A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies

M Scully¹, R Rayment², A Clark³, J P Westwood⁴, T Cranfield⁵, R Gooding⁶, C N Bagot⁷, A Taylor⁸, V Sankar⁹, D Gale¹⁰, T Dutt¹¹, J McIntyre¹², W Lester¹³; BSH Committee

Affiliations

¹ Department of Haematology, UCLH and Haematology Programme, University College London Hospitals Biomedical Research Centre, National Institute for Health Research, London, UK.
² Department of Haematology, University Hospital of Wales, Cardiff, UK.
³ Department of Haematology, University Hospitals Bristol NHS Foundation Trust, Bristol, UK.
⁴ Department of Haematology, UCLH, London, UK.
⁵ Department of Haematology, Portsmouth Hospitals University NHS Trust, Portsmouth, UK.
⁶ Haematology Department, Belfast City Hospital, Belfast H&SC Trust, Belfast, UK.
⁷ Glasgow Royal Infirmary, Glasgow, UK.
⁸ Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
⁹ Department of Critical Care Medicine, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK.
¹⁰ Department of Renal Medicine, University College London, London, UK.
¹¹ Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK.
¹² TTPNetwork, London, UK.
¹³ Department of Haematology, University Hospitals Birmingham, Birmingham, UK.

PMID: 37586700
DOI: 10.1111/bjh.19026

A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies

M Scully et al. Br J Haematol. 2023 Nov.

. 2023 Nov;203(4):546-563.

doi: 10.1111/bjh.19026. Epub 2023 Aug 16.

Authors

M Scully¹, R Rayment², A Clark³, J P Westwood⁴, T Cranfield⁵, R Gooding⁶, C N Bagot⁷, A Taylor⁸, V Sankar⁹, D Gale¹⁰, T Dutt¹¹, J McIntyre¹², W Lester¹³; BSH Committee

Affiliations

¹ Department of Haematology, UCLH and Haematology Programme, University College London Hospitals Biomedical Research Centre, National Institute for Health Research, London, UK.
² Department of Haematology, University Hospital of Wales, Cardiff, UK.
³ Department of Haematology, University Hospitals Bristol NHS Foundation Trust, Bristol, UK.
⁴ Department of Haematology, UCLH, London, UK.
⁵ Department of Haematology, Portsmouth Hospitals University NHS Trust, Portsmouth, UK.
⁶ Haematology Department, Belfast City Hospital, Belfast H&SC Trust, Belfast, UK.
⁷ Glasgow Royal Infirmary, Glasgow, UK.
⁸ Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
⁹ Department of Critical Care Medicine, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK.
¹⁰ Department of Renal Medicine, University College London, London, UK.
¹¹ Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK.
¹² TTPNetwork, London, UK.
¹³ Department of Haematology, University Hospitals Birmingham, Birmingham, UK.

PMID: 37586700
DOI: 10.1111/bjh.19026

Abstract

The objective of this guideline is to provide healthcare professionals with clear, up-to-date and practical guidance on the management of thrombotic thrombocytopenic purpura (TTP) and related thrombotic microangiopathies (TMAs), including complement-mediated haemolytic uraemic syndrome (CM HUS); these are defined by thrombocytopenia, microangiopathic haemolytic anaemia (MAHA) and small vessel thrombosis. Within England, all TTP cases should be managed within designated regional centres as per NHSE commissioning for highly specialised services.

Keywords: HUS; TTP; plasma exchange; pregnancy.

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References

REFERENCES

1. Cuker A, Cataland SR, Coppo P, de la Rubia J, Friedman KD, George JN, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021;137(14):1855-1861.
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1. Zheng X, Chung D, Takayama TK, Majerus EM, Sadler JE, Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem. 2001;276(44):41059-41063.
1. Furlan M, Robles R, Lämmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996;87(10):4223-4234.
1. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998;339(22):1578-1584.

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A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies

Affiliations

A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies

Authors

Affiliations

Abstract

References

REFERENCES

MeSH terms

LinkOut - more resources

Full Text Sources