T-cell large granular lymphocytic leukemia

Name
T-cell large granular lymphocytic leukemia
ICD-O-3 Morphology
9831/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)

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Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
None
Alternate Names
Chronic NK-cell lymphocytosis
Chronic NK-large granular lymphocyte (LGL) lymphoproliferative disorder
CLPD-NK
Indolent large granular NK-cell lymphoproliferative disorder
Large granular lymphocytosis, NOS
NK-cell LGL lymphocytosis
NK-cell lineage granular lymphocyte proliferative disorder
T-cell large granular lymphocytosis
T-LGL
Definition
T-LGL is a heterogeneous disorder charcterized by a persistent (>6 months) increase in the number of peripheral blood large granular lymphocytes (LGL) without a clearly identified cause.
Abstractor Notes
(This neoplasm is reportable for cases diagnosed 2010 and later.)

This code is used to describe:
1.T-cell large granular leukemia and chronic lymphoproliferative disorder of NK cells/chronic NK-cell large granular lymphocytic leukemia-involves the peripheral blood, bone marrow, liver, and spleen. This was formerly 9831/1 in ICD-O-3.

2. Chronic lymphoproliferative disorder of NK cells- predominantly involves the peripheral blood and bone marrow. This neoplasm may occur in association with other medical conditions such as solid and hematologic tumors, vasculitis, splenectomy, neuropathy, and autoimmune disorders.

Many patients receive no treatment, some receive aspirin, others corticosteroids, or they may receive chemotherapy in low doses. Some patients have a splenectomy.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Peripheral blood smear
Genetics Data
TCR gene rearrangement
Immunophenotyping
CD3+
CD3c+ (CLPD-NK)
CD4-
CD8+
CD16+ (CLPD-NK)
CD56+
CD57+
Granzyme B + (CLPD-NK)
Granzyme M + (CLPD-NK)
Lost expression CD5
Lost expression CD7
Mature T-cells with rearranged TCRab genes
Surface CD3- (CLPD-NK)
T1A1+ (CLPD-NK)
TCRab
Weak CD56 expression (CLPD-NK)
Treatments
Chemotherapy
Other RX
Surgery (splenectomy)
Transformations to
None
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.7 Other lymphoid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.Z Other lymphoid leukemia
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)