T-cell large granular lymphocyte leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [5] Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [6] Maria Fernanda Villarreal, M.D. [7]

Synonyms and Keywords: LGL leukemia; Tγ-lymphoproliferative disorder; T-cell chronic lymphocytic leukemia; proliferation of large granular lymphocytes (LGLs)

Overview

T-cell large granular lymphocyte leukemia (also known as T-GLL) is a rare type of leukemia that exhibits an unexplained, chronic (> 6 months) elevation in large granular lymphocytes (LGLs) in the peripheral blood. T-cell large granular lymphocyte leukemia was first discovered by McKenna, in 1977. T-cell large granular lymphocyte leukemia may be classified into 2 groups: T-cell large granular lymphocyte leukemia (T-LGL) and natural-killer (NK) granular lymphocyte leukemia (NK-LGL). The pathogenesis of T-cell large granular lymphocyte leukemia is characterized by the involvement of cytotoxic T cells. The postulated cells of origin of T-LGL leukemia are transformed CD8+ T-cells with clonal rearrangements of β chain T-cell receptor genes for the majority of cases and a CD8- T-cell with clonal rearrangements of γ chain T-cell receptor genes for a minority of cases. The molecular pathogenesis of T-cell large granular lymphocyte leukemia is characterized by the disregulation of signaling pathways, such as: FAS/FAS-L, phosphatidylinositol-3 kinase (PI3K), and mitogen-activated proteinkinase/extracellular signal-regulated kinase (MAPK/ERK). Patients of all age groups may develop T-cell large granular lymphocyte leukemia. T-cell large granular lymphocyte leukemia is more commonly observed among middle aged adults. Laboratory findings consistent with the diagnosis of T-cell large granular lymphocyte leukemia include neutropenia, anemia, hypergammaglobulinemia, and lymphocytosis (most common). The diagnosis of T-cell large granular lymphocyte leukemia is made when the following diagnostic criteria is met: clonal rearrangements of the T-cell receptor (TCR) gene, chronic (> 6 months) elevation in large granular lymphocytes (LGLs) in the peripheral blood, large granular lymphocyte count greater than 2.0 × 109/L, and lymphocytosis (typically 2-20x109/L). The mainstay of therapy for T-cell large granular lymphocyte leukemia is targeted-chemotherapy. Initial therapy for patients with T-cell large granular lymphocyte leukemia includes corticosteroids and methotrexate. Alemtuzumab and tipifarnib are the treatment of choice for patients with refractory T-cell large granular lymphocytic leukemia.

Historical Perspective

  • T-cell large granular lymphocyte leukemia was first discovered by McKenna, in 1977.[1]

Classification

T-cell large granular lymphocyte leukemia may be classified into 2 groups:[2]

  • T-cell large granular lymphocyte leukemia (T-LGL)
  • Natural-killer (NK) granular lymphocyte leukemia (NK-LGL)

Pathophysiology

  • The increased expression of STAT3 has been associated with the development of T-cell large granular lymphocyte leukemia, involving the janus kinase family pathway.
  • On immunohistochemistry, characteristic findings of T-cell large granular lymphocyte leukemia include:

Causes

Common causes of T-cell large granular lymphocyte leukemia include:[1]

Differentiating T-cell Large Granular Lymphocyte Leukemia from Other Diseases

  • T-cell large granular lymphocyte leukemia must be differentiated from other diseases that cause recurrent infections, fatigue, and night fever, such as:

Epidemiology and Demographics

Age

  • Patients of all age groups may develop T-cell large granular lymphocyte leukemia.
  • T-cell large granular lymphocyte leukemia is more commonly observed among middle aged adults.[3]

Gender

  • T-cell large granular lymphocyte leukemia affects men and women equally.

Race

  • There is no racial predilection for T-cell large granular lymphocyte leukemia.

Risk Factors

  • The most common risk factor in the development of T-cell large granular lymphocyte leukemia is exposure to radiation.[3]

Natural History, Complications and Prognosis

  • Prognosis is generally good, and the 5 year survival rate of patients with T-cell large granular lymphocyte leukemia is approximately 89%.[2]

Diagnosis

Diagnostic Study of Choice

The diagnosis of T-cell large granular lymphocyte leukemia is made when the following diagnostic criteria is met:[3]

Symptoms

Physical Examination

Laboratory Findings

Laboratory findings consistent with the diagnosis of T-cell large granular lymphocyte leukemia include:

Imaging Findings

  • There are no specific imaging findings associated with T-cell large granular lymphocyte leukemia.

Other Diagnostic Studies

  • T-cell large granular lymphocyte leukemia may also be diagnosed using the following studies:

Immunophenotyping

Type Immunophenotype
Common type (80% of cases) CD3+, TCRαβ+, CD4-, CD8+
Rare variants CD3+, TCRαβ+, CD4+, CD8-
CD3+, TCRαβ+, CD4+, CD8+
CD3+, TCRγδ+, CD4 and CD8 variable

Peripheral blood smear

Treatment

Medical Therapy

Prevention

References

  1. 1.0 1.1 Leblanc F, Zhang D, Liu X, Loughran TP (2012). "Large granular lymphocyte leukemia: from dysregulated pathways to therapeutic targets". Future Oncol. 8 (7): 787–801. doi:10.2217/fon.12.75. PMC 3464048. PMID 22830400.
  2. 2.0 2.1 2.2 2.3 [1] Lamy T, Loughran TP Jr. "Clinical features of large granular lymphocyte leukemia." Semin Hematol. 2003 Jul;40(3):185-95. PMID: 12876667
  3. 3.0 3.1 3.2 3.3 3.4 3.5 [2] Chan WC, Link S, Mawle A, Check I, Brynes RK, Winton EF. "Heterogeneity of large granular lymphocyte proliferations: delineation of two major subtypes." Blood. 1986 Nov;68(5):1142-53. PMID: 3490288
  4. [3] Lamy T, Loughran TP. "Large Granular Lymphocyte Leukemia." Cancer Control. 1998 Jan;5(1):25-33. PMID: 10761014
  5. [4] Pandolfi F, Loughran TP Jr, Starkebaum G, Chisesi T, Barbui T, Chan WC, Brouet JC, De Rossi G, McKenna RW, Salsano F, et al. "Clinical course and prognosis of the lymphoproliferative disease of granular lymphocytes. A multicenter study." Cancer. 1990 Jan 15;65(2):341-8. PMID: 2403836

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