Near-complete vision recovery from bilateral serous retinal detachment associated with thrombotic thrombocytopenic purpura

BMJ Case Rep. 2022 Feb 7;15(2):e244030. doi: 10.1136/bcr-2021-244030.

Abstract

Ocular manifestations of thrombotic thrombocytopenic purpura (TTP) are uncommon, and bilateral retinal detachment is a rare presentation of TTP. We report a rare case of bilateral retinal detachment from underlying TTP in a patient presenting with vision loss. A 56-year-old man presented with a 4-day history of bilateral vision loss. Bilateral serous retinal detachment was confirmed using dilated ophthalmoscope examination. Laboratory results were significant for severe thrombocytopenia, peripheral smear revealed numerous schistocytes and ADAMTS13 activity of less than 1%. The patient was treated with plasma exchange (PLEX), prednisone, rituximab and caplacizumab. This case report highlights that prompt treatment of TTP with PLEX, prednisone, rituximab and caplacizumab could result in significant vision recovery.

Keywords: haematology (incl blood transfusion); retina.

Publication types

  • Case Reports

MeSH terms

  • ADAMTS13 Protein
  • Humans
  • Male
  • Middle Aged
  • Myopia*
  • Plasma Exchange
  • Purpura, Thrombotic Thrombocytopenic* / complications
  • Purpura, Thrombotic Thrombocytopenic* / diagnosis
  • Purpura, Thrombotic Thrombocytopenic* / therapy
  • Retinal Detachment* / diagnosis
  • Retinal Detachment* / etiology
  • Rituximab / therapeutic use

Substances

  • Rituximab
  • ADAMTS13 Protein