A case of ITP that'll make you go blue in the face

Abstract

Immune Thrombocytopenia Purpura (ITP) is a relatively common paediatric presentation (3-8 cases per 100,000). It is usually a benign condition with spontaneous resolution occurring in 80% of cases within 6 months. Chronic ITP is only rarely associated with severe symptoms and the mainstay of treatment remains supportive care. We describe an unusual case of chronic relapsing symptomatic ITP whose refractory nature challenged conventional treatment options and triggered reflection on ITP management. 3 year old AS presented acutely with fever and extensive petechiae, following a 6 month history of easy bruising. She was initially treated for meningococcaemia but a diagnosis of ITP was subsequently made based on the findings of an isolated thrombocytopenia (with an otherwise normal blood film) in the absence of hepatospenomegaly or lymphadenopathy. Initial management was conservative. However, symptomatic cutaneous and mucosal bleeding continued associated with thrombocytopenia (<20−×10 9/L). Active management was therefore considered at this stage. Lymphoproliferative, autoimmune and post-viral causes were excluded and empirical treatments were given for symptom relief. Prednisolone was ineffective and intravenous immunoglobulin provided only short-lived improvement. As symptoms persisted, a second line agent rituximab was given, achieving 10 months of remission. Following further relapse, a second course of rituximab achieved remission prior to performing a laparoscopic splenectomy. Despite this she relapsed again 6 months later. Treatment options were again reviewed and oral dapsone was started. This was ineffective and stopped due to symptomatic methaemoglobinaemia (22.6%). It was subsequently restarted at a lower dose but she again developed methaemoglobinaemia and therefore dapsone was discontinued and a yellow card was completed to highlight this dramatic reaction. Following national discussions, she was enrolled for a trial of a TPO-receptor agonist. However, whilst awaiting entry into this trial, she developed spontaneous remission – 2.5 years following initial diagnosis. Chronic ITP is rarely associated with significant thrombocytopenia and symptomatology. Medical and surgical treatments can effect remission but therapeutic cure is unusual. It is therefore essential to weigh up the risk balance when considering treatment. This case clearly highlights this point and prompts consideration of the indications for medical treatment in chronic ITP and of where the risk-benefit balance lies in complex cases such as this.