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Haematology
Thrombotic thrombocytopenic purpura in a patient with adult-onset Still’s disease: report of a rare entity
  1. Kirsten Maddox1,
  2. Anil Ananthaneni2,
  3. Neelay Patel2 and
  4. Poornima Ramadas2
  1. 1Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA
  2. 2Hematology/Oncology, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA
  1. Correspondence to Dr Anil Ananthaneni; anil_kumarans{at}yahoo.co.in

Abstract

Adult-onset Still’s disease (AOSD) is a rare, systemic inflammatory disorder characterised by daily fever, arthritis, a salmon-pink rash and leucocytosis. Thrombotic thrombocytopenic purpura (TTP) is included in the class of thrombotic microangiopathies and manifests clinically as microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and ischaemic tissue injury secondary to microthrombi. TTP is caused by either an autoimmune, congenital or idiopathic deficiency of ADAMTS13 and carries a high mortality rate. TTP can be seen in patients diagnosed with other rheumatologic conditions, with systemic lupus erythematosus being the most common, but is rarely seen in AOSD. Permanent complication rates from TTP in the setting of AOSD have been reported to be >50%. This case study aims to report one of the rare instances of TTP presenting in a patient with known AOSD to broaden the knowledge base regarding these rare coexisting pathologies.

  • Autoimmunity
  • Rheumatology
  • Pathology
  • General practice/family medicine

https://doi.org/10.1136/bcr-2024-260691

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    Footnotes

    • X @Ananth_MD

    • Contributors KM, AA and NP were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms and critical revision for important intellectual content. AA and PR gave final approval of the 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.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

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