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

被引：49

作者：

Scully, M. ^{[1
,2
,15
]}

Rayment, R. ^{[3
]}

Clark, A. ^{[4
]}

Westwood, J. P. ^{[5
]}

Cranfield, T. ^{[6
]}

Gooding, R. ^{[7
]}

Bagot, C. N. ^{[8
]}

Taylor, A. ^{[9
]}

Sankar, V. ^{[10
]}

Gale, D. ^{[11
]}

Dutt, T. ^{[12
]}

McIntyre, J. ^{[13
]}

Lester, W. ^{[14
]}

机构：

[1] Univ Coll London Hosp, Natl Inst Hlth Res, UCLH, Dept Haematol,Biomed Res Ctr, London, England

[2] Univ Coll London Hosp, Natl Inst Hlth Res, Biomed Res Ctr, Haematol Programme, London, England

[3] Univ Hosp Wales, Dept Haematol, Cardiff, Wales

[4] Univ Hosp Bristol NHS Fdn Trust, Dept Haematol, Bristol, England

[5] UCLH, Dept Haematol, London, England

[6] Portsmouth Hosp Univ NHS Trust, Dept Haematol, Portsmouth, England

[7] Belfast H&SC Trust, Belfast City Hosp, Haematol Dept, Belfast, North Ireland

[8] Glasgow Royal Infirm, Glasgow, Scotland

[9] Great Ormond St Hosp Children NHS Fdn Trust, London, England

[10] Liverpool Univ Hosp NHS Fdn Trust, Dept Crit Care Med, Liverpool, England

[11] UCL, Dept Renal Med, London, England

[12] Liverpool Univ Hosp NHS Fdn Trust, Liverpool, England

[13] TTPNetwork, Melbourn, England

[14] Univ Hosp Birmingham, Dept Haematol, Birmingham, England

[15] British Soc Haematol, 100 White Lion St, London N1 9PF, England

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2023年 / 203卷 / 04期

关键词：

HUS; plasma exchange; pregnancy; TTP; VON-WILLEBRAND-FACTOR; HEMOLYTIC-UREMIC SYNDROME; FACTOR-CLEAVING PROTEASE; SEVERE ADAMTS13 DEFICIENCY; FRESH-FROZEN PLASMA; SINGLE-CENTER; ANTI-ADAMTS13; ANTIBODIES; CLINICAL PRESENTATION; RECOMBINANT ADAMTS13; IGG ANTIBODIES;

D O I：

10.1111/bjh.19026

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：546 / 563

页数：18

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