T-cell large granular lymphocyte leukemia

Jump to navigation Jump to search
T-cell large granular lymphocyte leukemia
ICD-O: 9831/3
MeSH D054066

WikiDoc Resources for T-cell large granular lymphocyte leukemia

Articles

Most recent articles on T-cell large granular lymphocyte leukemia

Most cited articles on T-cell large granular lymphocyte leukemia

Review articles on T-cell large granular lymphocyte leukemia

Articles on T-cell large granular lymphocyte leukemia in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on T-cell large granular lymphocyte leukemia

Images of T-cell large granular lymphocyte leukemia

Photos of T-cell large granular lymphocyte leukemia

Podcasts & MP3s on T-cell large granular lymphocyte leukemia

Videos on T-cell large granular lymphocyte leukemia

Evidence Based Medicine

Cochrane Collaboration on T-cell large granular lymphocyte leukemia

Bandolier on T-cell large granular lymphocyte leukemia

TRIP on T-cell large granular lymphocyte leukemia

Clinical Trials

Ongoing Trials on T-cell large granular lymphocyte leukemia at Clinical Trials.gov

Trial results on T-cell large granular lymphocyte leukemia

Clinical Trials on T-cell large granular lymphocyte leukemia at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on T-cell large granular lymphocyte leukemia

NICE Guidance on T-cell large granular lymphocyte leukemia

NHS PRODIGY Guidance

FDA on T-cell large granular lymphocyte leukemia

CDC on T-cell large granular lymphocyte leukemia

Books

Books on T-cell large granular lymphocyte leukemia

News

T-cell large granular lymphocyte leukemia in the news

Be alerted to news on T-cell large granular lymphocyte leukemia

News trends on T-cell large granular lymphocyte leukemia

Commentary

Blogs on T-cell large granular lymphocyte leukemia

Definitions

Definitions of T-cell large granular lymphocyte leukemia

Patient Resources / Community

Patient resources on T-cell large granular lymphocyte leukemia

Discussion groups on T-cell large granular lymphocyte leukemia

Patient Handouts on T-cell large granular lymphocyte leukemia

Directions to Hospitals Treating T-cell large granular lymphocyte leukemia

Risk calculators and risk factors for T-cell large granular lymphocyte leukemia

Healthcare Provider Resources

Symptoms of T-cell large granular lymphocyte leukemia

Causes & Risk Factors for T-cell large granular lymphocyte leukemia

Diagnostic studies for T-cell large granular lymphocyte leukemia

Treatment of T-cell large granular lymphocyte leukemia

Continuing Medical Education (CME)

CME Programs on T-cell large granular lymphocyte leukemia

International

T-cell large granular lymphocyte leukemia en Espanol

T-cell large granular lymphocyte leukemia en Francais

Business

T-cell large granular lymphocyte leukemia in the Marketplace

Patents on T-cell large granular lymphocyte leukemia

Experimental / Informatics

List of terms related to T-cell large granular lymphocyte leukemia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [8]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [9]

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 is a disease that exhibits a unexplained, chronic (> 6 months) elevation in large granular lymphocytes (LGLs) in the peripheral blood.[1]

Pathology

The postulated cells of origin are a transformed CD8+ T-cell 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.[1]

Genetics

Clonal rearrangements of the T-cell receptor (TCR) genes are a necessary condition for the diagnosis of this disease. The gene for the β chain of the TCR is found to be rearranged more often than the γ chain. of the TCR.[2]

Gross Pathology

The leukemic cells of T-LGL can be found in peripheral blood, bone marrow, spleen, and liver. Nodal involvement is rare.[1][3]

Microscopic Pathology

Peripheral blood

The neoplastic lymphocytes seen in this disease are large in size with azurophilic granules that contains proteins involved in cell lysis such as perforin and granzyme B.[4]

Bone marrow

Bone marrow involvement in this disease is often present, but to a variable extent. The lymphocytic infiltrate is usually interstitial, but a nodular pattern rarely occurs.[1]

