large granular lymphocyte syndrome (LGLS, LGL)
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Introduction
Subset of patients with Felty's syndrome.
Genetics
- associated with overexpression of EDG8
Clinical manifestations
- inflammatory arthritis in 25%
Laboratory
- complete blood count
- lymphocytosis with profound neutropenia
- flow cytometry
- clonal proliferation of CD3+, CD8+, CD57+ T-cells
- peripheral blood smear shows large, granular lymphocytes
- large granular lymphocytes in blood
Complications
- recurrent life-threatening bacterial infections
- pure red cell aplasia
Management
- cytotoxic agents
- patients do NOT respond to splenectomy (in contrast to Felty's syndrome
More general terms
Additional terms
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: https://www.ncbi.nlm.nih.gov/pubmed/21546829