One hundred and twenty-four patients (from 120 families)diagnosed as primary immunodeficiency diseases were en-rolled from five tertiary medical centers. The distribution byan update eight categories showed 45 patients (13 femalea/32 males; 36.3%) with ‘‘predominant antibody deficiencies’’,27 patients (6/21; 21.8%) with ‘‘T-and B-cell immuno-deficiency’’, 25 patients (9/16; 20.2%)with ‘‘congenitaldefects of phagocyte’’, 25 patients (4/21; 20.2%) with ‘‘otherwell-defined immunodeficiency syndromes’’, one boy (0.8%)with ‘‘disease in immune dysregulation’’ (Chediak-Higashisyndrome) and another with ‘‘complement 3 deficiency’’.None had ‘‘defects in innate immunity’’ or ‘‘autoinflamma-tory disorders’’. Pseudomonas and Salmonella spp. were thetwomost identifiedmicroorganisms in septicemia (39.7%; 27/68 episodes). Twenty-three patients (18.5%) had mortality.Stem cell transplantation succeeded in 7 of 12 patients. Inaddition to 9 patients with DiGerge syndrome recognized byFISH, direct sequencing identified 12 unique mutations from20 families, reflecting distinct Taiwan geography, although aselection bias may exist.

doi:10.1016/j.clim.2006.04.336

Sa.105. Granulomatous AmoebicMeningoencephalitis (GAM) After NonmyeloablativeAllotransplantation and Chronic Graft-Versue-HostDisease.Robert Nelson,1 Bryan Schneider,1 Mitchell Goldman,2

Ayana Barbarin,3 Michael Robertson,1 Stephen Allen.31Internal Medicine, Division of Hematology/Oncology,Hematology Malignancy Program and Stem CellTransplantation, Indianapolis, IN; 2Medicine, Division ofInfectious Diseases, Indiana University School of Medicine,Indianapolis, IN; 3Department of Pathology and LaboratoryMedicine, Indiana University School of Medicine,Indianapolis, IN.

GAM is a rare infectious disease (est. b300—400 casesworld-wide) most often diagnosed at autopsy. A 52-year-old man with recurrent small lymphocytic lymphoma whounderwent nonmyeloablative matched related transplantafter conditioning with monoclonal MEDI-507/cyclophopha-mide conditioning and cyclosporine GVHD prophylaxis. Heexperienced extensive chronic GVHD and on post day +177was admitted with fever and mental status changes. Hedeveloped progressive signs of altered mental status,headache, stiff neck and obtundation and persistentfevers. During hospitalization, infectious diseases andneurological evaluations were extensive; in spite oftreatment with a number of antimicrobial agents, thepatient continued to deteriorate, and died with signs ofworsening neurologic illness. Autopsy did not show evi-dence of tuberculosis, leukemia or malignant lymphoma.Multiple coronal sections of the fixed brain revealedmultifocal lesions of hemorrhagic necrosis involving theright orbital cortex, bilateral cingulated gyri (anterior),left caudate, the right basal ganglia, right hippocampusand the base of the pons, and there were focal areas ofmeningeal involvement, consistent with granulomatous

amoebic meningoencephalitis. Electron microscopic studiesof the amoebic cysts reveal double cyst walls typical ofAcanthamoeba species. The patient’s death was caused bygranulomatous amoebic meningoencephalitis involving thesmall free-living water/soil Acanthamoeba. Acanthamoe-bae are now considered emerging agents of infectiousdiseases, particularly of Acanthamoeba keratitis which is avision-threatening infectious disease that is not uncommonin Indiana. This patient appears to be the first patientdiagnosed with granulomatous amoebic meningoencepha-litis in the history of the Indiana University MedicalCenter.

doi:10.1016/j.clim.2006.04.337

Sa.106. Histopathological Aspects of Acute-FormParacoccidioidomycosis in an IL-12 ReceptorDeficient Patient.Dewton Moraes-Vasconcelos,1 Anete Grumach,1

Mirian Sotto,1 Carla Pagliari,1 Joao Mello,2

Maria Elisa Andrade,2 Augusto Yamaguti,3

Alberto Jose Duarte.1 1Dermatology, University of Sao PauloMedical School, Sao Paulo, Brazil; 2Allergy and Immunology,Hospital of the Public Servants ‘‘Francisco Morato deOliveira’’, Sao Paulo, Brazil; 3Infectology, Hospital of thePublic Servants ‘‘Francisco Morato de Oliveira’’, Sao Paulo,Brazil.

Paracoccidioidomycosis is a deep mycosis caused byParacoccidioides brasiliensis, endemic in certain regions ofSouth America. PCM is a disease with twomain clinical forms:acute form (AF) and chronic form (CF). Over the last years,the molecular basis of Mendelian susceptibility to mycobac-terial disease (MIM209950) was determined in a number ofpatients. We showed that AF PCM could be related to adeficiency of the IL-12/23 receptor. DLS, 18-years old male,presented fever, diffuse lymphadenopathy and hepatosple-nomegaly, being diagnosed an AF of PCM. The patient had hadBCGitis and disseminated salmonellosis, suggesting a defectof the IL-12/23-IFN-g axis, proved by molecular biology. Thehistology of the lymph nodes showed well-formed granulo-mas with occasional giant cells and central necrosis. TheGrocott staining showed several yeasts with multiple exo-sporulation. The staining of immune cells showed thepresence of a CD4+ T-cells palisade. The number of CD8+ T-cells was much less representative. Macrophages were insidethe central areas of the lesions, some of them filled by fungalforms. The staining for cytokines showed no IL-4. TNF-a wasobserved in few cells, and IFN-g in scarcely distributed cells.This case report emphasizes the critical importance of IL-12 /IFN-g axis in the development of functional granulomata inPCM patients. There is an interesting clinical-pathologicalrelationship between the clinical features, the evolution ofthe PCM and the histopathological aspects, with a reasonablywell-defined granuloma, containing the fungi. It is interest-ing to stress that due to the fact that the patient didnTtexpress any functional IL-12/23 Rh1 the production of IFN-gmust be dependent on other cytokines. These points areimportant to understand the benign features of the PCM in

AbstractsS142