Refractory immune thrombocytopenic purpura (ITP) after accessory splenectomy: A case report and literature review

Clin Hemorheol Microcirc. 2023;85(2):189-194. doi: 10.3233/CH-231881.

Abstract

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a platelet count of less than 100 x 109 /L, resulting from antibody-mediated platelet destruction. Treatment for ITP typically involves steroids, and intravenous immunoglobulins (IVIG) can be added. Splenectomy is performed in cases with refractory ITP. Rituximab can suppress immunity but has limited efficacy in ITP cases. Herein, we present a rare case of a 30-year-old female who was first diagnosed with ITP and underwent a splenectomy two years later. However, seven years after surgery, she was presented with symptoms of ITP. A splenic scan showed an accessory spleen in the spleen bed, for which she underwent accessory spleen removal surgery. Her laboratory tests three days post-operation showed a rise in platelet count and hence was discharged a few days later. The patient had recurrent attacks of ITP even after the removal of the normal and accessory spleen, suggesting that accessory spleen removal may not always be an effective treatment for ITP. The patient eventually died. While splenectomy is a common treatment for ITP, it may not always be effective in all cases, and other treatments such as bone marrow transplantation may be necessary.

Keywords: Immune thrombocytopenic purpura (ITP); intravenous immune globulins (IVIG); rituximab; splenectomy; steroid.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Adult
  • Female
  • Humans
  • Platelet Count
  • Purpura, Thrombocytopenic, Idiopathic* / surgery
  • Splenectomy / methods
  • Splenic Diseases*