number of IFN-γ and a similar number of IL-2 producing Tcells compared to control group. Conclusion: Our patientspresented a decreased number of T cells producing both Th1and Th2 cytokines. Despite the presence of 2 differentgroups in relation to cytokine production there was nocorrelation to the clinical features.

doi:10.1016/j.clim.2007.03.310

F.98 Immunomodulation by Cytokines in ChronicMucocutaneous Candidiasis Patients: Evaluation ofthe Production of T Cell Cytokines at a Single CellLevelMauricio Domingues Ferreira, MD, PhD, Department ofDermatology, University of São Paulo Medical School, SãoPaulo, Brazil, Alberto Jose da Silva Duarte, MD, PhD,Department of Dermatology, University of São PauloMedical School, São Paulo, Brazil, Dewton MoraesVasconcelos, MD, PhD, Department of Dermatology,University of São Paulo Medical School, São Paulo, Brazil,Tatiana Negri Santi-BSc, Department of Dermatology,University of São Paulo Medical School, São Paulo, Brazil,Anete S. Grumach, MD, PhD, Department of Dermatology,University of São Paulo Medical School, São Paulo, Brazil,Alexandre Almeida, MD, MSc, Department of Dermatology,University of São Paulo Medical School, São Paulo, Brazil

Background: Chronic mucocutaneous candidiasis (CMC)is a rare primary immune deficiency characterized bychronic or recurrent Candida infection in mucous mem-branes, nails and skin. Impaired cell mediated responseand dysregulation of the production of Th1 and Th2cytokines to Candida are important in CMC. We previouslyfound a lower number of IL-2, IL-10, IL-4 and IFN-γproducing T cells and its CD4+ and CD8+ subsets comparedto the control group when stimulated with Candida andtetanus toxoid. Objective: Modulate the T cell response byadding to the cell culture medium IL-2, or IL-12, or anti-IL-10. Methods: We studied the production of cytokines of 15patients with CMC and 13 controls stimulated by Candidaand tetanus toxoid. We evaluated IL-2, IL-4, IL-10 and IFN-γin total T cells, as well as CD4+ and CD8+ subsets by anintracellular cytokine flow cytometric assay. We tried toimmunomodulate with recombinant human IL-2, or IL-12, ora monoclonal antibody anti-IL-10. Results: We did not find asignificant response to the immunomodulation trials, butthere was a trend of improvement in the synthesis of IL-2from the CD4+ cells of patients when stimulated by Candidaand modulated by IL-2, IL-12 or anti-IL-10. Conclusion:There was no significant improvement by the immunomo-dulation by cytokines, but there was a tendency ofimprovement. It is possible that if we have used acombination of cytokines, the results could be better. Ourdata suggest a new possible way of treatment to patientswith CMC.

doi:10.1016/j.clim.2007.03.311

F.99 Hereditary Angiodema (HAE) in Brazil: Registryof 100 CasesAnete Sevciovic Grumach, Department of Dermatology,University of São Paulo, Brazil, São Paulo, Brazil, JorgeAndrade Pinto, Department of Pediatrics, FederalUniversity of Minas Gerais, Belo Horizonte, Alexandre PiresCorrea, BSc, Department of Dermatology, University ofSão Paulo, Brazil, São Paulo, Brazil, DewtonMoraes-Vasconcelos, MD, PhD, Department of Dermatology,University of São Paulo, Brazil, São Paulo, Brazil, RosemeireNavickas Constabtino-Silva, BSc, Department ofDermatology, University of São Paulo, Brazil, São Paulo,Brazil, Sollange Valle, Federal University of Rio de Janeiro,Rio de Janeiro, Eli Mansour, Department of Pediatrics,University of Campinas, Campinas, Maria Marluce Vilela,Department of Pediatrics, University of Campinas,Campinas, Ricardo Zollner, Clinical Medicine, University ofCampinas, Campinas, Alberto Jose da Silva Duarte,Professor, Department of Dermatology, University of SãoPaulo, Brazil, São Paulo, Brazil, Evandro Rivitti, Professor,Department of Dermatology, University of São Paulo, Brazil,São Paulo, Brazil

