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Ref Number = PITIKA-ASPR0203
INTRAVENOUS IMMUNOGLOBULIN (IVIG) THERAPY IN PEDIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) WITH PERSISTENT THROMBOCYTOPENIA: A CASE REPORT
Ganda Ilmana, Teny Tjitra Sari, Nia Kurniati, Dina Muktiarti
Objective
To report effectiveness of IVIg in children with persistent thrombocytopenia caused by SLE.

Case
A 4-year-old girl came to our hospital with recurrent epistaxis, gum bleeding and petechie since 1 week before admission. She was diagnosed with aplastic anemia one year before and had no symptoms for about 10 months. The patient was also on tuberculosis treatment. At admission, the platelet level was as low as 1000/mm3 with Hb of 9.4 g/dl. She also had malar rash and oral ulcers. Evaluation for SLE was performed with positive ANA (titer > 1/1000, homogen pattern, anti ds-DNA of 893.6 IU/ml, C3 64 mg/dl and C4 13 mg/dl). Diagnosis of SLE was established. Serial platelet transfusion every 24 hours was administered but the platelet did not improve (ranged from 0 to 3000/mm3). Evaluation of bone marrow revealed amegakariocyte. Oral methylprednisolonone was started with dose of 2 mg/kg/day, however after 2 weeks the response still was unfavorable, so mycophenolate acid was added and then we put her on pulse methylprednisolone (30 mg/kg/day for 3 days). She also received treatment for otitis media and dental infections. The platelet level was persistently low (0-3000/mm3). She was then received 2 times apheresis platelet transfusion. Platelet level was increased to 6000/mm3 but bleeding was still observed. During hospitalization she developed sepsis, diarrhea with positive stool culture of Klebsiella pneumonia, positive CMV and EBV PCR. We decided to give her high dose intravenous immunoglobulin (2 g/kg) instead of other immunosuppressant in this condition. Platelet level increased to 22.000 /mm3 and no bleeding was observed. Treatment with oral methylprednisolone and mycophenolate acid was continued.

Conclusion
High dose IVIg can be considered in children with SLE who do not respond to standard treatment especially patient with infections
Keywords: VIG, pediatric, systemic lupus erythematosus, therapy
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