Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ DEFINITION ++ T-cell large-granular lymphocytic (T-LGL) leukemia results from the clonal expansion of large granular lymphocytes (LGLs) with a T-cell (CD3+) phenotype and a clonal T-cell receptor gene rearrangement(s). Natural killer (NK)-LGL leukemia is a clonal expansion of LGL with a NK cell (CD3–) phenotype. It lacks convenient markers to determine clonality, such as antigen receptor rearrangements. (See Chap. 66.) +++ T-LGL LEUKEMIA +++ Etiology and Pathogenesis ++ There is suggestive evidence of a role for human T lymphotropic virus (HTLV) retroviral infection in some patients. Most patients are not infected with either HTLV-I or HTLV-II. Cytomegalovirus implicated in rare cases of CD4+ T-LGL. Leukemic cells have features of antigen-activated cytotoxic T lymphocytes, suggesting a role for antigen in initial LGL expansion. Constitutive overexpression of the Fas ligand (CD178), which also is found at high levels in patients’ sera, may be a factor in many disease manifestations (eg, neutropenia, rheumatoid arthritis). +++ Clinical Features ++ About half of patients have palpable splenomegaly. (Table 57–1) About one-third of patients have recurrent bacterial infections. “B symptoms” (eg, low-grade fevers, night sweats, and/or weight loss) (aggressive variant) are very infrequent. About one-fourth of patients have rheumatoid arthritis, often with features of “Felty syndrome.” Less than one-tenth of patients have lymphadenopathy. ++Table Graphic Jump LocationTABLE 57–1CLINICAL FEATURES OF T-CELL LARGE GRANULAR LYMPHOCYTIC LEUKEMIAView Table||Download (.pdf) TABLE 57–1CLINICAL FEATURES OF T-CELL LARGE GRANULAR LYMPHOCYTIC LEUKEMIA Pandolfi (1990) Loughran (1993) Dhodapkar (1994) Semenzato (1997) Neben (2003) Bareau (2010) Number of patients 151 129 68 162 44 201 Median age 55 57 61 59 63 59 Male/Female 1.3 0.8 1 0.8 1.0 0.8 Symptomatic 72% – 69% – 73% 82% Splenomegaly 50% 50% 19% 50% 35% 24% Hepatomegaly 34% 23% 1% 32% – 10% Adenopathy 13% 1% 3% 13% 5% 6% B symptoms – – 12% – – 7% Infections 38% 39% 15% 56% – 23% Rheumatoid arthritis 12% 28% 26% 36% 20% 17% Rheumatoid factor – 57% 61% 43% 48% 41% Antinuclear antibodies – 38% 44% 38% 48% 48% Autoimmune cytopenias – – 7% 9% 5% 7% Lymphocytosis 29% LGL > 4 × 109/L 52% 52% – – – 14% LGL 1–4 × 109/L 38% 40% – – – 50% LGL < 1 × 109/L 10% 8% – 7% – 36% Neutropenia Moderate (< 1.5 × 109/L) 64% 84% 74% – 52% 61% Severe (0.5 × 109/L) 7% 48% 40% 37% 41% 26% Anemia Any severity 25% 49% 51% 26% 89% 24% Severe (Hgb < 8 g/dL) 37% – 19% – 36% 7% Thrombocytopenia 9% 19% 20% 29% 36% 19% LGL marrow infiltration 67% 88% – 76% 83% 72% Hypergammaglobulinemia – 45% 5% 43% – 35% Monoclonal gammopathy – 45% 8% – – 10% Need for treatment 30% 73% 69% 33%... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Download the Access App: iOS | Android Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.