Name

Mixed-phenotype acute leukemia with t(9;22)(q34.1;q11.2); BCR-ABL1

ICD-O-3 Morphology

9806/3: Mixed phenotype acute leukemia with t(9;22)(q34;q11.2);BCR-ABL1
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Abstractor Notes

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

Patients present with features similar to those of other patients with acute leukemia. Mixed phenotype acute leukemia (MPAL) diagnosis should not be made in patients known to have had CML.

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(9;22)(q34;q11.2); BCR-ABL1
MPAL

Definition

Mixed-phenotype acute leukemia (MPAL) with t(9;22)(q34.1;q11.2) fulfills the criteria for MPAL and has blasts with t(9;22) and/or BCR-ABL1 rearrangement. Some patients with chronic myeloid leukemia (CML) develop or even present with a mixed blast phase that would fulfill the criteria for MPAL; however, this diagnosis should not be made in patients known to have had CML.

Definitive Diagnostic Methods

FISH
Genetic testing
Karyotyping
Polymerase chain reaction (PCR)

Genetics Data

BCR-ABL1
p210 transcript
t(9;22)

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures

Transformations to

There are no known transformations

Transformations from

There are no known transformations

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 lesions
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 adults
Incidence: <1% of acute leukemias
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: 182-183

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