Evidence-based management of immune thrombocytopenia: ASH guideline update

Abstract

In 1996 and 2011, the American Society of Hematology (ASH) supported efforts to create guidelines for the diagnosis and management of patients with immune thrombocytopenia (ITP). These guidelines used different approaches to arrive at recommendations for testing and treatment. Despite differences in methodology, in both cases there was a paucity of randomized trials to inform recommendations. As data on the diagnosis and management of ITP expands, the ASH Committee on Quality is dedicated to maintaining updated guidelines representing recent evidence and guideline methodology. Here, we will review the updated ASH guidelines on ITP with a focus on recommendations with new understanding and future research to close knowledge gaps.

Combination of thrombopoietin receptor agonists, immunosuppressants and intravenous immunoglobulin as treatment of severe refractory immune thrombocytopenia in adults and children

“…Recently an increasing focus on the role of T cells in the pathogenesis of ITP has developed (Zufferey et al , ). CSA as single agent or in combination with prednisone has been recommended as second‐line treatment of refractory ITP(Neunert et al , ; Neunert & Cooper, )Romiplostim and eltrombopag have been validated as treatment of refractory ITP in large placebo‐controlled trials reporting complete or partial response in most patients (Bussel et al , ; Wong et al , ; Ghanima et al , ).…”

“…Primary immune thrombocytopenia (ITP) is characterized by immune‐mediated peripheral platelet destruction and suboptimal platelet production in the bone marrow affecting both children and adults. The pathogenesis of ITP is not fully understood, but anti‐platelet autoantibodies as well as autoreactive Th and Tc cells have been identified in ITP patients with active disease(Neunert et al , ; Zufferey et al , ; Neunert & Cooper, ).…”

Secondary Immune Thrombocytopenic Purpura in Renal Cell Cancer: A Paraneoplastic Syndrome

“…X-ray, ultrasound, computed tomography are the diagnostic tools of choice. There needs to be a high index of suspicion to screen patients with a new diagnosis of ITP for an underlying malignancy, especially if treatment with steroids and IVIG has proved unsuccessful [18].…”

Was sind die Indikationen für Rescue-Verfahren?

“…Die Gabe von IVIG kann in Analogie zur idiopathischen Immunthrombozytopenie (ITP) auch bei Patienten mit schwerer und therapierefraktärer Autoimmunthrombozytopenie im Rahmen eines SLE erwogen werden. Die Gabe von IVIG wird in Kombination mit GC bei akuten Blutungskomplikationen im Rahmen einer ITP empfohlen [23,49] und ist in dieser Indikation auch zugelassen. Auch vor operativen Eingriffen oder bei Schwangeren mit ITP kann ein Einsatz von IVIG erwogen werden, wenn GC nicht ausreichend wirksam waren oder vermieden werden müssen [73].…”