117 Portuguese Journal of Pediatrics CASE REPORT Abstract Pediatric systemic lupus erythematosus is a rare auto- immune disease that accounts for 10% of all cases of systemic lupus erythematosus. Isolated cytopenia as the only presentaton of systemic lupus erythematosus is rare in children. An 8-year-old female with an unremark- able personal and family history was admited to our pediatric rheumatology department due to refractory chronic thrombocytopenia afer having received pulses of methylprednisolone, polyvalent immune globulin, ant-D immune globulin and eltrombopag. Laboratory tests revealed thrombocytopenia (< 10 x 10 9 cells/L), positve antnuclear antbodies and ant-double stranded DNA antbodies, consumpton of complement and a positve Coombs test, allowing us to establish the diagnosis of systemic lupus erythematosus. She received pulses of methylprednisolone for three days, followed by oral cortcoid, associated with azathioprine, with clinical and laboratorial improvement. The presence of refractory thrombocytopenia highlights the possibility of systemic lupus erythematosus when other diseases, mainly infec- tous or hemato-oncological diseases, are excluded. Keywords: Child; Lupus Erythematosus, Systemic/compli- catons; Lupus Erythematosus, Systemic/diagnosis; Throm- bocytopenia/diagnosis; Thrombocytopenia/therapy Introduction Systemic lupus erythematosus (SLE) is a chronic autoim- mune disease, 1-3 which can afect several organs and sys- tems. It is more common among women of childbearing age. 1-3 Only 10%-20% of the reported cases develop dur- ing childhood. 4 Clinical manifestatons may vary and are nonspecifc, with hematological abnormalites (anemia, leukopenia, and thrombocytopenia) being described in more than half of the patents. 1,2 Isolated thrombocy- topenia is relatvely rare as the inital presentaton of a child with SLE. Therefore, the diagnosis of immune thrombocytopenia is common and, thereby, delayed diagnosis and inappropriate therapy may occur. 5 Case Report An 8-year-old female, with unremarkable past medical and family history, was admited to the rheumatology depart - ment because of a persistent decreased platelet count. She reported easy bruising for 10 months, initally involving the lower limbs with progression to the abdo- men. No gross bleeding was reported, such as gingival bleeding, epistaxis, hematuria, or rectal bleeding. There was no recent history of infectons, vaccinatons, or traveling, and she denied the use of any new drugs. Six months later, she was evaluated at the emergency department because of bruising and petechiae, with predominant involvement of the lower limbs. No other relevant abnormalites were present on physical exam- inaton. Laboratory tests showed thrombocytopenia (platelets 21 x 10 9 cells/L), without any other hemato- logical abnormality. She was hospitalized and received intravenous methylprednisolone at a dose of 30 mg/kg/ day for three consecutve days, followed by oral predni- solone at a dose of 1 mg/kg/day. A slight improvement of thrombocytopenia (platelets 34 x 10 9 cells/L) was documented. A direct Coombs test was positve and antnuclear antbodies (ANA) and ant-double stranded DNA (ant-dsDNA) were negatve. Bone marrow exam- inaton was consistent with immune thrombocytope- nia. She completed 15 days of oral prednisolone with tapering. At the hematology department, due to the worsening of thrombocytopenia (platelets 10 x 10 9 cells/L), she received polyvalent immunoglobulin, with a partal but not sustained improvement. Fifeen days later, she received another administraton, associated with ant-D immunoglobulin. Consequently to persistent severe thrombocytopenia, with no response to diferent treat- Sofia Helena Ferreira 1 , Ana Catarina Carvalho 2 , Teresa Rocha 3 , Fátima Ferreira 4 , Iva Brito 3 Port J Pediatr 2019;50:117-21 DOI: htps://doi.org/10.25754/pjp.2019.14284 Refractory Thrombocytopenia in Children: A Case Report 1. Pediatrics Department, São João Hospital Center, Oporto, Portugal 2. Pediatrics Department, Tâmega e Sousa Hospital Center, Penafel, Portugal 3. Rheumatology Department, São João Hospital Center, Oporto, Portugal 4. Hematology Department, São João Hospital Center, Oporto, Portugal Corresponding Author Sofa Helena Ferreira Hospital de São João, Alameda Prof. Hernâni Monteiro, 4200-319 Porto, Portugal sofahferreira@gmail.com Received: 03/05/2018 | Accepted: 10/12/2018