HAE is a primary quantitative or functional defect of C1inhibitor. Clinical manifestations include subcutaneous,respiratory and gastrointestinal edema. Asphyxia couldoccur in 25–40% of the non-treated cases. There was noregistry in Brazil until now. Objective: Report the clinical andlaboratorial characteristics of HAE in Brazilians. Methods:Collaborative work groups were established among specia-lized services. The following parameters were evaluated:gender, age, age of diagnosis, main clinical manifestations,time for diagnosis, triggering factor, treatment, familialhistory and C1 inhibitor and C4 levels and CH50. Laboratorialdiagnosis of C1 INH deficiency was confirmed in all patients.Results: HAE affected 67 F:33 M, 1–70 years of age and thefirst symptoms were reported during childhood in most of thecases. One episode of laryngeal edema was present in 17%and the triggering factors were trauma (31/100), stress (11/100), and menses (6/100). Familial history was positive in53/100. Conclusion: Familial history was determinant fordiagnosis in half of the cases. Late diagnosis had beenestablished but the first symptoms occurred during child-hood. HAE diagnosis is still not well recognized in ourcountry.

doi:10.1016/j.clim.2007.03.312

F.100 Autosomal Dominant Combined ImmuneDeficiency: A Unique Family with Absent B-Cells andDecreased T-CellsMichael Land, Physician, University of California, LosAngeles, Pediatrics, Los Angeles, CA, Raffi Tachdjian,Physician, University of California, Los Angeles, Pediatrics,Los Angeles, CA, Erina Lin, Physician, University ofCalifornia, Los Angeles, Pediatrics, Los Angeles, CA, RobertRoberts, Physician, University of California, Los Angeles,Pediatrics, Los Angeles, CA, Marc Riedl, Physician,University of California, Los Angeles, Department ofMedicine, Los Angeles, CA

S48 Abstracts

Rationale: We present a family of four individuals withimmunodeficiency. The father and daughter were initiallydiagnosed with Common Variable Immune Deficiency (CVID)and were found to have absent B cells and low T cells. Thefather’s brother and mother also had immunodeficiency butdied years ago. This may represent a unique form ofCombined Immunodeficiency.

Methods/Results: The father is a 35-year-old man with ahistory of CVID diagnosed at age 12 following multiple upperrespiratory infections. He was started on intravenousimmunoglobulin (IVIG) for hypogammaglobulinemia, butnever had any severe infections. His mother and olderbrother also received gamma globulin for presumed CVID,but both died from infections at age 36. He had one episodeof pneumonia at age 19, with occasional sinus infections. Histrough IgG level recently was 162, but increased to 724 withhigher dosing. His IgA was b7, IgM b0.5, IgE b0.1. Thepatient’s 3-year-old daughter has been receiving IVIG sinceage 8 months for hypogammaglobulinemia. She has a historyof sinusitis and has never been hospitalized. Her IgA is b7,IgM 6, IgE 0.1. Interestingly, both father and daughter haveundetectable B cells (CD19 b1%) and CD4 lymphopenia(father: 202, daughter: 608). Both patients also have lowCD8 counts (father: 162, daughter: 189). They have normalresponses to mitogens and antigens. Conclusion: This family,along with the deceased relatives, exhibits an unusualpattern of inheritance for B cell and T cell deficiencyconsistent with combined immunodeficiency, suggestingautosomal dominance. Further characterization and investi-gation of this disease are warranted.

doi:10.1016/j.clim.2007.03.313

F.101 Circulating Class-Switched Memory B Cellsare Markedly Reduced in Some Patients withSpecific Antibody Deficiency (SAD) and/or Mild IgGSubclass DeficiencyLily E. Leiva, Associate Professor, Louisiana State UniversityHealth Sciences Center and Children’s Hospital, Departmentof Pediatrics, New Orleans, LA, Kenneth Paris, AssistantProfessor, Louisiana State University Health Sciences Centerand Children’s Hospital, Department of Pediatrics, NewOrleans, LA, Hanh Monjure, Research Associate, LouisianaState University Health Sciences Center and Children’sHospital, Department of Pediatrics, New Orleans, LA,Ricardo U. Sorensen, Professor and Chair, Louisiana StateUniversity Health Sciences Center and Children’s Hospital,Department of Pediatrics, New Orleans, LA, Dana Minor,Research Technician, Research Institute for Children,Children’s Hospital, New Orleans, LA

