A middle-aged man diagnosed with a drug reaction with eosinophilia and systemic symptom (DRESS) syndrome, secondary to phenytoin use, subsequently developed thrombotic thrombocytopaenic purpura. The patient improved with steroids and plasmapheresis. Their diagnosis can be challenging, and an early recognition and treatment are critical owing to their high mortality rates. Both diseases are thought to be of an autoimmune origin, and a potential relationship between them led to the consideration of the DRESS syndrome as an aetiology for thrombotic thrombocytopaenic purpura in this case. We concluded that two possibilities exist: some type of antibody developed during the clinical presentation of DRESS syndrome and subsequently resulted in an inhibition of a disintegrin and metalloproteinase with a thrombospondin type-1 motif, member 13 (ADAMTS13) leading to thrombotic thrombocytopaenic purpura, or perhaps this patient's autoimmune predisposition to thrombotic thrombocytopaenic purpura contributed to the drug reaction.
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http://dx.doi.org/10.1136/bcr-2012-007558 | DOI Listing |
BMJ Case Rep
April 2024
Brookdale Department of Geriatrics and Palliative Medicine, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
South Afr J HIV Med
October 2023
Department of Internal Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
Background: Thrombotic thrombocytopaenia purpura (TTP) is a rare disorder which carries a high mortality. HIV is an important cause of TTP.
Objectives: We assessed the presentation and response to plasma exchange (PEX) by HIV status.
Nephrol Dial Transplant
February 2023
Tokushima University Hospital, Tokushima, Japan.
Background: Eculizumab was approved for atypical haemolytic uraemic syndrome (aHUS) in Japan in 2013. Post-marketing surveillance (PMS) was mandated by regulatory authorities to assess the safety and effectiveness of eculizumab in patients with aHUS in a real-world setting.
Methods: Paediatric patients in the PMS cohort who were <18 years of age at the first administration of eculizumab and diagnosed with aHUS [excluding Shiga toxin-producing Escherichia coli HUS, thrombotic thrombocytopaenic purpura and secondary thrombotic microangiopathy (TMA)] were included in the effectiveness and safety analysis.
Nephrol Dial Transplant
June 2022
Department of Medicine III - Nephrology, Ordensklinikum Linz Elisabethinen, Linz, Austria.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that is caused by severe ADAMTS-13 deficiency. Immune-mediated TTP develops due to autoantibodies against ADAMTS-13, whereas congenital TTP is caused by mutations in the ADAMTS13 gene. Diagnostic possibilities and treatment options in TTP have emerged in recent years, which prompted the International Society on Thrombosis and Haemostasis (ISTH) to publish clinical practice guidelines for the diagnosis and treatment of TTP in 2020.
View Article and Find Full Text PDFBMJ Case Rep
September 2021
Department of Critical Care, University College London Hospitals NHS Foundation Trust, London, UK.
A 40-year-old man with no cardiac history presented with central chest pain 8 days after receiving the ChAdOx1 nCov-19 vaccine against COVID-19. Initial blood tests demonstrated a thrombocytopaenia (24×10 μg/L) and a raised d-dimer (>110 000 μg/L), and he was urgently transferred to our tertiary referral central for suspected vaccine-induced immune thrombocytopaenia and thrombosis (VITT). He developed dynamic ischaemic electrocardiographic changes with ST elevation, a troponin of 3185 ng/L, and regional wall motion abnormalities.
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