Severe primary refractory thrombotic thrombocytopenic purpura (TTP) in the post plasma exchange (PEX) and rituximab era.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Goranta, S; Deliwala, SS; Haykal, T; Bachuwa, G

Published Date

  • June 11, 2020

Published In

Volume / Issue

  • 13 / 6

Start / End Page

  • e234091 -

PubMed ID

  • 32532908

Pubmed Central ID

  • PMC7295375

Electronic International Standard Serial Number (EISSN)

  • 1757-790X

International Standard Serial Number (ISSN)

  • 1757-790X

Digital Object Identifier (DOI)

  • 10.1136/bcr-2019-234091


  • eng