Some patients with recurrent respiratory infections areaffected by specific antibody deficiency (SAD) and/or IgGsubclass deficiencies. In this study we investigated if theinability of patients with SAD and/or IgG subclass deficiency torespond to pneumococcal polysaccharide antigens (PP) wasassociated with reduced numbers of circulating class-switchedmemoryBcells.We studied7 childrenwith recurrent respiratoryinfections with SAD and/or mild IgG subclass deficiency and 8healthy controls. All patients were evaluated with total

immunoglobulin and IgG subclass concentrations. We measuredIgG and IgM anti-PPantibody levels by a standardized ELISA test.Purified PBMC were stained for the expression of CD27 and IgDon CD19+ B cells. Percentages of naïve (CD27NIgD+), non-switched memory (CD27+IgD+) and class-switched memory(CD27+IgDN) B cells were determined by flow cytometry.Results: In patients in whom IgG anti-PP antibody levels werelow, IgM levels were within the normal ranges for each of the 8serotypes tested. The B cell compartment in these patientsshowed amarked reduction in the percentage of class-switchedmemory B cells in 2 patients (6% and 2%, respectively) incomparison to the other 5 patients (range: 32%–90%) and tocontrols (range: 22%–87%). These results suggest that adeficientIgM to IgG antibody switch is associatedwith a lack of circulatingclass-switched memory B cells in some patients, and that thismay be the explanation for the failure of some patients torespond to PP. Studies are in progress to determine theprognostic implications of these differences.

doi:10.1016/j.clim.2007.03.314

F.102 HIV Treatment RevealsHypogammaglobulinemia in an Individual withCommon Variable Immune DeficiencyLulu Sun, Immunodeficiency Treatment Centre, McGillUniversity Health Centre, Montreal, QC, Canada, ChristosM. Tsoukas, Immunodeficiency Treatment Centre, McGillUniversity Health Centre, Montreal, QC, Canada

While common variable immune deficiency (CVID) is char-acterized by hypogammaglobulinemia, HIV-1 infection is asso-ciated with elevated immunoglobulin levels. There have beenfour CVID patients reported in the literature who recoveredantibody production after contracting HIV-1. Here we describean HIV patient with concomitant CVID whose immunoglobulinlevels dramatically decreased upon successful antiretroviraltherapy (ART), and investigate a plausible mechanism by whichHIV is able to modulate circulating immunoglobulin throughregulation of the B cell activation factor (BAFF)/BAFF-receptorpathway. Four cohorts were compared: our HIV/CVID patient,HIV patients, CVID patients, and healthy controls. Phenotypicand functional analyses of theTand B cells of these cohortswereperformed using flow cytometry, and BAFF serum levels weremeasured by ELISA. In addition to marked hypogammaglobuli-nemia, the patient has a complete defect of memory IgD+/−CD27+ CD19+ B cells thatwas not altered by ART, consistentwithsevere CVID. When viral load briefly increased, a transientnormalization of immunoglobulins was also observed. At thesame time, the percentage of BAFF-R+B cells was boosted fromlow to normal levels. Plasma BAFF levels were high compared tocontrols during viral suppression, due primarily to the lack ofmemory B cells. Finally, Tcell phenotypes remained abnormal,with a low CD4+ Tcell count that is characteristic of rigorous HIVdisease, and impaired in vitro cellular proliferation.Our findingssuggest that high HIV viraemia was required to maintain normalimmunoglobulin levels in the CVID patient, possibly throughupregulating BAFF-R.

doi:10.1016/j.clim.2007.03.315

S49Abstracts