Search Database ICD-O-3 Code Lists

Name

T-lymphoblastic leukemia/lymphoma (T-ALL/LBL)

ICD-O-3 Morphology

9837/3: Adult T-cell leukemia/lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 4: Rules PH7, PH8
Most common sites of involvement: lymph nodes, peripheral blood.

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

T-lymphoblastic leukemia/lymphoma (T-ALL/LBL) is part of the Precursor T-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B19).

“T-lymphoblastic leukaemia” (T-ALL) is used when the peripheral blood and bone marrow are the primary sites of involvement, and the term “T-lymphoblastic lymphoma” (T-LBL) is used when the primary sites of involvement are lymph node, mediastinum (thymus), or other extranodal sites including the skin, tonsils, liver, spleen, CNS, and testes.

T-ALL typically manifests with a high leukocyte count and often with a concurrent large mediastinal or other tissue mass. Lymphadenopathy and hepatosplenomegaly are common.

T-LBL frequently present with a mass in the anterior mediastinum, often exhibiting rapid growth and respiratory emergency. Pleural and/or pericardial effusions are common.

ETP-ALL typically has bone marrow and peripheral blood involvements. Lymph node and hepatosplenic involvement is common, along with mediastinal involvement, and CNS involvement.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

Module 4: PH7, PH8

Alternate Names

Convoluted T-cell lymphoma (see 9729/3 prior to 2010)
Cortical T ALL
Early T lymphoblastic leukemia/lymphoma
Early T-cell precursor acute lymphoblastic leukemia
Early T-precursor lymphoblastic leukemia/lymphoma (ETP-ALL)
Mature T ALL
Precursor T-cell acute lymphoblastic lymphoma (see 9729/3 prior to 2010)
Precursor T-cell acute lymphoblastic leukemia (Pre-T-ALL)
Precursor T-cell lymphoblastic leukemia/lymphoma (see 9729/3 prior to 2010)
Precursor T-cell lymphoblastic lymphoma, NOS (T-LBL) (see 9729/3 prior to 2010)
Precursor T lymphoblastic lymphoma (see 9729/3 prior to 2010)
Pro T ALL

Definition

T-lymphoblastic leukaemia/lymphoma (T-ALL/LBL) NOS is a neoplasm of hematopoietic progenitors committed to T-lineage differentiation. (WHO 5th edition)

Early T-precursor lymphoblastic leukaemia/lymphoma (ETP-ALL) is a neoplasm composed of blasts committed to the T-cell lineage with a unique immunophenotype that includes the expression of stem cell markers and/or myeloid lineage markers. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Mutations affecting the NOTCH pathway
Mutations in epigenetic regulators (EZH2, SUZ12, and EED)
Mutations in chromatin modifiers ((PHF6, KDM6A, and USP7)

Immunophenotyping

CD1a+ (expression/positive)
CD2+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD5+ (expression/positive)
CD7+ (expression/positive)
CD8+ (expression/positive)
C13+ (expression/positive)
CD19+ (expression/positive)
CD25+ (expression/positive)
CD33+ (expression/positive)
CD79a+ (expression/positive)
FLT3 mutation
KIT (CD117)+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Radiation therapy

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)

None

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

C84.4_ Peripheral T-cell lymphoma, not elsewhere classified (effective October 01, 2015)
C84.A Peripheral T-cell lymphoma, not elsewhere classified, in remission (effective October 01, 2024)
C91.0 Acute lymphoblastic leukemia, not having achieved remission (effective October 01, 2015)
C91.0 Acute lymphoblastic leukemia, in remission (effective October 01, 2024)
C91.0 Acute lymphoblastic leukemia, in relapse (effective October 01, 2024)

Signs and Symptoms

Hepatosplenomegaly
High leukocyte count
Lymphadenopathy
Pleural effusions

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

Incidence: ~15% of childhood ALL (T-ALL/LBL)
Age: more common in adolescents than younger children and adults (T-ALL/LBL)
Sex: slight female predilection (T-ALL/LBL)
Survival: 85% in children and adolescences, 60% in adults (T-ALL/LBL)
Incidence: 12-17% of pediatric and 22-40% of T-ALL cases (ETP-ALL)
Age: Pediatric and adults (ETP-ALL)
Sex: no male or female predilection (ETP-ALL)
Survival: Increasing survival rates, comparable to T-ALL (ETP-ALL)

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: Precursor T-cell neoplasms
Pages: Part B: 651-658

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

PDQ® Adult Treatment Editorial Board. PDQ Acute Lymphoblastic Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <02/28/2025>. Available at: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed <03/29/2025>. [PMID: 26389283]
Section: Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version
Pages: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq
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