PB1250 - Haemostatic Management of a Moderate Haemophilia B (MHB) Patient with respiratory failure through VV-Extracorporeal Membrane Oxygenation (ECMO)

Tuesday, June 25, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

Maria Rita Peralta, MD

Haemostasis&Thrombosis Consultant
St Thomas Hospital & Royal Brompton Hospital, London, UK
LONDON, England, United Kingdom

Co-Author(s)

Jj

JohnMichael james

Haematology Registrar
Guy’s & St Thomas’ NHS Foundation Trust
London, England, United Kingdom
Uzma Faruqi

Haemostasis&thrombosis consultant
Guy’s & St Thomas’ NHS Foundation Trust
London, England, United Kingdom
PL

Pu-Lin Luo

Clinical Lead for Haemostasis and Thrombosis
Guy’s & St Thomas’ NHS Foundation Trust
London, England, United Kingdom
Gerard Dolan

Haemophilia Director
Guy’s & St Thomas’ NHS Foundation Trust
London, England, United Kingdom
LN

Lucy Neave

Consultant Haematologist
Guy's and St Thomas's NHS Foundation Trust
London, England, United Kingdom
LC

Luigi Camporota

Consultant in Critical Care and Extracorporeal Membrane Oxygenation (ECMO)
Guy’s & St Thomas’ NHS Foundation Trust
London, England, United Kingdom
AD

Anicee Danaee

Consultant Haematologist- Haemostasis and Thrombosis & Transfusion
Guy’s & St Thomas’ NHS Foundation Trust
London, England, United Kingdom
HL

HoPuiJeff Lam

Haematology Registrar
Guy's and St Thomas' NHS Foundation Trust London, UK
London, England, United Kingdom
KB

Karen Breen

Consultant Haematologist
Guy's and St Thomas' NHS Foundation Trust London, UK
London, England, United Kingdom
FM

Francesca Momiglliano

Haematology Registrar
Guy's and St Thomas' NHS Foundation Trust London, UK
London, England, United Kingdom
AM

Aine N McCormick

Principal Biomedical Scientist
Synnovis analytics, Guy's and St Thomas' NHS Foundation Trust London, UK
London, England, United Kingdom
AD

Andrew J Doyle

Consultant Haematologist
Haemostasis & Thrombosis Centre, St Thomas’ Hospital, London, UK
London, England, United Kingdom

Background: Extracorporeal Membrane Oxygenation (ECMO) is a form of organ support offered to patients with severe but potentially reversible respiratory failure. Bleeding and thrombotic events are common complications. The haemostatic management of patients with bleeding disorders, is especially challenging due to their intrinsic high bleeding risk.

Aims: To report the haemostatic management of a patient with moderate haemophilia B (MHB) who received veno-venous (VV) ECMO for severe respiratory failure secondary to aspiration pneumonia

Methods: Case report: A 41-year old male with MHB (baseline FIX 2 IU), developed an acute hypoxemic, hypercapnic respiratory failure from an aspiration pneumonia following a laparotomy for acute perforated appendicitis. We report, factor IX (FIX) replacement and anticoagulation strategy, from ECMO implantation to lung recovery and ECMO decannulation.
Assays: FIX measurement was performed using one-stage clotting assay (3 dilutions). Unfractionated heparin UFH was monitored using chromogenic anti-Xa activity.

Results: FIX concentrate (Alprolix) was initially administered as a bolus dose pre-ECMO (levels 50-100IU/dL) and UFH was started (targeting an anti-Xa 0.3-0.5U/mL). The ECMO run was 32 days, during which he also required bilateral chest drain insertion for bilateral pneumothoraces and an abdominal drain for an intra-abdominal collection. He received daily FIX replacement, on average 5000 iu daily (weight 97 kg) maintaining a mean trough of 49 IU/L and a mean peak of 82IU/L. He had a good clinical outcome with no major bleeding complications and was discharged from ICU with no organ support. A non–occlusive cannula-associated thrombus was the only thrombotic complication.

Conclusion(s): This experience shows that ECMO may be successfully undertaken in patients with haemophilia supported by careful factor replacement and multidisciplinary management. There are few data in the literature on such procedures in bleeding disorders and sharing experience through registers would be a valuable initiative.