T-lymphoblastic Leukemia/lymphoma

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Home Registry Operations Reporting Guidelines Hematopoietic Project Neoplasm Information T-lymphoblastic leukemia/lymphoma (T-ALL/LBL) 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.

Help me code for diagnosis year : 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 2021 2022 2023 2024 2025 2026

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 ALLEarly T lymphoblastic leukemia/lymphomaEarly T-cell precursor acute lymphoblastic leukemiaEarly T-precursor lymphoblastic leukemia/lymphoma (ETP-ALL)Mature T ALLPrecursor 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

CytogeneticsGenetic testingHistologic confirmationImmunohistochemistryImmunophenotyping

Genetics Data

Mutations affecting the NOTCH pathwayMutations 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 mutationKIT (CD117)+ (expression/positive)

Treatments

ChemotherapyHematologic Transplant and/or Endocrine ProceduresHormone therapyRadiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

9590/3 Malignant lymphoma, NOS 9591/3 Malignant lymphoma, non-Hodgkin, NOS 9729/3 Precursor T-cell lymphoblastic lymphoma, NOS (T-LBL) (for 2010+, see 9837/3) 9800/3 Leukemia, NOS 9801/3 Acute undifferentiated leukemia 9820/3 Lymphoid leukemia, NOS

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

HepatosplenomegalyHigh leukocyte countLymphadenopathyPleural effusionsRapid growth mediastinal mass (anterior mediastinum)Respiratory failure (emergency)

Diagnostic Exams

Blood chemistry studiesBone marrow aspiration and biopsyCT (CAT) scanComplete blood count (CBC)Cytogenetic analysisFlow cytometryImmunohistochemistryImmunophenotypingLaparoscopy (rarely performed)Laparotomy (rarely performed)Lymph node biopsyMRI (magnetic resonance imaging)PET (positron emission tomography) scanPhysical exam and history (H&P)

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 neoplasmsPages: 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 CodesPages: 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 VersionPages: https://www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq Glossary