An official website of the United States government
Home
Effective
2001 and later
Reportable
for cases diagnosed
1978 and later
Primary Site(s)
See Module 4: Rules PH7, PH8 and Module 7 (for primary site for lymphoma presentation)
Usually presents as a leukemia (primary site C421). Lymphomatous presentation is rare.
See abstractor notes.
See abstractor notes.
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 codes: 9728/3 [lymphoma] or 9836/3 [leukemia].)
B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with TCF3::PBX1 is part of the Precursor B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B14)
All subtypes of ALL are coded as the same primary. If you have an ALL diagnosed, then a subsequent ALL diagnosed at a later time, this is the same primary.
If there are multiple diagnoses for ALL
- 9812/3-9819/3 take priority over 9811/3
- 9812/3 takes priority over 9813/3-9819/3
- Remaining combinations: Use the first diagnosis
B-ALL always involves the bone marrow and usually also involves the peripheral blood. Extramedullary involvement is common and is by definition the primary site of involvement in B-LBL, with frequent sites being the CNS, testes, lymph nodes, liver, spleen, skin, and soft tissues
Immunophenotyping will confirm that the patient has a B-lymphoblastic B-ALL; the genetic testing provides the information needed to classify this neoplasm as a B-lymphoblastic leukemia/lymphoma with TCF3::PBX1.
B-lymphoblastic leukemia/lymphoma [B-ALL/LBL] with TCF3::PBX1 is part of the Precursor B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B14)
All subtypes of ALL are coded as the same primary. If you have an ALL diagnosed, then a subsequent ALL diagnosed at a later time, this is the same primary.
If there are multiple diagnoses for ALL
- 9812/3-9819/3 take priority over 9811/3
- 9812/3 takes priority over 9813/3-9819/3
- Remaining combinations: Use the first diagnosis
B-ALL always involves the bone marrow and usually also involves the peripheral blood. Extramedullary involvement is common and is by definition the primary site of involvement in B-LBL, with frequent sites being the CNS, testes, lymph nodes, liver, spleen, skin, and soft tissues
Immunophenotyping will confirm that the patient has a B-lymphoblastic B-ALL; the genetic testing provides the information needed to classify this neoplasm as a B-lymphoblastic leukemia/lymphoma with TCF3::PBX1.
Diagnostic Confirmation
This histology can only be determined by positive genetics, diagnostic confirmation will always be 3.
Module Rule
Module 4: PH7, PH8
Alternate Names
Definition
B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) with TCF3::PBX1 fusion is a neoplasm of lymphoblasts of B-cell lineage defined by the presence of rearrangement between TCF3 on chromosome 19 and PBX1 on chromosome 1. (WHO 5th edition)
Definitive Diagnostic Methods
Cytogenetics
Genetic testing
Genetics Data
TCF3::PBX1 rearrangement
t(1;19)(q23;p13.3) (E2A-PBX1)
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)
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 [LBL] (effective October 01, 2015)
C83.5A Lymphoblastic (diffuse) lymphoma [LBL] in remission (effective October 01, 2024)
C91.0 Acute lymphoblastic leukemia [ALL] (effective October 01, 2015 - September 30, 2024)
C91.00 Acute lymphoblastic leukemia [ALL] not having achieved remission (effective October 01, 2024)
C91.01 Acute lymphoblastic leukemia [ALL], in remission (effective October 01, 2024)
C91.02 Acute lymphoblastic leukemia [ALL], in relapse (effective October 01, 2024)
Signs and Symptoms
Anemia
Arthralgias
Bone pain
Hepatomegaly
Lymphoadenopathy
Neutropenia
Splenomegaly
Thrombocytopenia
Diagnostic Exams
Blood chemistry studies
Bone marrow aspiration and biopsy
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
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: B-lymphoblastic leukemia/lymphoma
Pages: Part B: 354-355, 358-359
Section: B-lymphoblastic leukemia/lymphoma
Pages: Part B: 354-355, 358-359
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
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
PDQ® Adult Treatment Editorial Board. PDQ Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <02/13/2025>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-all-treatment-pdq. Accessed <02/28/2025>. [PMID: 26389171]
Section: Acute Lymphoblastic Leukemia Treatment (PDQ®)–Health Professional Version
Pages: Acute Lymphoblastic Leukemia Treatment (PDQ®) - NCI
Section: Acute Lymphoblastic Leukemia Treatment (PDQ®)–Health Professional Version
Pages: Acute Lymphoblastic Leukemia Treatment (PDQ®) - NCI
