A male infant bleeds excessively from the umbilical stump on day 5 of life. Circumcision at birth was complicated by oozing that began several hours after the procedure. PT, aPTT, platelet count, and bleeding time are all normal. Which defect is most likely?
- A Mild hemophilia A
- B Glanzmann thrombasthenia
- C Factor XIII deficiency ✓
- D Alpha-2 antiplasmin excess
Correct answer: C. Factor XIII deficiency
Explanation
Factor XIII cross-links fibrin monomers and stabilizes the clot against fibrinolysis. Deficiency classically causes delayed bleeding from the umbilical stump and delayed wound dehiscence, with completely normal PT, aPTT, platelet function, and bleeding time because initial clot formation is intact. Diagnosis uses the clot solubility test in 5 M urea or monochloroacetic acid: an unstable clot dissolves. Treatment is factor XIII concentrate or cryoprecipitate. Glanzmann gives abnormal platelet aggregation and mucosal rather than delayed bleeding.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.