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Case report
Severe primary refractory thrombotic thrombocytopenic purpura (TTP) in the post plasma exchange (PEX) and rituximab era
  1. Sowmya Goranta,
  2. Smit S Deliwala,
  3. Tarek Haykal and
  4. Ghassan Bachuwa
  1. Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, Michigan, USA
  1. Correspondence to Dr Ghassan Bachuwa; gbachuw2{at}


Acute acquired thrombotic thrombocytopenic purpura (TTP) requires prompt recognition and initiation of plasma exchange (PEX) therapy and immunosuppression. When PEX fails, mortality nears 100%, making finding an effective treatment crucial. Primary refractory TTP occurs when initial therapies fail or if exacerbations occur during PEX therapy, both signifying the need for treatment intensification to achieve clinical remission. Rituximab helps treat most of the refractory TTP cases, except those that are severely refractory. A paucity of studies guiding severely refractory TTP makes management arbitrary and individualised, highlighting the value of isolated reports. We present an extremely rare case of primary refractory TTP with an insufficient platelet response to numerous types of treatments, including emerging therapies such as caplacizumab, on the background of repeated PEX and immunosuppressive therapies.

  • thrombotic thrombocytopenic purpura
  • haematology (incl blood transfusion)
  • oncology
  • drugs and medicines

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  • Contributors SG: conception, design, acquisition of data and manuscript. SSD, TH and GB: conception, design, acquisition of data, edit and manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.