Search Database ICD-O-3 Code Lists

Name

Acute leukemia of ambiguous lineage with other defined genetic alterations

ICD-O-3 Morphology

9805/3: Acute biphenotypic leukemia
Effective 2001 and later

Reportable

2001-2009, 2026 and later; see Abstractor Notes

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

(This code is effective for cases diagnosed 2026 and later. For cases diagnosed prior to 2026 see codes: 9801/3, 9808/3, 9809/3.)

Acute leukemia of ambiguous lineage with other defined genetic alterations is part of the Acute leukemias of mixed or ambiguous lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B9)

Bone marrow and peripheral blood are always involved.

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

Acute bilineal leukemia
Acute mixed lineage leukemia
Acute biphenotypic leukemia
Acute leukemia of ambiguous lineage, NOS (see 9801/3 prior to 2026)
Acute leukemia of ambiguous lineage with BCL11B rearrangement
Mixed-phenotype acute leukemia B/T (MPAL-BT)
Mixed-phenotype acute leukemia B/T/Myeloid (MPAL-B/T/M)
Mixed-phenotype acute leukemia (MPAL), T/megakaryocytic (MPAL-T/Mk)
Mixed-phenotype acute leukemia (MPAL) with ZNF384 rearrangement

Definition

Acute leukemia of ambiguous lineage (ALAL) with other defined genetic alterations includes acute leukemias with mixed-lineage or ambiguous immunophenotypes harbouring newly recognized defined genetic alterations. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

BCL11B rearrangement.
ZNF384 rearrangement

Immunophenotyping

CD10+ (expression/positive)
CD19+ (expression/positive)
CD22+ (expression/positive)
CD34+ (expression/positive)
cCD79+ (expression/positive)
HLA-DR+ (expression/positive)
KIT (CD117)+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

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)

C95.0 Acute leukemia of unspecified cell type

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

C95.0 Acute leukemia of unspecified cell type (effective October 01, 2015 - September 20, 2024)
C95.00 Acute leukemia of unspecified cell type not having achieved remission (effective October 01, 2024)
C95.01 Acute leukemia of unspecified cell type, in remission (effective October 01, 2024)
C95.02 Acute 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 lesions
Fatigue
Fever
High white blood cell counts
Night sweats
Pain or feeling of fullness below the ribs
Painless lumps
Petechiae
Shortness of breath
Weakness

Diagnostic Exams

Bone marrow aspiration and biopsy
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

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 leukemias of mixed or ambiguous lineage
Pages: Part A: 210-211

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