Search Database ICD-O-3 Code Lists

Name

T-large granular lymphocytic leukemia (T-LGLL)

ICD-O-3 Morphology

9831/3: T-cell large granular lymphocytic leukemia
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

T-large granular lymphocytic leukemia (T-LGLL) 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)

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9820/3).

T-LGLL involves the peripheral blood, bone marrow, and spleen. Liver involvement may be present, whereas lymph node involvement is exceedingly rare.

Frequently associated with auto immune disorders.

NK-LGLL typically involves the peripheral blood and bone marrow. Splenomegaly is uncommon, and hepatomegaly, lymphadenopathy, and skin involvement are not typical

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

None

Alternate Names

Chronic lymphoproliferative disorder of NK cells (CLPD-NK)
Chronic NK-large granular lymphocyte lymphoproliferative disorder
Indolent CD4+/CD8+ T-granular lymphocytic leukemia
Large granular lymphocytosis, NOS
T-cell large granular lymphocytosis
T-cell lymphoproliferative disease of granular lymphocytes

Definition

T-large granular lymphocytic leukaemia (T-LGLL) is a neoplastic proliferation of cytotoxic large granular T cells presenting with persistent absolute lymphocytosis (> 2 × 109/L). (WHO 5th edition)

NK-large granular lymphocytic leukaemia (NK-LGLL) is a neoplasm characterized by a persistent increase in peripheral blood NK cells (usually > 2 × 109/L) and a chronic indolent clinical course. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

STAT3 mutations
TR genes are clonally rearragned

Immunophenotyping

CD2+ (expression/negative)
CD3+ (expression/negative)
CD4+/- (positive and negative)
CD8+/- (positive and negative)
CD16+ (expression/negative)
CD57+ (expression/negative)
TCR+ (expression/negative) cytotoxic T cells (alpha beta and gamma delta)

Treatments

Chemotherapy
Other therapy
Surgery

Transformations to

None

Transformations from

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C91.7 Other lymphoid leukemia

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

C91.Z0 Other lymphoid leukemia not having achieved remission (effective October 01, 2015)
C91.Z1 Other lymphoid leukemia, in remission (effective October 01, 2024)
C91.Z2 Other lymphoid leukemia, in relapse (effective October 01, 2024)

Signs and Symptoms

Anemia
Cytopenia
Lymphocytosis
Neutropenia
Rheumatoid arthritis
Splenomegaly (moderate)
Thrombocytopenia

Diagnostic Exams

Blood chemistry studies
Bone marrow biopsy and aspiration
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Peripheral blood smear

Progression and Transformation

None

Epidemiology and Mortality

Incidence: <5% of lymphoproliferative disorders (0.02 cases per 100,000) (T-LGLL)
Age: Median age 60 years (T-LGLL)
Sex: no male or female predilection (T-LGLL)
Survival: indolent course over prolonged period, mortality associated with cytopenias (mostly neutropenia) (T-LGLL)
Incidence: no information available (NK-LGLL)
Age: Median age 60 years (NK-LGLL)
Sex: no male or female predilection (NK-LGLL)
Survival: indolent course over prolonged period, mortality associated with cytopenias (mostly neutropenia) (NK-LGLL)

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: 662-666

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2011, 2020.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2
Glossary