Anemia Associated with Increased IL-6 and IL-8 Levels Predicts for Worse Progression-Free Survival in T-Cell and NK-Cell Large Granular Lymphocyte (LGL) Leukemia but Is Improved by Treatment with Cyclophosphamide

• Published in: Blood 2012-11, Vol.120 (21), p.1261-1261
• Main Authors: Portell, Craig A., Visconte, Valeria, Clemente, Michael J., Afable, Manuel G., Tabarroki, Ali, Traina, Fabiola, Liu, Yang, Hamilton, Betty K., Jarez, Andres, Bupathi, Manoj, Horwitz, Leonard J., Lichtin, Alan E., Advani, Anjali, Sekeres, Mikkael A., Maciejewski, Jaroslaw P., Tiu, Ramon V.
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V120.21.1261.1261

Abstract 1261 LGL leukemia is a rare bone marrow failure disorder characterized by a clonal expansion of terminally differentiated T- or NK-cells with large cytoplasmic granules. Cytopenias of one or multiple lineages are the dominant phenotype, though lymphocytosis without cytopenias can also be present. Goals of therapy are minimizing cytopenias. Currently used immunosuppressive and chemotherapeutic agents have produced variable clinical responses. Predictors of response to therapy and overall outcomes have not been well studied. We reviewed 92 LGL patients (pts, T-LGL=79, NK-LGL=13) seen at Cleveland Clinic from January 2000 to July 2012 treated with various therapeutic agents. Overall (OS) and Progression-Free (PFS) Survival and Time to Next Treatment (TTNT) were estimated using the Kaplan-Meier method. The first three treatments for LGL pts were evaluated with TTNT; observation was not considered a treatment for this analysis. Risk factors for PFS and OS after initial treatment (including those who were never treated) were identified using Cox proportional hazard analysis. Clinical factors evaluated at time of diagnosis include age, sex, neutropenia (absolute neutrophil count ≤1000 per μL), anemia (hemoglobin ≤10gm/dL or transfusion requirement), lymphocytosis (absolute lymphocyte count >4000 per μL), lymphopenia (absolute lymphocyte count ≤1000 per μL), thrombocytopenia (platelet count ≤100 per μL), transfusion dependence (any red-cell transfusion requirement around time of diagnosis), history of B-cell dyscrasia and history of an autoimmune disease. Clinicopathologic risk factors included type of LGL leukemia (T- vs. NK-cell), detection of a monoclonal protein, number of Vβ clones measured by flow cytometry, and T-cell clonality by T-cell receptor (TCR)-γ rearrangement. P values of ≤0.05 were considered statistically significant. The median age of the cohort was 63 years (range: 16–85). 61% were male. Treatments (n=129) included cyclophosphamide (n=31), cyclosporine (n=33), methotrexate (n=25), alemtuzumab (n=12), chemotherapy (n=7), others (n=21), and supportive care or observation (n=28). Median TTNT was 18 months (range