Chapter 57: Large Granular Lymphocytic Leukemia

T-cell large-granular lymphocytic (T-LGL) leukemia results from the clonal expansion of large granular lymphocytes (LGLs) with a T-cell (CD3+) phenotype and a clonal T-cell receptor gene rearrangement(s).
Natural killer (NK)-LGL leukemia is a clonal expansion of LGL with a NK cell (CD3–) phenotype. It lacks convenient markers to determine clonality, such as antigen receptor rearrangements. (See Chap. 66.)

There is suggestive evidence of a role for human T lymphotropic virus (HTLV) retroviral infection in some patients.
Most patients are not infected with either HTLV-I or HTLV-II.
Cytomegalovirus implicated in rare cases of CD4+ T-LGL.
Leukemic cells have features of antigen-activated cytotoxic T lymphocytes, suggesting a role for antigen in initial LGL expansion.
Constitutive overexpression of the Fas ligand (CD178), which also is found at high levels in patients’ sera, may be a factor in many disease manifestations (eg, neutropenia, rheumatoid arthritis).

About half of patients have palpable splenomegaly. (Table 57–1)
About one-third of patients have recurrent bacterial infections.
“B symptoms” (eg, low-grade fevers, night sweats, and/or weight loss) (aggressive variant) are very infrequent.
About one-fourth of patients have rheumatoid arthritis, often with features of “Felty syndrome.”
Less than one-tenth of patients have lymphadenopathy.

TABLE 57–1CLINICAL FEATURES OF T-CELL LARGE GRANULAR LYMPHOCYTIC LEUKEMIA

TABLE 57–1CLINICAL FEATURES OF T-CELL LARGE GRANULAR LYMPHOCYTIC LEUKEMIA

	Pandolfi (1990)	Loughran (1993)	Dhodapkar (1994)	Semenzato (1997)	Neben (2003)	Bareau (2010)
Number of patients	151	129	68	162	44	201
Median age	55	57	61	59	63	59
Male/Female	1.3	0.8	1	0.8	1.0	0.8
Symptomatic	72%	–	69%	–	73%	82%
Splenomegaly	50%	50%	19%	50%	35%	24%
Hepatomegaly	34%	23%	1%	32%	–	10%
Adenopathy	13%	1%	3%	13%	5%	6%
B symptoms	–	–	12%	–	–	7%
Infections	38%	39%	15%	56%	–	23%
Rheumatoid arthritis	12%	28%	26%	36%	20%	17%
Rheumatoid factor	–	57%	61%	43%	48%	41%
Antinuclear antibodies	–	38%	44%	38%	48%	48%
Autoimmune cytopenias	–	–	7%	9%	5%	7%
Lymphocytosis				29%
LGL > 4 × 10⁹/L	52%	52%	–	–	–	14%
LGL 1–4 × 10⁹/L	38%	40%	–	–	–	50%
LGL < 1 × 10⁹/L	10%	8%	–	7%	–	36%
Neutropenia
Moderate (< 1.5 × 10⁹/L)	64%	84%	74%	–	52%	61%
Severe (0.5 × 10⁹/L)	7%	48%	40%	37%	41%	26%
Anemia
Any severity	25%	49%	51%	26%	89%	24%
Severe (Hgb < 8 g/dL)	37%	–	19%	–	36%	7%
Thrombocytopenia	9%	19%	20%	29%	36%	19%
LGL marrow infiltration	67%	88%	–	76%	83%	72%
Hypergammaglobulinemia	–	45%	5%	43%	–	35%
Monoclonal gammopathy	–	45%	8%	–	–	10%
Need for treatment	30%	73%	69%	33%	80%	44%
LGLL-related death	14%	36%	8%	27%	–	7%

Hgb, hemoglobin; ...

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