Search Database ICD-O-3 Code Lists

Name

Mixed-phenotype acute leukemia with t(v;11q23.3); KMT2A-rearranged

ICD-O-3 Morphology

9807/3: Mixed phenotype acute leukemia with t(v;11q23); MLL rearranged
Effective 2010 and later

Reportable

for cases diagnosed 2010 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.)

This is a rare leukemia that is more common in children than in adults. Patients with B/myeloid leukemia with MLL translocations are often treated differently from patients diagnosed with ALL with MLL translocations, but there is no evidence that the difference in treatment is necessary or helpful.

Diagnostic Confirmation

This histology can only be determined by positive genetics and/or immunophenotyping, diagnostic confirmation will always be 3.

Grade

Not Applicable

Module Rule

None

Alternate Names

Mixed phenotype acute leukemia with t(v;11q23); MLL rearranged
Mixed-phenotype acute leukemia with MLL rearrangement
MLL
MPAL with t(v;11q23.3); KMT2A rearranged

Definition

Mixed-phenotype acute leukemia (MPAL) with t(v;11q23.3) fulfills the criteria for MPAL and has blasts with a translocation involving KMT2A (previously called MLL).

Definitive Diagnostic Methods

Bone marrow biopsy
FISH
Genetic testing
Immunophenotyping
Karyotyping
Polymerase chain reaction (PCR)

Genetics Data

AFF1 (AF4) on chromosome 4 band q21
KMT2A rearrangement

Immunophenotyping

CD10- (no expression/negative)
CD15+ (expression/positive)
CD19+ (expression/positive)
CD22+ (expression/positive)
CD79a+ (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-9 Codes

208.0 Acute leukemia of unspecified cell type

Corresponding ICD-10 Codes

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)

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
Chest x-ray
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Lymph node biopsy
Molecular testing
Peripheral blood smear

Progression and Transformation

None

Epidemiology and Mortality

Age: more common in children, especially infancy
Incidence: rare
Survival: 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 leukemias of ambiguous terminology
Pages: 183-184

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
Glossary