Name

B-lymphoblastic leukemia/lymphoma, BCR-ABL1-like

ICD-O-3 Morphology

9819/3: B-lymphocytic leukemia/lymphoma, BCR-ABL1-like
Effective 2021 and later

Reportable

for cases diagnosed 2021 and later

Primary Site(s)

No primary site specified

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021, see code: 9811/3)

The presenting clinical features are generally similar to those seen inpatients with other ALLs, although patients tend to have high white blood cell counts at presentation.

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

None

Definition

B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) is a neoplasm of lymphoblasts committed to the B-cell lineage that lack the BCR-ABL1 translocation but show a pattern of gene expression very similar to that seen in ALL with BCR-ABL1.

Definitive Diagnostic Methods

Bone marrow biopsy
Genetic testing
Immunophenotyping

Genetics Data

BCR-ABL1-like
CRLF2 rearrangements

Immunophenotyping

CD10+ (expression/positive)
CD19+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

200.1 Lymphosarcoma (Lymphoma presentation)
204.0 Acute lymphoid leukemia (Leukemia presentation)

Corresponding ICD-10 Codes

C83.5 Non-Hodgkin lymphoma lymphoblastic (diffuse) (Lymphoma presentation)
C91.0 Acute lymphoblastic leukemia (Leukemia presentation)

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

C83.5 Lymphoblastic (diffuse) lymphoma (Lymphoma presentation) (effective October 01, 2015)
C91.0 Acute lymphoblastic leukemia [ALL] (Leukemia presentation) (effective October 01, 2015)

Signs and Symptoms

Anemias
Arthralgias
Bone pain
Elevated white blood cell (WBC) count
Hepatomegaly
Lymphadenopathy
Neutropenia
Splenomegaly
Thrombocytopenia

Diagnostic Exams

Blood chemistry studies
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Age: all ages, lowest in children
Incidence: occurs in 10-25% of patients with ALL
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: Precursor lymphoid neoplasms
Pages: 208

National Cancer Institute
Section: General Information About Acute Lymphoid Leukemia
Pages: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq

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