319 Outside the Bubble: An Atypical Presentation of Severe Com-bined Immunodeficiency Disease (SCIDs)

D. G. DeMerell, J. M. El-Dahr; Tulane University School of Medicine,New Orleans, LA.RATIONALE: SCIDs is a pediatric emergency which usually presents inthe first few months of life with recurrent infections, diarrhea, and failureto thrive. Most infants with SCIDs are lymphopenic from birth. The Tcells, when present, fail to proliferate in response to antigens and mito-gens. Patients can have varying numbers of B and NK cells depending onthe specific mutation.CLINICAL COURSE: We describe an 12 m/o child who had been rel-atively healthy except for recurrent wheezing and milk intolerance. Hisoriginal lymphocyte enumeration revealed an ALC of 3655, CD3511(14%), CD4 241(6.6%) and CD8 73(2%). He had normal numbers ofB and NK cells with low but positive response to mitogens (PHA,PWM, Con A) and antigens (tetanus and candida). Immunoglobulin lev-els were normal with protective titers to protein and polysaccharide anti-gens. All of the lymphocytes were from the patient and not of maternalorigin. Originally, the low T cell number (with normal function) wasattributed to high dose steroids given to the patient. Over the next yearof life, the patient developed recurrent infections, multiple warts and adrop in growth rate. Upon retesting at 22 months of age, his T cells didnot respond to mitogen or antigen stimulation. He had become IgM defi-cient and lost protective titers to diptheria and pneumococcus duringthis time as well.CONCLUSION: We present an unusual patient with as yet unclassifiedSCIDs who was born with low T cell numbers but normal function whichwaned over time along with loss of B cell function.

320 Idiopathic CD4+ Lymphopenia in Conjuction With AlopeciaAreata: A Case Report

J. H. C. Friesen; University of Pittsburgh, Pittsburgh, PA.RATIONALE: To present a patient manifesting both alopecia areata andidiopathic CD4+ lymphopenia.METHODS: The patient is a 51yo female who has had alopecia areatafor eight years and arthritis for five years. A baseline CD4 count wasobtained in anticipation of alefacept treatment. Her CD4 count was 71with a CD4/CD8 ratio of 0.08. The patient has a benign infectious histo-ry over 35 years except for recurrent herpetic cold sores. No illnesses haverequired hospitalization except pneumonia at age seven. She has not hadproblems with thrush, fingernail infections or dermatitis. Her medicationsat workup included Vioxx, Fosamax, prn Valtrex and vitamin/mineral sup-plements.RESULTS: Her CD4+ count has been confirmed with multiple tests overfive months (range 50-98). HIV has been negative by PCR and ELISA.CMV Ab was negative. Mitogen stimulation was normal. A workup forautoimmune SLE was negative on multiple occasions (negative ANA,Smith, RNP, RF). The patient was placed on prophylactic Bactrim andremains asymptomatic.CONCLUSIONS: CD4+ lymphopenia has become synonymous withHIV infection. It is important to remember other causes in the differential.Idiopathic CD4+ lymphopenia is a rare condition with a prognosis thatvaries from benign to fatal. An association between alopecia areata andCD4 lymphopenia has been previously reported although not to thisseverity.

321 The Impact of Pneumococcal Vaccines in Adult Patients WithSinopulmonary Infections and Low Pneumococcal AntibodyTiters—Pilot Observation

L. Yao, P. Kumar; Allergy & Immunology, LSUMC, New Orleans, LA.RATIONALE: The studies on the impact of pneumococcal vaccines inrecurrent respiratory infections in patients with low pneumococcal anti-body titers were performed only in children. This pilot project is focusedon adult patients.METHODS: Four patients with recurrent sinopulmonary infections andlow pneumococcal antibody titers but normal immunoglobulins werestudied. All of the patients received pneumococcal polysaccharide vac-cines. Repeat titers were obtained 4 weeks after the vaccines. Threepatients demonstrated three - fold increases or above 1.5�g/ml in morethan 70 % of the serotypes of pneumococcal titers, which was consid-ered a good response. The fourth patient, who failed to respond to pneu-mococcal polysaccharide vaccine, did respond to pneumococcal conju-gate vaccine. The patients were followed every 3 months up to a year.RESULTS: Significant clinical improvements had already been noted in 2out of the 4 patients. One patient with a history of sinusitis with 6 to 7 sinusinfections per year in the previous 5 years had only one episode 6 monthsafter the vaccine was given. Another patient had 2 episodes of pneumoniaand numerous sinus infections 4 years prior to the vaccine. This patient hashad no evidence of pneumonia or sinusitis 1 year after the vaccination.CONCLUSIONS: The observation suggested that adult patients with lowpneumococcal antibody titers and recurrent respiratory infections may showserological and clinical improvements after use of pneumococcal vaccines.

S80 Abstracts J ALLERGY CLIN IMMUNOL

FEBRUARY 2005

SU

ND

AY

322 Caseating Granulomatous Disease in Common Variable

Immunodeficiency Treated With InfliximabA. Z. Hatab, Z. K. Ballas; Internal Medicine, University of Iowa Hospi-tals and Clinics and VA medical Center, Iowa City, IA.RATIONALE: Non-caseating granulomas have been reported to occur in5.4%-10% of common variable immunodeficiency (CVID) patients. Caseat-ing granulomas, on the other hand, have been only rarely described. We reporta patient with caseating granulomas successfully treated with infliximab.CASE REPORT: A 31-year-old white male, with CVID since age 4 (com-plicated by splenomegaly and thrombocytopenia, and maintained on IVIGinfusions), presented with fever and massive lymphadenopathy in axillae,groins, chest, abdomen and pelvis. Several biopsies showed caseating granu-lomas. No infectious organisms, including atypical mycobacteria, were iden-tified despite culture of several tissues including bone marrow. Steroids andincreasing IVIG infusions resulted in a significant but incomplete response.Six months later, he developed multiple splenic abscesses measuring up to 4cm in diameter. He also developed an enlarging, thick, oval skin plaque overhis back, which, by biopsy, showed a focal granulomatous response. A trial ofRituximab resulted in a transient response and a 4 cm decrease in the size ofthe spleen. Fever, lymphadenopathy and the skin plaque continued to worsen.He was started on infliximab infusions. Following the third infusion, theplaque was almost completely healed. The lymphadenopathy and splenicabscesses, by CT, were decreased by at least 50%.CONCLUSIONS: TNF-� is believed to be essential for the maintenanceof granulomas. Blockade of TNF, especially by infliximab, has resulted inreactivation of granulomatous infections. We have utilized the ability ofinfliximab to reverse granuloma formation to treat granulomatous diseaseassociated with CVID.