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Large Granular Lymphocytic Leukemia

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A spectrum of disorders characterized by clonal expansions of the peripheral blood LYMPHOCYTE populations known as large granular lymphocytes which contain abundant cytoplasm and azurophilic granules. Subtypes develop from either CD3-negative NATURAL KILLER CELLS or CD3-positive T-CELLS. The clinical course of both subtypes can vary from spontaneous regression to progressive, malignant disease.

Also Known As:

Leukemia, Large Granular Lymphocytic; LGL Leukemia; Large Granular Lymphocyte Leukemia; Leukemia, LGL; Leukemia, NK-LGL; Leukemia, T-LGL; Lymphoproliferative Disease of Granular Lymphocytes; Lymphoproliferative Disease of Large Granular Lymphocytes; NK-Cell Large Granular Lymphocytic Leukemia; NK-LGL Leukemia; T-Cell Large Granular Lymphocyte Leukemia; T-LGL Leukemia; LGL Leukemias; Leukemia, NK LGL; Leukemia, T Cell Large Granular Lymphocytic; Leukemia, T LGL; Leukemias, LGL; Leukemias, NK-LGL; Leukemias, T-LGL; NK Cell Large Granular Lymphocytic Leukemia; NK LGL Leukemia; NK-LGL Leukemias; T Cell Large Granular Lymphocyte Leukemia; T Cell Large Granular Lymphocytic Leukemia; T LGL Leukemia; T-LGL Leukemias; Leukemia, Lymphocytic, Large Granular; Leukemia, Natural Killer Cell Large Granular Lymphocytic; Leukemia, T-Cell Large Granular Lymphocytic; Natural Killer Cell Large Granular Lymphocytic Leukemia; T-Cell Large Granular Lymphocytic Leukemia

Networked: 227 relevant articles (16 outcomes, 18 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Rare Diseases (Rare Disease)
3.	Pure Red-Cell Aplasia
4.	Infection
5.	Rheumatoid Arthritis

Experts

1.	Loughran, Thomas P: 33 articles (03/2015 - 12/2002)
2.	Maciejewski, Jaroslaw P: 11 articles (12/2013 - 10/2005)
3.	Epling-Burnette, P K: 9 articles (04/2011 - 05/2000)
4.	Sokol, Lubomir: 8 articles (10/2013 - 08/2003)
5.	Liu, Xin: 7 articles (12/2013 - 08/2008)
6.	Lamy, Thierry: 6 articles (04/2014 - 07/2003)
7.	Clemente, Michael J: 6 articles (12/2013 - 10/2009)
8.	Kothapalli, Ravi: 6 articles (08/2008 - 12/2002)
9.	Loughran, T P: 5 articles (04/2015 - 05/2000)
10.	Zambello, Renato: 5 articles (12/2014 - 03/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Large Granular Lymphocytic Leukemia:

