Search Database ICD-O-3 Code Lists

Name

Adult T-cell leukemia/lymphoma (ATLL)

ICD-O-3 Morphology

9827/3: Adult T-cell leukemia/ lymphoma (HTLV-1 positive)
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: peripheral blood, bone marrow, lymph nodes, spleen, liver, gastrointestinal tract, bone, lung, CNS

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Adult T-cell leukemia/lymphoma (ATLL) is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)

Patients with ATLL exhibit diverse clinical features, including generalized lymphadenopathy, cutaneous lesions, hepatosplenomegaly, and features related to the infiltration of various organs, particularly the CNS, gastrointestinal tract, bone, and lung. Many patients have an associated immunodeficiency.

ATLL is endemic in several regions of the world south-western Japan, Carribean, Intertropical Africa, Middle East, South American and Papua New Guinea.

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

Adult T-cell leukemia/lymphoma (HTLV-1 positive/associated)
Chronic adult T-cell leukemia/lymphoma (Chronic ATLL)
Cutaneous adult T-cell leukemia/lymphoma
Smoldering adult T-cell leukemia/lymphoma (Smoldering ATLL)

Definition

Adult T-cell leukaemia/lymphoma (ATLL) is a mature T-cell neoplasm associated with HTLV-1. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

HTLV-1 monoclonal integration
TR genes are clonally rearranged

Immunophenotyping

CCR4+ (expression/positive)
CD2+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD5+ (expression/positive)
CD7- (no expression/negative)
CD8+ (expression/positive)
CD25+ (expression/positive)
FOXP3+ (expression/positive)

Treatments

Chemotherapy
Immunotherapy
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)

C91.5 Adult T-cell leukemia

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

C91.50 Adult T-cell lymphoma/leukemia (HTLV-1-associated), not having achieved remission (effective October 01, 2015)
C91.51 Adult T-cell lymphoma/leukemia (HTLV-1-associated), in remission (effective October 01, 2024)
C91.52 Adult T-cell lymphoma/leukemia (HTLV-1-associated), in relapase (effective October 01, 2024)

Signs and Symptoms

Elevated white blood cell count
Exfoliative skin rash
Fatigue
Fever
Hepatosplenomegaly
Hypercalcemia
Lymphadenopathy
Lymphocytosis
Pneumocystitis
Skin or pulmonary lesions
Weight loss
Widespread lymph node involvement

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Lymph node biopsy
Peripheral blood smear
PET (positron emission tomography) scan

Progression and Transformation

Progression from the chronic or smoldering to the acute variant occurs in 25% of cases, but usually after a long duration.

Epidemiology and Mortality

Incidence: endemic in several regions of the world Southwestern Japan, Caribbean, Central Africa
Age: adults, median age 68
Sex: no information
Survival: depends on stage and the clinical subtype

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: Mature T- and NK-cell neoplasms
Pages: Part B: 667-673

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 Peripheral T-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <03/13/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq. Accessed <03/31/2025>. [PMID: 37437079]
Section: Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq#_2289
Glossary