2015 Factor XIII (FXIII) is composed of two catalytic A subunits and two carrier B subunits. Following activation by thrombin FXIII becomes plasma transglutaminase, which crosslinks the γ-glutamyl-ɛ-lysine residues of fibrin chains stabilizing fibrin clot. Congenital deficiency of factor XIII results in a severe life-long hemorrhagic disorder, abnormal wound healing in about 30% of patients and...

Inhibitor associated clotting factor XIII deficiency is a potentially life-threatening bleeding disorder with normal baseline coagulation studies, and may be associated with various drugs and autoimmune diseases. Failure to recognize this rare condition may lead to catastrophic outcome. We report a 52-year-old male with systemic lupus erythematosus (SLE) presented with recurrent spontaneous hae...

BACKGROUND Autosomal recessive bleeding disorders (ARBDs) include deficiencies of clotting factors I, II, V, VII, X, XI, XIII, vitamin K dependent clotting factors, combined factor V & VIII, Von Willebrand Disease (vWD) type 3, Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome. Patients with primary bleeding disorders from all the major provincial capitals of Pakistan were screened f...

Pelvic examination showed that it was normal and adequate for vaginal delivery. Her haemoglobin at the time of booking was 12 g/dl, group 0, rhesus positive. Haemoglobin electrophoretic pattern was A+C. Serological test was negative. Urine analysis showed no abnormality. The pregnancy progressed normally and she went into labour spontaneously at term. On 18 February 1976, after being in labour ...

Pelvic examination showed that it was normal and adequate for vaginal delivery. Her haemoglobin at the time of booking was 12 g/dl, group 0, rhesus positive. Haemoglobin electrophoretic pattern was A+C. Serological test was negative. Urine analysis showed no abnormality. The pregnancy progressed normally and she went into labour spontaneously at term. On 18 February 1976, after being in labour ...

BACKGROUND Post-tonsillectomy hemorrhage (PTH) has been reported in the literature as a serious complication after tonsillectomy that has high morbidity and can be life threatening. In cases of recurrent secondary PTH, one should consider coagulopathies as the hidden pathology. Factor XIII deficiency is very rare, suggested to be present 1 in 2 million people. Patients with undiagnosed factor X...

PURPOSE OF REVIEW Here we review recent developments concerning the diagnosis, classification and treatment of factor XIII (FXIII) deficiency and new findings related to the pathogenesis of the disease. RECENT FINDINGS Most recently, the International Society on Thrombosis and Haemostasis, Scientific and Standardization Committee published a guideline for the diagnosis and classification of F...