1.	alemtuzumab (Campath)FDA Link 10/01/2009 - "While alemtuzumab may be highly effective in large granular lymphocytic leukemia, prospective serial monitoring for the presence of CD52-deficient clonal cytotoxic T-lymphocytes should be a component of clinical trials investigating the efficacy of this drug. " 03/01/2004 - "Efficacy of alemtuzumab treatment for refractory T-cell large granular lymphocytic leukemia." 08/01/2010 - "Rapid and durable molecular response of refractory T-cell large granular lymphocyte leukemia after alemtuzumab treatment." 05/01/2005 - "Few reports on the successful treatment of T-cell large granular lymphocyte (LGL) leukemia with the humanized anti-CD52 monoclonal antibody alemtuzumab are emerging in the literature. " 05/01/2005 - "CD52 expression in T-cell large granular lymphocyte leukemia--implications for treatment with alemtuzumab."
2.	Cyclosporine (Ciclosporin)FDA LinkGeneric 07/01/2007 - "We conclude that cyclosporine therapy may be beneficial for T-LGL leukemia patients. " 02/01/2010 - "Long-term safety and efficacy of cyclosporin A therapy for T-cell large granular lymphocyte leukemia." 05/01/2013 - "Treatment of T-cell large granular lymphocyte leukemia with cyclosporine A: experience in a Chinese single institution." 02/01/2002 - "Response to cyclosporine in a patient with disseminated granuloma annulare associated with CD4+/CD8+(dim)/CD56+ large granular lymphocytic leukemia." 05/01/1998 - "We report reversal of severe neutropenia associated with T-LGL leukemia in five patients treated with cyclosporine (CSA). "
3.	fludarabineIBA 07/01/2012 - "T-cell large granular lymphocytic leukemia demonstrated a high rate of complete hematologic and molecular response to fludarabine, with excellent compliance and tolerability rates. " 07/01/2012 - "Of the six patients investigated with T-cell large granular lymphocytic leukemia who were treated with fludarabine, five (83.3%) were female, and their median age was 36.5 years (range 18 to 73). " 07/01/2012 - "We evaluated the records of six patients with T-cell large granular lymphocytic leukemia who were treated with fludarabine as a first-, second-, or third-line therapy, at a dose of 40 mg/m², for three to five days per month and 6 to 8 cycles. " 07/01/2012 - "The aim of this retrospective study was to investigate the results of T-cell large granular lymphocytic leukemia treatment with fludarabine by assessing the complete hematologic response, the complete molecular response, progression-free survival, and overall survival. " 07/01/2012 - "T-cell large granular lymphocytic leukemia: treatment experience with fludarabine."
4.	AntibodiesIBA 01/01/2013 - "LGL leukemia treatment clearly improved LEMS symptoms, and led to anti-VGCC antibodies value decrease. " 01/01/2015 - "Use of a wide panel of antibodies and newer modalities such as Vβ repertoire analysis helps in accurate subtyping of LGL leukemia. " 12/01/1994 - "Careful clinical observation, periodic examination of anti-EBV antibodies, and the analysis of EBV genome from PBMNC are needed in young patients with CD3-negative lymphoproliferative disease of granular lymphocytes." 12/01/1994 - "CD3-negative lymphoproliferative disease of granular lymphocytes in a girl with an unusual pattern of anti-Epstein-Barr virus antibodies." 08/01/1997 - "However, skin involvement is a frequent feature of T prolymphocytic leukemia (T-PLL) and antibodies against HTLV-I and -II have been reported in individuals with large granular lymphocytic (LGL) leukemia. "
5.	AntigensIBA 09/01/2011 - "Refining the diagnosis of T-cell large granular lymphocytic leukemia by combining distinct patterns of antigen expression with T-cell clonality studies." 08/01/2014 - "Altogether, our results further corroborate antigen selection in the ontogeny of T-LGL leukemia and point to the interplay between genetics and the (micro)environment in shaping the outcome of cytotoxic T cell responses." 05/01/2014 - "LGL leukemia is thought to arise from chronic antigen stimulation, which drives long-term cell survival through the activation of survival signaling pathways and suppression of pro-apoptotic signals. " 05/01/2012 - "Large granular lymphocyte (LGL) leukemia is characterized by clonal expansion of antigen-activated cytotoxic T cells (CTL). " 07/01/2011 - "Phenotypic and functional data have strongly suggested that CD3 LGL leukemia is antigen-activated. "
6.	Methotrexate (Mexate)FDA LinkGeneric 10/01/1994 - "Low-dose oral methotrexate therapy is an effective treatment for some patients with LGL leukemia." 04/01/2015 - "This first prospective trial in LGL leukemia was a multicenter, phase 2 clinical trial evaluating methotrexate (MTX) at 10 mg/m(2) orally weekly as initial therapy (step 1). " 09/01/1996 - "Oral methotrexate is given continuously as treatment for T-LGL leukemia. " 10/01/1994 - "Treatment of large granular lymphocyte leukemia with oral low-dose methotrexate." 03/10/2011 - "The authors use low-dose methotrexate initially for T-LGL leukemia patients with neutropenia and/or RA. "
7.	Sirolimus (Rapamycin)FDA Link 02/01/2012 - "This study was aimed to investigate the immunogenetic diagnosis of large granular lymphocytic leukemia (LGLL) and therapeutic efficacy of sirolimus, and to analysis 256 cases of LGLL reported at home and abroad within 2000 - 2010. " 02/01/2012 - "[Immunogenetic diagnosis of large granular lymphocytic leukemia and therapy by sirolimus]."
8.	T-Cell Antigen Receptors (T-Cell Receptor)IBA 10/01/2015 - "T-cell large granular lymphocytic (T-LGL) leukemia is a rare disorder in which the neoplastic cells usually express the αβ T-cell receptor (TCR). " 02/01/2015 - "Assessment of clonality in T-cell large granular lymphocytic leukemia: flow cytometric T cell receptor Vβ repertoire and T cell receptor gene rearrangement." 01/01/2015 - "T-cell LGL leukemia is typically a disorder of mature CD3, CD8 and T-cell receptor TCR (TCR - T cell receptor)-αβ positive cytotoxic T-cells. " 01/01/2014 - "Clonal CD8(+)/T-cell receptor (TCR)αβ(+) T-cell large granular lymphocyte (T-LGL) proliferations constitute the most common subtype of T-LGL leukemia. " 12/12/2013 - "Deep sequencing of the T-cell receptor repertoire in CD8+ T-large granular lymphocyte leukemia identifies signature landscapes."
9.	Mitoxantrone (Novantrone)FDA LinkGeneric 10/01/2007 - "Fludarabine, mitoxantrone and dexamethasone as first-line treatment for T-cell large granular lymphocyte leukemia."
10.	Dexamethasone (Maxidex)FDA LinkGeneric 10/01/2007 - "Fludarabine, mitoxantrone and dexamethasone as first-line treatment for T-cell large granular lymphocyte leukemia."

