Refractory acquired thrombotic thrombocytopenic purpura in a patient with sickle cell trait successfully treated with caplacizumab

Hematology. 2021 Dec;26(1):590-593. doi: 10.1080/16078454.2021.1959984.

Abstract

Methods: We report a case of a 20-year-old Nigerian male who presented with acquired thrombotic thrombocytopenic purpura (aTTP) and sickle cell trait. The coexistence of published cases of TTP and sickle cell hemoglobinopathies is rare.Results: Despite the initial treatment with plasma exchange and glucocorticoids, our patient relapsed and also required caplacizumab which resulted in successful remission.Discussion: We conclude by reviewing the cases of TTP in patients with sickle cell hemoglobinopathies and review how vaso-occlusive crises with multiorgan injury can mimic TTP.Conclusion: Ours is the first published case of aTTP with confirmed ADAMTS13 autoantibodies in a patient with a sickle cell hemoglobinopathy and contributes to the literature on the successful use of caplacizumab in clinical practice.

Keywords: ADAMTS13; Caplacizumab; TTP; acquired thrombotic thrombocytopenic purpura; hemoglobinopathy; plasma exchange; sickle cell; vaso-occlusive crisis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Fibrinolytic Agents / therapeutic use*
  • Humans
  • Male
  • Plasma Exchange
  • Purpura, Thrombotic Thrombocytopenic / complications*
  • Purpura, Thrombotic Thrombocytopenic / drug therapy*
  • Purpura, Thrombotic Thrombocytopenic / therapy
  • Sickle Cell Trait / complications*
  • Single-Domain Antibodies / therapeutic use*
  • Young Adult

Substances

  • Fibrinolytic Agents
  • Single-Domain Antibodies
  • caplacizumab

Supplementary concepts

  • Thrombotic thrombocytopenic purpura, acquired