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large granular lymphocyte syndrome (LGLS, LGL)
Subset of patients with Felty's syndrome.
Genetics:
- associated with overexpression of EDG8
Clinical manifestations:
- inflammatory arthritis in 25%
Laboratory:
1) complete blood count
- lymphocytosis with profound neutropenia
2) flow cytometry
- clonal proliferation of CD3+, CD8+, CD57+ T-cells
3) peripheral blood smear shows large, granular lymphocytes
4) large granular lymphocytes in blood
Complications:
- recurrent life-threatening bacterial infections
- pure red cell aplasia
Management:
1) cytotoxic agents
- methotrexate
2) patients do NOT respond to splenectomy (in contrast to Felty's syndrome
Related
CD3
CD57 antigen (LEU7)
CD8
methotrexate (Rheumatrex, MTX)
General
Felty's syndrome
lymphoproliferative disorder
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 17
American College of Physicians, Philadelphia 1998, 2015
- Liu X, Loughran TP Jr.
The spectrum of large granular lymphocyte leukemia and
Felty's syndrome.
Curr Opin Hematol. 2011 Jul;18(4):254-9
PMID: 21546829