Therapies and Procedures

1.	Splenectomy 09/01/2008 - "Outcomes of splenectomy in T-cell large granular lymphocyte leukemia with splenomegaly and cytopenia." 10/01/1996 - "Resolution of autoimmune hemolytic anemia following splenectomy in CD3+ large granular lymphocyte leukemia." 10/01/1996 - "We suggest that splenectomy may be an effective treatment for autoimmune hemolytic anemia and/or thrombocytopenia often associated with T-LGL leukemia. "
2.	Photopheresis 09/01/2012 - "To evaluate the efficacy of extracorporeal photopheresis (ECP) in noncutaneous T-cell lymphoma and large granular lymphocytes leukemia (LGL). " 09/01/2012 - "Extracorporeal photopheresis as a curative treatment strategy in non epidermotropic T-cell lymphoma and large granular lymphocyte leukemia."
3.	Therapeutics 05/01/2014 - "New studies surrounding Jak-Stat signaling suggest this may reveal new avenues for LGL leukemia therapeutics. " 12/01/2013 - "This model should be useful for investigating molecular pathogenesis of the disease and for developing new therapeutics targeting T-LGL leukemia. " 12/01/2011 - "In order to establish new therapeutics it is important to understand the current state of LGL leukemia both in the clinic and in basic research." 05/01/2001 - "The epidemiology, prognosis factors, therapeutics and the pathogenesis of LGL leukemia are unknown. "
4.	Kidney Transplantation 01/15/2007 - "Large granular lymphocyte leukemia after renal transplantation: an immunologic, immunohistochemical, and genotypic study." 01/01/1996 - "Successful renal transplantation in a patient with large granular lymphocytic leukemia with natural killer cell proliferation." 01/01/1998 - "Herein, we report a large granular lymphocyte (LGL) leukemia patient with pure red cell aplasia (PRCA) after renal transplantation. "
5.	Intravenous Infusions 06/01/2015 - "We gave intravenous infusions of Resimmune 2.5 - 11.25 μg/kg over 15 minutes to 30 patients (25 with cutaneous T-cell lymphoma, 3 with peripheral T-cell lymphoma, 1 with T-cell large granular lymphocytic leukemia and 1 with T-cell prolymphocytic leukemia) in an inter-patient dose escalation trial. "