State of the art – how I manage immune thrombocytopenia

Abstract

The management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with many steroid-sparing agents and a reduction in the progression to splenectomy. Although this has improved clinical care, many therapeutic challenges remain. There is no diagnostic test, no biomarkers to direct treatment and few comparative studies to help management decisions. Development of up to date guidelines is difficult with little high-grade evidence. First line treatment continues to be steroids and intravenous immunoglobulins (IVIG) although both are often poorly tolerated and not curative. Common second line treatments include rituximab, immunosuppressive agents, such as azathioprine and mycophenolate mofetil, and the thrombopoietin receptor agonists romiplostim and eltrombopag. There are no comparative studies to decide between these agents and treatment is generally individualized, depending on comorbidity. Use of splenectomy has declined and is generally reserved for patients with chronic disease, although the exact position of splenectomy is subject to debate. Further understanding of the cause of disease in individual patients may help guide treatment. Randomized controlled studies of common treatments and novel treatments for refractory patients are urgently needed.

Újdonságok a gyermekkori immunthrombocytopenia kezelésében – 2017

“…Sok esetben a gyógyszerek adását fel lehet függeszteni és a THR-szám csökkenése esetén újraindí-tani (így például a költségek is csökkenthetők). Több -elsősorban amerikai -ajánlás már másodvonalban javasolja gyermekeknek a TPO-agonistákat [19,20]. Így a gyermekkori splenectomiák nagy része elkerülhető.…”

Altered circulating T follicular helper cells in patients with chronic immune thrombocytopenia

“…The incidence primary ITP accounts for 3.3/100,000 adults per year (3). Although recent progress on the development of thrombopoietin receptor agonists have changed the management of chronic disease (4), the detailed underlying mechanisms involved in the pathophysiology of cITP remain poorly understood.…”

Dapsone therapy for immune thrombocytopenic purpura: old but still unfamiliar

“…Accordingly, there are varieties of treatments corresponding to those pathophysiologic aspects. First line treatments intended to suppress the autoantibody production and interfere with removal of the opsonized platelets in RES include corticosteroid treatment and intravenous immunoglobulin or anti-D immunoglobulin [1]. If indicated, splenectomy is considered for refractory thrombocytopenias [1].…”

“…First line treatments intended to suppress the autoantibody production and interfere with removal of the opsonized platelets in RES include corticosteroid treatment and intravenous immunoglobulin or anti-D immunoglobulin [1]. If indicated, splenectomy is considered for refractory thrombocytopenias [1]. Options other than splenectomy include more nerve wrecking agents such as cyclophosphamide, cyclosporine A, mycophenolate mofetil [1].…”

“…If indicated, splenectomy is considered for refractory thrombocytopenias [1]. Options other than splenectomy include more nerve wrecking agents such as cyclophosphamide, cyclosporine A, mycophenolate mofetil [1]. Medical splenectomy like rituximab and thrombopoietin mimetics to stimulate thrombopoiesis are recently added therapies [1].…”