Oral Presentations - Federation of Clinical Immunology Societies

S44 Abstracts
had significant hepatosplenomegaly. Bone marrow biopsy
showed cytoplasmic inclusions granule and hemophagocytic
cells. For treatment, he received 3 courses of IVIG (4.5 g/kg
total). He was also given methlyprednisolone. He was then
placed on dexamethasone until the time of transplantation.
Over the course of several weeks, his mental status returned
to baseline, seizure activity ceased, hepatomegaly resolved,
and pancytopenia improved. Therefore his accelerated
phase had undergone remission. He then received a 10/10
match cord blood transplantation. The patient appeared to
engraft successfully following the transplantation and
routine infusions of IVIG were stopped. Approximately
15 months after transplantation, the patient was clinically
stable but serum IgG level was found to be 4000 mg/dL and
his CD 4/8 ratio was 0.55. Immunoelectrophoresis revealed
that he had developed a monoclonal IgG gammapathy. Bone
marrow and repeat RFLP studies are being done to further
investigate this abnormality.
doi:10.1016/j.clim.2007.03.300
F.88 Distribution, Infections, Treatments and
Molecular Analysis in a Large Cohort of Patients with
Primary Immunodeficiency Diseases (PIDD) in
Taiwan
Wen-I Lee, Assistant Professor, Immunodeficiency Diagnosis
and Research Institute, Chang Gung University and
Children’s Hospital, Taoyuan, China, Tang-Her Jaing,
Assistant Professor, Department of Pediatric Hematology
and Oncology, Chung Gung Children’s Hospital, Taoyuan,
China, Meng-Ying Hsieh, MD, Department of Pediatric
Neurology, Chang Gung University and Children’s Hospital,
Taoyuan, China, Syh-Jae Lin, Associated Professor,
Department of Pediatric Allergy, Immunology and
Rheumatology, Chang Gung University, Taoyuan, China,
Jing-Long Huang, Professor, Department of Pediatric
Allergy, Immunology and Rheumatology, Chang Gung
University, Taoyuan, China, Li-Chen Chen, Assistant
Professor, Department of Pediatric Allergy, Immunology and
Rheumatology, Chang Gung University, Taoyuan, China,
Ming-Ling Kuo, Professor, Department of Microbiology and
Immunology, Graduate Institute of Basic Medical Sciences,
Taoyuan, China
One hundred and twenty-four patients (from 120
families) to date diagnosed as primary immunodeficiency
diseases were enrolled from five tertiary medical centers.
The distribution by an update eight categories showed 45
patients (13 females/32 males; 36.3%) with predominant
antibody deficiencies, 27 patients (6/21; 21.8%) with T and
B cell immunodeficiency, 25 patients (9/16; 20.2%) with
congenital defects of phagocyte, 25 patients (4/21; 20.2%)
with other well-defined immunodeficiency syndromes, one
boy (0.8%) with disease in immune deregulation (Chediak–
Higashi syndrome) and another with complement 3 deficiency.
None had defects in innate immunity or auto
inflammatory disorders. Pseudomonas and Salmonella spp.
were the two most identified microorganisms in septicemia
(39.7%; 27/68 episodes). Twenty-three patients (18.5%) had
mortality. Stem cell transplantation succeeded in 7 of 12
patients. In addition to nine patients with DiGerge
syndrome recognized by FISH, direct sequencing identified
12 unique mutations from 20 families, reflecting distinct
Taiwan geography, although a selection bias may exist. The
study is still ongoing.
doi:10.1016/j.clim.2007.03.301
F.89 Expression of FoxP3 and CD25 in T Cells is
Altered in ICOS-/- Common Variable
Immunodeficiency (CVID) patients
Hans Hartmut Peter, Head of Department,University
Hospital Freiburg, Freiburg, Germany, Julia Horn, Assistant
Doctor, University Hospital Freiburg, Department of Clinical
Immunology and Rheumatology, Freiburg, Germany, Ulrich
Salzer, Senior Postdoctoral Fellow, University Hospital
Freiburg, Department of Clinical Immunology and
Rheumatology, Freiburg, Germany, Jennifer Birmelin, MTA,
Royal Free Hospital Department of Immunology, London,
England, Klaus Warnatz, Consultant, University Hospital
Freiburg, Department of Clinical Immunology and
Rheumatology, Freiburg, Germany, Michael Schlesier, Head
of Laboratory, University Hospital Freiburg, Department of
Clinical Immunolgy and Rheumatology, Freiburg,
Germany, Bodo Grimbacher, Consultant, Royal Free
Hospital, Department of Immunology,
London, England
Objective: FoxP3+CD25+ high regulatory T cells play a
central role in suppressing immune responses to self
antigens. To determine whether CVID patients have an
altered frequency of FoxP3+CD25+ high regulatory T cells
(Tregs), we studied this cell type in 48 CVID patients
including four ICOS−/− CVID patients from the Freiburg
CVID cohort by FACS analysis. Results: First, we found that
the frequency of FoxP3+CD25+ Tregs (median +/− SD; p>=
Wilcoxon rank sum test) was not significantly different in
CVID patients (1.9% +/− 0.9%, n = 48; p>= 0.05) as compared
to healthy controls (3.41% +/− 0.9%, n = 17). Second, CVID
patients with autoimmune phenomena (n = 17 of 48) do not
have lower frequencies of Tregs than CVID patients without
autoimmune phenomena except one patient who presents
with immune thrombocytopenia (ITP). Interestingly, ICOS-/-
CVID patients (n = 4 of 48) have normal or increased Treg
frequencies. In addition, in ICOS−/− CVID patients an
increased FoxP3+CD25− T cell population in comparison to
other CVID patients and healthy controls is observed.
However, culturing ICOS−/− CD4+ T cells with IL-2 for 72 h
results in decreased FoxP3 expression (16.4% to 4.68%) of
CD25- T cells due to activation. Conclusion: Our data suggest
that CVID patients with autoimmune phenomena, do not
have reduced numbers of Treg cells. However, ICOS−/− CVID
patients who have no autoimmune phenomena show an
increased FoxP3+CD25+ T cell population and an increased
Foxp3 expression of CD25− T cells.
doi:10.1016/j.clim.2007.03.302