Format

Send to

Choose Destination
Blood Rev. 2014 May;28(3):87-94. doi: 10.1016/j.blre.2014.02.001. Epub 2014 Mar 7.

The pathogenesis and treatment of large granular lymphocyte leukemia.

Author information

1
Penn State Hershey Cancer Institute, Penn State College of Medicine, Hershey, PA, USA.
2
University of Virginia Cancer Center, University of Virginia, Charlottesville, VA, USA. Electronic address: TL7CS@hscmail.mcc.virginia.edu.

Abstract

Large granular lymphocyte (LGL) leukemia is a spectrum of rare lymphoproliferative diseases of T lymphocytes and natural killer cells. These diseases frequently present with splenomegaly, neutropenia, and autoimmune diseases like rheumatoid arthritis. LGL leukemia is more commonly of a chronic, indolent nature; however, rarely, they have an aggressive course. 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. These include Jak-Stat, Mapk, Pi3k-Akt, sphingolipid, and IL-15/Pdgf signaling. Treatment traditionally includes immunosuppression with low dose methotrexate, cyclophosphamide, and other immunosuppressive agents; however, prospective and retrospective studies reveal very limited success. New studies surrounding Jak-Stat signaling suggest this may reveal new avenues for LGL leukemia therapeutics.

KEYWORDS:

Cell signaling; Large granular lymphocyte leukemia; Pathogenesis

PMID:
24679833
PMCID:
PMC4155502
DOI:
10.1016/j.blre.2014.02.001
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Elsevier Science Icon for PubMed Central
Loading ...
Support Center