presence of neoplastic disease (p b 0.01).Receiver-operator characteristic curve (ROC)analysis using a cut-off of ≤ 1.0 for SMB showed a sensitivity of 100% and specificity of 80% in predicting malignancy in CVID patients. Decrease in the SMB compartment has been previously reported to be associated with splenomegaly, autoimmunity and granulomatous disease; however, this is the first report of the association of reduced SMB with malignancy in CVID. In summary, evaluation of a cohort of CVID patients at a tertiary-care center with detailed B cell analysis provides additional evidence for the clinical relevance of SMB in the etio-pathogenesis of this disease. doi:10.1016/j.clim.2009.03.401 S.15. A Whole Blood Assay to Assess the ex vivo Responsiveness of Blood pDC, BDCA1+and BDCA3+ Dendritic Cell Subsets to TLR Ligands Antoine Neel 1 , Marie Rimbert 2 , Cécile Braudeau 2 , Régis Josien 1 . 1 INSERM, Nantes Cedex 1, France; 2 CHU Nantes, France, France Blood dendritic cells (DC) encompass plasmacytoid DC (pDC) and several subsets of so-called conventional DC (cDC): BDCA1+DC (CD1c), BDCA3+DC (CD141, thrombomodulin) and CD16+DC which also express CD14. Here we set up an whole blood assay to assess DC responsiveness to various TLR ligands. 200 ul of heparanized blood were incubated with various TLR ligands in the presence of brefeldin A and then stained with CD45, Lin cocktail, HLA-DR, CD11c and CD123 mAb followed by intracellular cytokine staining (IL- 12p40, TNF-α and IFNα). Importantly, samples must be processed in less than 6 h to avoid a major decrease in DC cytokine production. cDC were separated with BDCA1 and BDCA3 mAb whereas CD16+DC were excluded from this analysis. We demonstrate that, as expected, cDC (CD11c+ CD123-) responded to to TLR 1, 2, 3, 4, 5, 6 and 8 but not 7 and 9 and produce IL12-p40 and TNFα but not IFNα whereas pDC (CD11c-CD123+) respond only to TLR 7 and 9 by producing IFNα and TNF-α but not IL-12 although we could consistently detect 1-2% of IL-12p40 producing pDC upon TLR7/8 triggering. BDCA1+DC responded to TLR2/6, 3, 4, 5, 7/8 and slightly to TLR1/2 and produced IL-12p40 and TNF-α. In contrast, BDCA3+DC responsiveness appeared restricted to TLR1/2 (IL-12p40 but not TNF-α) and 3 (IL-12p40 and TNF-α) with a limited responsiveness to TLR9 ligands (IL-12 but not TNF-α). Of note, a higher frequency of BDCA3+DC produced IL-12p40 as compared to BDCA1+cells. Therefore this short and rather simple assay appears suitable to assess the responsiveness of blood DC subsets to TLR ligands in patients. doi:10.1016/j.clim.2009.03.402 S.16. Register of the Patients with Primary Immunodeficiency Disorders in Macedonia Mironska Kristina. University Clinic for Children Diseases, Skopje, Macedonia Aim: Pediatric patients with Primary Immunodeficiency Disorders diagnosed in Macedonia are presented. Material and Methods: Patients with PID are find out between the investigated children, suspected for immunodeficiency dis- orders, referred and hospitalized at the department of pediatric immunology, University Clinic for Children`s Diseases, the unique pediatric tertiary hospital in Macedonia, between 1976 and 2009 year. Most of the patients had confirmed diagnosis for PID on molecular level in referent immunology laboratories. Results: Different PID are diagnosed in (51) patients. With primary antibody deficiencies are (25) patients: (15) have selective IgA deficiency, (6) have X-linked agamma- globulinemia, (2) have common variable immunodeficiency and (2) are IgG2 subclass deficient. Another (26) patients have: Di George anomaly (5), X-linked SCID (3), chronic mucocutaneus candidiasis (2), Wiskott-Aldrich syndrome (2), ataxia-telean- giectasia (3), and X-linked lymphoproliferative syndrome (1). One (1) case is with chronic granulomatous disease and (4) cases are with hereditary angioedema. Two (2) patients are with congenital neutropenia. Three patients are with anti- inflammatory disorders, classified as a separate group in PID classification: (1) case od hyperimmunoglobulinaemia D (HIDS), and (2) cases with Familial Mediterranean Fever. Those patients who are receiving monthly immunoglobulin replacement therapy are healthy, with controlled infections. The oldest patient with X-linked agammaglobulinemia is 27 years old now. (9) of the patients with PID had died. One X-linked SCID patient, after the BMT, is in good condition. There was no one case with developed malignancy or autoimmunity. Conclusion: We pre- sent register of (51) investigated, diagnosed and cured PID patients in Macedonia. doi:10.1016/j.clim.2009.03.403 S.17. Identifying Diagnostic Markers and Studying Etiologic Factors of Inflammatory Bowel Diseases Using a High-throughput Proteomic Approach Xiaoxiao Li 1 , James LeBlanc 1 , David Elashoff 1 , Laura Presley 2 , James Borneman 2 , Lee Goodglick 1 , Jonathan Braun 1 . 1 University of California, Los Angeles, CA; 2 University of California, Riverside, CA The objectives of our study are to identify diagnostic markers for Inflammatory Bowel Diseases (IBDs), define the features of the proteome at different colonic locations and under different physiologic conditions, and investigate the host and microbial interactome at the mucosal interface. We collected bowel-rinse samples from IBD patients and normal individuals, and analyzed each sample using high-throughput surface enhanced laser desorption/ionization-time of flight (SELDI-TOF) mass spectrometry (MS). SELDI data preprocessing was done in CiphergenExpress ® . The resulting spectra were further analyzed in R. Peaks were compared between groups to search for biomarker candidates, whereas the Permutation analysis was used to evaluate the featured differences between groups of samples. Hierarchical clustering heat maps were generated in Cluster3.0 and visualized in TreeView. We also used oligonucleotide fingerprinting of ribosomal RNA genes (OFRG) to study the microbes in the bowel-rinse samples. We have thus far identified several potential IBD biomarkers which are confirmed by western blotting. The heat- S137 Abstracts