OC 24.4 - Factor XIII levels correlate with fibrinogen concentrations in patients with venous malformations and chronic disseminated intravascular coagulopathy

Sunday, July 10, 2022

3:30 PM – 3:45 PM

Location: ExCel Center, ICC Capital Suite Room 14-16

Presentation Format: In-Person

Presenting Author(s)

NF

Nick Fordham

Haematology SpR
Great Ormond Street Hospital for Children
London, England, United Kingdom

Co-Author(s)

JC

James Clark

Haematology SpR
Great Ormond Street Hospital for Children
London, England, United Kingdom
AT

Alice Taylor

Haematology Consultant
Great Ormond Street Hospital for Children
London, England, United Kingdom
KS

Keith Sibson

Haematology Consultant
Great Ormond Street Hospital for Children
London, England, United Kingdom
LS

Lea Solman

Dermatology Consultant
Great Ormond Street Hospital for Children
London, England, United Kingdom
MG

Mary Glover

Dermatology Consultant
Great Ormond Street Hospital for Children
London, England, United Kingdom
MM

Mary Mathias

Haemophilia Centre Director
Great Ormond Street Hospital for Children, NHS Foundation Trust
London, England, United Kingdom

Background: Patients with slow flow venous malformations (VM) have a coagulopathy which can worsen with age. This is characterised by disseminated intravascular coagulopathy (DIC) and heralded by elevated D-dimers, but severe cases can lead to depletion of fibrinogen and significant haemorrhage. Little data exists on extended coagulation parameters in this cohort of patients.

Aims: The identification of clinically important coagulation abnormalities, to guide management of surgery and haemorrhage in VM patients.

Methods: A single-centre retrospective data review on all patients attending the VM service between June 2014 and June 2021 with factor XIII assays and basic coagulation screens assessed.

Results: 84 patients with VMs were identified. Where assessed, the majority (48/77) of patients had elevation of D-dimers at initial review, but the prothrombin time (PT) and activated partial thromboplastin time (APTT) were abnormal in only 8 patients. Extended PT and APTT based factor assays revealed two patients had a mild reduction in factor V, common in DIC. Fibrinogen concentration was decreased in ~24% (20/84) of VM patients. Thrombocytopenia ( < 150x109/L) was rare (5/84 patients) and predicted for a worsening coagulopathy. Almost all patients (15/16) with a fibrinogen concentration of < 1.5g/L had a low factor XIII level, some profound (range 8-62), whereas a fibrinogen >1.5g/L predicted for a normal factor XIII.

Conclusion(s): Our data suggests that acquired factor XIII deficiency is more common and severe in patients with VMs (15/84) than previously described, and clinicians should be aware of this. We suggest that all patients with VMs should have a basic coagulation screen assessed, including PT, APTT, D-dimers and fibrinogen concentration. Where the coagulation profile is normal, little is gained from extended factor screening, however, a factor XIII level should be measured in all patients with a low fibrinogen.