Epidemiology and Demographics

T-LGL is a rare form of leukemia, comprising 2-3% of all cases of small lymphocytic leukemias.[1]

Natural History, Complications ans Prognosis

  • This disease is known for an indolent clinical course and incidental discovery.[1]
  • The 5 year survival has been noted as 89% in at least one study from France of 201 patients with T-LGL leukemia.[5]

Diagnosis

Symptoms

Physical Examination

Skin

Abdomen

Laboratory Findings

  • Complete blood count and differential count - neutropenia
  • The requisite lymphocytosis of this disease is typically 2-20x109/L.
  • Immunoglobulin derangements including hypergammaglobulinemia, autoantibodies, and circulating immune complexes are commonly seen.[8][9][10][11]

Immunophenotype

The neoplastic cells of this disease display a mature T-cell immunophenotype, with the majority of cases showing a CD4-/CD8+ T-cell subset immunophenotype versus other permutations of those markers.[6][7] Variable expression of CD11b, CD56, and CD57[8] are observed. Immunohistochemisty for perforin, TIA-1, and granzyme B are usually positive.[1]

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

Treatment

Alemtuzumab has been investigated for use in treatment of refractory T-cell large granular lymphocytic leukemia.[12]

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 [1] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001
  2. [2] Vie H, Chevalier S, Garand R, Moisan JP, Praloran V, Devilder MC, Moreau JF, Soulillou JP. "Clonal expansion of lymphocytes bearing the gamma delta T-cell receptor in a patient with large granular lymphocyte disorder." Blood. 1989 Jul;74(1):285-90. PMID: 2546620
  3. 3.0 3.1 [3] Lamy T, Loughran TP. "Large Granular Lymphocyte Leukemia." Cancer Control. 1998 Jan;5(1):25-33. PMID: 10761014
  4. [4] Semenzato G, Zambello R, Starkebaum G, Oshimi K, Loughran TP Jr. "The lymphoproliferative disease of granular lymphocytes: updated criteria for diagnosis." Blood. 1997 Jan 1;89(1):256-60. PMID: 8978299
  5. Bareau, B; Rey, J; Hamidou, M; Donadieu, J; Morcet, J; Reman, O; Schleinitz, N; Tournilhac, O; Roussel, M (2010). "Analysis of a French cohort of patients with large granular lymphocyte leukemia: A report on 229 cases". Haematologica. 95 (9): 1534–41. doi:10.3324/haematol.2009.018481. PMC 2930955. PMID 20378561.
  6. 6.0 6.1 [5] 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
  7. 7.0 7.1 [6] 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
  8. 8.0 8.1 8.2 [7] Lamy T, Loughran TP Jr. "Clinical features of large granular lymphocyte leukemia." Semin Hematol. 2003 Jul;40(3):185-95. PMID: 12876667
  9. Oshimi K, Yamada O, Kaneko T; et al. (1993). "Laboratory findings and clinical courses of 33 patients with granular lymphocyte-proliferative disorders". Leukemia. 7 (6): 782–8. PMID 8388971. Unknown parameter |month= ignored (help)
  10. Loughran TP, Starkebaum G, Aprile JA (1988). "Rearrangement and expression of T-cell receptor genes in large granular lymphocyte leukemia". Blood. 71 (3): 822–4. PMID 3345349. Unknown parameter |month= ignored (help)
  11. Loughran TP, Kadin ME, Starkebaum G; et al. (1985). "Leukemia of large granular lymphocytes: association with clonal chromosomal abnormalities and autoimmune neutropenia, thrombocytopenia, and hemolytic anemia". Ann. Intern. Med. 102 (2): 169–75. PMID 3966754. Unknown parameter |month= ignored (help)
  12. Rosenblum MD, LaBelle JL, Chang CC, Margolis DA, Schauer DW, Vesole DH (2004). "Efficacy of alemtuzumab treatment for refractory T-cell large granular lymphocytic leukemia". Blood. 103 (5): 1969–71. doi:10.1182/blood-2003-11-3951. PMID 14976065. Unknown parameter |month= ignored (help)

Template:WikiDoc Sources