James A. Tarbox, MD

James Tarbox, MDWashington University in St. LouisCIS School in PIDManagement Dilemma

11 year old Caucasian girl Hypogammaglobulinemia for which she receivesIVIG every 2 weeks Low T cell count with poor T-cell function Hypoparathyroidism Pancytopenia Tetralogy of Fallot status post repair Chronic respiratory disease with bronchitis andlymphocytic interstitial pneumonitis in 2009treated with steroids

Right axillary LN biopsy in March 2010 showing noncaseatinggranulomas Negative work-up for CMV, Bartonella, fungal, AFB,aerobic cultures Worsening axillary, left superior mediastinal,prevascular and paratracheal lymphadenopathy Started on monthly infliximab (Remicade) inApril 2010 Recurrent fevers/chills, blood stream and portinfections though SOB improved

This case highlights the challenges of treating animmunodeficient patient withimmunosuppressive therapy for significantgranulomatous disease, recurrent fevers andchronic respiratory disease How to best proceed with the need to suppressthe immune system to treat her granulomatousdisease while balancing this with the risk ofserious infection related to immunodeficiency?

Sirolimus - inducing T cell tolerance Dr. Huttenlocher 1▪13F with CVID, chronic ITP, and granulomatous disease Rituximab- DiGeorge with ITP Dr. Cunningham-Rundles Low dose steroids and plaquenil Consider modifying IVIG regimen1. Simonson WT, Markovina S, Grossman WJ et al. AAAI 2009

Started on a low dose of corticosteroids (prednisolone3 mg/day) Later underwent three treatments with Rituximab Subsequent Chest CT showed marked intervaldecrease in size of mediastinal and retroperitoneallymphadenopathy Still has occasional fevers and chills, but appears to bedoing much better compared to 2 years ago