RT-PCR)

Progression and Transformation

None

Epidemiology and Mortality

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

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: Acute myeloid leukemia and related precursor neoplasms
Pages: 164-165

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 Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary