Introduction: Congenital factor XIII deficiency is a rare bleeding disorder. Factor XIII deficiency is classified as severe when the factor level is < 5%, moderate between 5% -10%, mild > 10%. Acquired factor deficiency due to development of inhibitory autoantibodies has been reported in leukemia, liver disease, sepsis, major surgery or secondary to drugs like isoniazid etc. Commonly employed coagulation assays (PT, APTT) are unable to identify FXIII deficiency. With only a few case reports available, we performed this study to assess prevalence of factor XIII deficiency in hospital setting and evaluate clinical features.
Aim: To study the prevalence, clinical features, laboratory findings in patients with factor XIII deficiency in a hospital setting.
Study Design: Retrospective study of one year for patients in whom a coagulation profile including Factor XIII was performed. Factor XIII was done by ‘Berichrom Factor XIII assay” on coagulation analyzer Sysmex CS2400. Correlation between urea clot solubility and factor XIII assay levels was studied.
Results: Out of 4132 patients, 25 showed Factor XIII assay levels < 50 %, so the prevalence was 0.6 %. 18 were males and 7 were females. The age distribution was as : 1-10yrs=1, 11-20yrs=1, 21-30 yrs=2, 31-40 yrs= 5 , 41-60 yrs= 6, >60 yrs= 10.
Bleeding episodes were present in 8/24. We noted that in patients with factor < 30% had increased tendency to bleed.
We performed urea clot solubility test for all these 25 patients and assessed the nature of the clot from three different pathologists
Conclusion: We found that prevalence of factor XIII deficiency in a hospital setting is 0.6%. Urea clot solubility lacked sensitivity and was subjective when the factor levels were above > 10 %.
This study highlights that mild factor deficiency, probably acquired is much more common in a hospital setting but goes unrecognised.