Laboratory Diagnosis of Factor XIII Deficiency, Routine Coagulation Tests with Quantitative and Qualitative Methods.

Background: Factor XIII (FXIII) deficiency is a severe bleeding disorder with normal routine coagulation tests that makes diagnosis of the disorder complicated. After normal results in routine coagulation tests, clot solubility test, and FXIII activity, antigen assays along with molecular methods can be used for precise diagnosis of disorder. In the present study, we described routine coagulation tests along with clot solubility test and FXIII activity and antigen assays.

Methods: Data were collected from all relevant publications until 2015.

Results: All routine coagulation tests including prothrombin time (PT), partial thromboplastin time (PTT), thrombin time (TT), and platelet count are normal in FXIII deficiency (FXIIID) but different conditions such as blood collection, transport, and storage can result in abnormal results in these first line screening tests. In addition to these tests, clot solubility tests as the most common screening tests of FXIIID can influenced by different factors including clotting and solubilizing agents. Different commercial kits are available for FXIII activity and antigen assays with different sensitivity and specificity which could affect diagnosis of FXIIID.

Conclusions: Results of routine coagulation tests as well as clot solubility tests along with specific coagulation tests can affect diagnosis of FXIIID; therefore, all steps of these tests should be under control.