Refractory thrombocytopenia in SLE patient with massive thigh hematoma and hematuria successfully treated with Rituximab: Case Report

Abstract

Background: Systemic Lupus Erythematosus is a multisystemic inflammatory disease with broad clinical presentation, 9.5% to 44.5% of patients with SLE have thrombocytopenia complication of different grades. No significant increase in risk of hemorrhage was found in patients with SLE with moderate thrombocytopenia, while those with severe thrombocytopenia frequently developed bleeding, which is associated with the activity of the disease and potentially life-threatening condition, if left untreated. Case presentation: We report a case of 35-year-old SLE male patient, presented with sudden painful large non-traumatic ecchymotic lesion on the right thigh extending to calf with macroscopic hematuria. At presentation, platelets count was 50 x 109/L with hemoglobin dropping from 10.3 to 6.9 g/dl. Anticardiolipin antibody and Antiphospholipid antibody were absent. Other factors including infection and visceral involvement were excluded. MRI of the thigh showed huge intramuscular hematoma extending to calf. Satisfactory control was partially achieved with platelet transfusion, pulse steroid, cellcept, IVIG (intravenous immunoglobulins) and consequently Rituximab. Our case describes refractory thrombocytopenia not responding to multiple agents including high dose steroids, cellcept, platelet transfusion, IVIG (intravenous immunoglobulins) and cyklokapron; however, it achieved its complete remission with Rituximab. Bleeding is not usual presentation with moderate thrombocytopenia in SLE patients and presumably played a role in prognosis. Conclusion: Our case report has demonstrated the efficacy of treating refractory thrombocytopenia resistant to steroids due to Systemic Lupus Erythematosus in whom other reasonable options have been exhausted.