P492 Resolution of factor XI (FXI) deficiency in refractory Ulcerative Colitis (UC) after surgery: A case report
A. Angelakopoulou1, M. Mathias2, A. Windsor3, S. McCartney4, F. Kiparissi*1, 4
1Great Ormond Street Hospital, Paediatric Gastroenterology, London, United Kingdom, 2Great Ormond Street Hospital, Haematology, London, United Kingdom, 3University College London Hospitals NHS Foundation Trust, Department of Colorectal Surgery, London, United Kingdom, 4University College London Hospitals NHS Foundation Trust, Department of Gastroenterology, London, United Kingdom
Coagulation abnormalities in acute inflammatory conditions such as sepsis are well recognised. Chronic inflammatory conditions, such as Inflammatory Bowel Disease, have the potential to induce a coagulopathic or procoagulant state with potential devastating consequences.
We present the case of a 14 year old male with severe UC, FXI deficiency and an acute cerebrovascular event. We report resolution of FXI deficiency post colectomy and restorative surgery for UC.
The patient presented with two-week history of headache, vomiting, altered consciousness and left leg weakness, one year after diagnosis of severe UC refractory to combination immunosuppressive therapy. MRI changes were in keeping with Right Middle Cerebral Artery ischaemic stroke. There was no radiological evidence of systemic vasculitis, APTT was prolonged (67.3s; N:26-38s), FXI (13 IU/dl; N:50-150), Thrombocytopenia post stroke (lowest 76) with subsequent gradual recovery. Lifelong aspirin anticoagulation was commenced. No neurological sequelae were observed. UC remained uncontrolled despite escalation to non-conventional immunosuppression.
Patient underwent subtotal colectomy and ileostomy formation with good results. Lowest FXI levels documented pre-operatively (8 IU/dl) and treated with FXI concentrate. Congenital FXI deficiency was excluded by patient genetic and parental FXI testing. Spontaneous normalisation of FXI within 2 years of surgery (FXI>50 IU/dl) with no further complications.
Clinicians should recognise the haematological implications of UC, especially in cases refractory to medical therapy. In our patient, FXI deficiency was secondary to uncontrollable gastrointestinal inflammation and normalised after definitive surgical treatment. FXI deficiency was a likely contributing factor in this patient's stroke.