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Name
T-cell large granular lymphocytic leukemia
ICD-O-3 Morphology
Effective
2010 and later
Reportable
for cases diagnosed
2010 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
(This neoplasm is reportable for cases diagnosed 2010 and later.)
Many patients receive no treatment, some receive aspirin, others corticosteroids, or they may receive chemotherapy in low doses. Some patients have a splenectomy.
Many patients receive no treatment, some receive aspirin, others corticosteroids, or they may receive chemotherapy in low doses. Some patients have a splenectomy.
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.
Grade
Not Applicable
Module Rule
None
Alternate Names
Chronic lymphoproliferative disorder of NK cells
Chronic NK-large granular lymphocyte (LGL) lymphoproliferative disorder
CLPD-NK
Indolent large granular NK-cell lymphoproliferative disorder
Large granular lymphocytosis, NOS
LGL leukemia
NK-cell LGL lymphocytosis
NK-cell large granular lymphocytic leukemia
NK-cell lineage granular lymphocyte proliferative disorder
T-cell granular lymphocytic leukemia
T-cell large granular lymphocytosis
T-cell lymphoproliferative disease of granular lymphocytes
T-gamma lymphoproliferative disease [OBS]
T-LGL
Definition
T-cell large granular lymphocytic leukemia (T-LGLL) is a heterogenuous disorder characterized by a persistent (> 6 months) increase in the number of peripheral blood large granular lymphocytes (LGLs) without a clearly identified cause.
Chronic lymphoproliferative disorder of NK cells (CLPD-NKs) are rare and heterogenuous. They are characterized by a persistent (> 6 months) increase in the peripheral blood NK-cell count without a clearly identified cause. It is difficult to distinguish between reactive and neoplastic conditions without highly specialized techniques. CLPD-NK is a proliferation of NK cells associated with a chronic clinical course and is considered a provisional entity.
Chronic lymphoproliferative disorder of NK cells (CLPD-NKs) are rare and heterogenuous. They are characterized by a persistent (> 6 months) increase in the peripheral blood NK-cell count without a clearly identified cause. It is difficult to distinguish between reactive and neoplastic conditions without highly specialized techniques. CLPD-NK is a proliferation of NK cells associated with a chronic clinical course and is considered a provisional entity.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear
Immunophenotyping
CD2+ (expression/negative)
CD3+ (expression/negative)
CD4+/- (positive and negative)
CD8+/- (positive and negative)
CD16+ (expression/negative)
CD57+ (expression/negative)
Granzyme B+ (expression/negative)
Granzyme M+ (expression/negative)
KIR+ (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-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.7 Other lymphoid leukemia
Corresponding ICD-10-CM Codes (U.S. only)
C91.Z Other lymphoid leukemia (effective October 01, 2015)
Signs and Symptoms
Anemia
Cytopenia
Lymphadenopathy (rare)
Lymphocytosis
Neutropenia
Rheumatoid arthritis
Splenomegaly (moderate)
Thrombocytopenia
Diagnostic Exams
Progression and Transformation
None
Epidemiology and Mortality
Age: 45-75 years (rare before 25 years)
Incidence: 2-3% of all mature lymphocytic leukemias
Sex: no male or female predominance
Survival: indolent course over prolonged period, mortality associated with cytopenias (mostly neutropenia)
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 348-352
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 348-352
International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2
