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

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Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
None
Alternate Names
Chronic large granular lymphocyte lymphoproliferative disorder
Chronic lymphoproliferative disorder of NK cells
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
LGL leukemia
NK-cell LGL lymphocytosis
NK-cell lineage granular lymphocyte proliferative disorder
T-cell granular lymphocytic leukemia
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 T-cell large granular leukemia and chronic lymphoproliferative disorder of NK cells/chronic NK-cell large granular lymphocytic leukemia. This was formerly 9831/1 in ICD-O-3.

T-LGL leukemia involves the peripheral blood, bone marrow, liver, and spleen. Lymphadenopathy is very rare. This leukemia represents 3% of cases of mature lymphocytic leukemias. The disease is rare before 25 years of age and the majority of cases occur in those between ages 45-75. Severe neutropenia with or without anemia is frequent while thrombocytopenia is not. Moderate splenomegaly is the main physical finding. Rheumatoid arthritis, the presence of autoantibodies in the circulating immune complexes and hypergammaglobulinemia are common. The lymphoproliferation is typically indolent and non-progressive. Mortality is associated with cytopenias, especially neutropenia. Many patients receive no treatment, some receive aspirin, others corticosteroids, or they may receive chemotherapy in low doses. Some patients have a splenectomy.

Chronic lymphoproliferative disorder of NK cells predominantly involve the peripheral blood and bone marrow. The majority of patients are asymptomatic but some present with systemic symptoms and/or cytopenias (mainly neutropenia and anemia). Lymphadenopathy, hepatosplenomegaly, and cutaneous lesions are infrequent. This neoplasm may occur in association with other medical conditions such as solid and hematologic tumors, vasculitis, splenectomy, neuropathy, and autoimmune disorders. CLP-NK occurs predominantly in adults with a median age of 60 years. In the majority of patients the clinical course is indolent over a prolonged period. Disease progression with increasing lymphocytosis and worsening of cytopenia is observed in some cases.
Definitive Diagnostic Methods
Bone Marrow biopsy
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
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
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None