Syndecan-1 (sCD138) levels in chronic lymphocytic leukemia: clinical and hematological correlations

BACKGROUND: Syndecan-1 (sCD138) has recently been suggested to predict the clinical course of early-stage chronic lymphocytic leukemia (CLL), but few studies have been reported. This study assessed the role of syndecan-1 in the prognosis of patients with CLL and its correlation with other prognostic markers. METHODS: This prospective study was performed in the hematology department of an Indian tertiary care center, over nineteen months (Jun. 2009–Jan. 2011). Forty-nine new patients with CLL presented during this period and were included. Twenty age- and gender-matched healthy patients served as controls, and six patients with multiple myeloma were included as positive controls. Baseline serum syndecan-1 concentrations were measured for all patients at presentation using ELISA (Diaclone, Besancon, France). At baseline, patients were divided into low (N=10), intermediate (N=18) and high (N=21) risk cohorts. Serum syndecan-1 levels in these patient subgroups were compared with clinical and laboratory parameters. RESULTS: The median syndecan-1 level in patients with CLL (73.32 ng/mL, range, 28.71–268.0 ng/mL) was marginally higher than that in healthy patients (63.10 ng/mL, range, 55.0–75.11 ng/mL). At presentation, syndecan-1 levels in patients with CLL correlated strongly with symptomatic disease (cytopenias, P=0.004) and higher clinical stage (Rai stage III and IV, P=0.001) markers and poorly with β2-microglobulin level (P=0.270), diffuse BM infiltration (P=0.882), and surrogate mutation status markers (CD 38, P=0.174 and ZAP-70, P=0.459). Syndecan-1 levels dichotomized by the median value were higher with progressive disease markers, e.g. shorter lymphocyte doubling time (LDT, P=0.015) and increased treatment (P=0.099). CONCLUSION: In CLL, serum syndecan-1 (sCD138) levels at presentation correlate with disease burden, and higher baseline levels may predict early treatment.

Genotypic influence of alpha-deletions on the phenotype of Indian sickle cell anemia patients / 대한혈액학회지

BACKGROUND: Some reports have shown that co-inheritance of alpha-thalassemia and sickle cell disease improves hematological parameters and results in a relatively mild clinical picture for patients; however, the exact molecular basis and clinical significance of the interaction between alpha-thalassemia and sickle cell disease in India has not yet been described. There is little agreement on the clinical effects of alpha-thalassemia on the phenotype of sickle cell disease. METHODS: Complete blood count and red cell indices were measured by an automated cell analyzer. Quantitative assessment of hemoglobin variants HbF, HbA, HbA2, and HbS was performed by high performance liquid chromatography (HPLC). DNA extraction was performed using the phenol-chloroform method, and molecular study for common alpha-deletions was done by gap-PCR. RESULTS: Out of 60 sickle cell anemia patients, the alpha-thalassemia genotype was found in 18 patients. Three patients had the triplicated alpha-genotype (Anti alpha-3.7 kb), and the remaining patients did not have alpha-deletions. This study indicates that patients with co-existing alpha-thalassemia and sickle cell disease had a mild phenotype, significantly improved hematological parameters, and fewer blood transfusions than the patients with sickle cell anemia without co-existing alpha-deletions. CONCLUSION: Co-existence of alpha-thalassemia and sickle cell anemia has significant effects on the phenotype of Indian sickle cell patients.