Search Database ICD-O-3 Code Lists

Name

Mixed-phenotype acute leukemia (MPAL), T/myeloid

ICD-O-3 Morphology

9809/3: Mixed phenotype acute leukemia, T/myeloid, NOS
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

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9801/3.)

Mixed-phenotype acute leukemia (MPAL), T/myeloid 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. Lymph node involvement is common in MPAL with T-lineage differentiation

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

T/myeloid mixed-phenotype acute leukemia (MPAL-T/M) is an acute leukemia that expresses T-lineage and myeloid-lineage markers but does not meet criteria for mixed-phenotype acute leukemia (MPAL) with defined genetic alterations. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

BCL11A fusion
ETV6 fusion
PICALM::MLLT10 rearrangement
NUP214 fusion

Immunophenotyping

CD7+ (expression/positive)

Treatments

Chemotherapy

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 30, 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 rash
Fatigue
Fever
Night sweats
Pain or feeling of fullness below the ribs
Painless lumps
Petechiae
Shortness of breath
Weakness

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Molecular testing
Peripheral blood smear
Physical exam and history

Progression and Transformation

None

Epidemiology and Mortality

Age: more common in adults (can occur in children)
Incidence: <1% of all Leukemia cases
Survival: 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: Myeloid/lymphoid neoplasms with eosinophils and tyrosine kinase gene fusions
Pages: Part A: 214-215

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
Glossary