A critical appraisal of the evidence for the role of splenectomy in adults and children with ITP

Br J Haematol. 2018 Apr;181(2):183-195. doi: 10.1111/bjh.15090. Epub 2018 Feb 26.

Abstract

In primary chronic immune thrombocytopenia, long-term response to splenectomy, with 60% of patients enjoying a treatment-free life, is higher when compared with rituximab and similar to that with continuous thrombopoietin-receptor agonists (TPO-RA) administration. Splenectomy should continue to be offered to patients failing initial treatments in the absence of increased surgery-related risks. The higher lifelong safety concerns with splenectomy (increased risk of infection, shared in part with rituximab, and of thrombosis, in common with TPO-RA) and a mortality <1-2%, justify postponing surgery to the chronic phase, when spontaneous remissions are rarer. Patients failing initial treatment with corticosteroids/intravenous immunoglobulin may use TPO-RA (or rituximab in selected cases) as a bridge to surgery if they prefer to reconsider splenectomy later on, in case of no response, adverse effects or if sustained response after stopping TPO-RA is not attained. Special considerations apply in children aged ≤5 years, with splenectomy playing a marginal role. The recent approval of TPO-RA in children may represent a major advancement.

Keywords: immune thrombocytopenia; rituximab; splenectomy; thrombopoietin-receptor agonists.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Infections / drug therapy
  • Infections / etiology
  • Infections / immunology
  • Male
  • Postoperative Complications / immunology
  • Postoperative Complications / therapy*
  • Purpura, Thrombocytopenic, Idiopathic / immunology
  • Purpura, Thrombocytopenic, Idiopathic / surgery*
  • Receptors, Thrombopoietin / agonists
  • Receptors, Thrombopoietin / immunology
  • Risk Factors
  • Rituximab / adverse effects
  • Rituximab / therapeutic use
  • Splenectomy / adverse effects
  • Splenectomy / methods*
  • Thrombosis / etiology
  • Thrombosis / immunology
  • Thrombosis / therapy*

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous
  • Receptors, Thrombopoietin
  • MPL protein, human
  • Rituximab