Search Database ICD-O-3 Code Lists

Name

Mixed-phenotype acute leukemia (MPAL) with KMT2A-rearranged

ICD-O-3 Morphology

9807/3: Mixed phenotype acute leukemia with t(v;11q23); MLL rearranged
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) with KMT2A-rearranged 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. Some patients present with myeloid sarcoma.

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 only be determined by positive genetics, diagnostic confirmation will always be 3.

Module Rule

None

Alternate Names

Mixed phenotype acute leukemia (MPAL) with t(v;11q23); MLL rearranged

Definition

Mixed-phenotype acute leukemia (MPAL) with KMT2A rearrangement is an acute leukemia that fulfils the criteria for MPAL and in which the KMT2A locus on chromosome 11q23.3 is fused with any of a number of partner genes. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

AFF1 (AF4) on chromosome 4 band q21
KMT2A gene rearrangement
MLLT1 (ENL) at 19p13
MLLT3 (AF9) at 9p22

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

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 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
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
Flow cytometry
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 children, especially infancy
Incidence: rare
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: Acute leukemias of mixed or ambiguous lineage
Pages: Part A: 208-209

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