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  • Severe refractory warm autoimmune haemolytic anaemia after the SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA) managed with emergency splenectomy and complement inhibition with eculizumab

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Case Reports: Rare disease
Severe refractory warm autoimmune haemolytic anaemia after the SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA) managed with emergency splenectomy and complement inhibition with eculizumab
  1. Emma Marguerite Jackson1,
  2. Simon Harper2,
  3. Gwilym J Webb3 and
  4. Will Thomas4
  1. 1Clinical School, University of Cambridge, Cambridge, UK
  2. 2Hepatopancreaticobiliary Surgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  3. 3Department of hepatology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  4. 4Department of haematology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  1. Correspondence to Dr Will Thomas; w.thomas1{at}nhs.net

Abstract

A male in his teens with a history of liver transplant for biliary atresia (aged 2 years) and autoimmune haemolytic anaemia (AIHA, aged 6 years) presented with jaundice, dark urine, fatigue and chest discomfort that began 48 hours after the first dose of SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA). Investigations revealed a warm AIHA picture. Over 4 weeks the patient developed life-threatening anaemia culminating in haemoglobin of 35 g/L (after transfusion), lactate dehydrogenase of 1293 units/L and bilirubin of 228 µmol/L, refractory to standard treatment with corticosteroids and rituximab. An emergency splenectomy was performed that slowed haemolysis but did not completely ameliorate it. Eculizumab, a terminal complement pathway inhibitor, was initiated to arrest intravascular haemolysis and showed a favourable response. AIHA is rare but described after the SARS-CoV-2 Pfizer-BioNTech vaccine. This case highlights the rare complication of AIHA, the use of emergency splenectomy for disease control, and the use of eculizumab.

  • Haematology (incl blood transfusion)
  • Transplantation

https://doi.org/10.1136/bcr-2022-250774

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    Footnotes

    • Twitter @liver

    • Contributors EJ and WT wrote the manuscript. SH and GW provided clinical care and critically appraised 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 WT has received speakers fees from Pfizer, Bayer, Takeda, CSL Behring, Alexion, Portola, Sanofi, Sobi, NovoNordisk and advisory board fees from Daiichi Sankyo, Ablynx, Sanofi, Takeda, Grifols, LFB Biopharmaceuticals and Takeda. EJ, GW and SH have no competing interests.

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