PB0240 - Possible Autoimmune Acquired Factor 13 deficiency : Tailoring management in a resource constrained setting in India, A Case Report.

Background: Congenital deficiency of FXIII is an extremely rare disorder and acquired FXIII deficiency is even rarer. Acquired FXIII deficiency can be secondary to autoimmune conditions and non-immune conditions. Autoimmune acquired Factor XIII deficiency (AAXIII/13D) is rarer with only 93 reported cases in literature to date and present with subcutaneous or intramuscular spontaneous bleeding. Given the rarity of this condition, evidence-based recommendations do not exist and further more management becomes difficult in LMIC.

Aims: We present an otherwise healthy 69-year-old male, who presented with recurrent intramuscular hematomas from 3 months.

Methods: The basic haemostasis study and the coagulation factors were within normal ranges. Bearing in mind the nature of the bleed, we considered a FXIII deficiency, that was confirmed (FXIII-9.9%). An acquired FXIII deficiency was suspected since the patient did not report a personal or family history of bleeding. Non-immune causes were ruled out. Testing for plasma autoantibodies against FXIII could not be carried out due to non-availability. So, a diagnosis of Possible AAXIII/13D was made as per the ISTH/SSC Criterion 2015.

Results: Haemostatic treatment with fresh frozen plasma (15 mL/kg) and tranexamic acid was administered during his intramuscular bleeding in left anterior compartment of thigh (Figure 1). Factor XIII concentrate was not available. Post-FFP transfusion, factor XIII level was 14.90%. As evidence based recommendations do not exist for this condition, the treatment options: immunosuppression (steroids, azathioprine), rituximab and IVIg were discussed. He received prednisolone but continued to have spontaneous intramuscular hematomas

Conclusion(s): In resource constrained setting management has to be tailored according to the available resources. AAXIII/13 D has major differences when compared to congenital FXIII deficiency: bleeding does not correlate with factor levels, prophylactic FFP transfusions have limited role and immunosuppression is required. There is need for better collaboration among centres worldwide for formulation of treatment recommendations in setting with variable resources