What to do for a 1-year-old patient with prolonged bleeding after circumcision, bleeding time prolonged, prothrombin time (PT) elevated, activated partial thromboplastin time (APTT) prolonged, thrombocytopenia, and international normalized ratio (INR) slightly elevated?

Urgent Hematology Evaluation and Factor Replacement Required

This 1-year-old with prolonged bleeding after circumcision, markedly elevated PT (40 seconds) and APTT (120 seconds), has a severe coagulation factor deficiency—most likely hemophilia A or severe vitamin K deficiency—and requires immediate factor replacement therapy, not reassurance or simple local measures. 1, 2

Critical Laboratory Interpretation

The coagulation profile reveals:

• Severely prolonged APTT (120 seconds vs normal 30-40) indicates intrinsic pathway deficiency (Factors VIII, IX, XI, or XII) 1, 2
• Markedly prolonged PT (40 seconds vs normal 12-14) suggests extrinsic pathway involvement or combined deficiency 1
• Normal platelet count (160 × 10^9/L) excludes thrombocytopenic disorders 2
• INR of 1 appears inconsistent with the PT of 40 seconds and suggests either a reporting error or laboratory artifact 1

The combination of prolonged bleeding after circumcision with both PT and APTT elevation is a classic presentation of either severe hemophilia A (if primarily APTT-driven) or vitamin K deficiency bleeding (VKDB) in infancy. 3, 4

Immediate Management Algorithm

Step 1: Emergency Factor Replacement

• Do NOT reassure parents that "this is fine" (Option A is dangerous) 5, 6
• Administer recombinant Factor VIII at 50 IU/kg immediately if hemophilia A is suspected, as bleeding after circumcision in previously undiagnosed hemophilia can be life-threatening 4, 5, 6
• If vitamin K deficiency is suspected, give fresh frozen plasma (FFP) 15 ml/kg to provide all clotting factors immediately 3
• Vitamin K 1-2 mg IV should be administered regardless, as VKDB remains in the differential 3

Step 2: Local Hemostasis

• Packing alone (Option C) is insufficient without correcting the underlying coagulation defect 3
• Apply local pressure and hemostatic agents (gelatin sponge) to the circumcision site as adjunctive measures 7
• Packing may be used as a temporizing measure while factor replacement is being prepared 3

Step 3: Blood Transfusion Considerations

• Blood transfusion (Option B) addresses anemia but does NOT correct coagulation factor deficiencies 5, 6
• Transfuse packed red blood cells only if hemoglobin drops significantly from ongoing blood loss 3
• Hemoglobin/hematocrit monitoring is more reliable than repeated physical examination for detecting ongoing bleeding 4

Diagnostic Workup While Treating

Mixing Study

• Perform 1:1 mixing study immediately to distinguish factor deficiency from inhibitor 1
• Immediate correction indicates factor deficiency (hemophilia A, VKDB); failure to correct suggests inhibitor 1

Specific Factor Assays

• Measure Factor VIII activity level as the most likely deficiency given the clinical presentation 1, 2
• Check Factor IX if Factor VIII is normal 5
• Measure proteins induced by vitamin K absence (PIVKA-II) if VKDB is suspected 3

Family History

• Bleeding after circumcision is the classic presenting symptom of hemophilia A 3, 5, 6
• Two-thirds of patients with hemophilia may have no family history due to spontaneous mutations 6

Critical Pitfalls to Avoid

• Never dismiss prolonged post-circumcision bleeding as benign—23% of hemophilia patients have bleeding complications after circumcision even with adequate factor replacement 8
• Do not delay factor replacement while awaiting laboratory confirmation in active bleeding with severely abnormal coagulation studies 3
• Do not rely on local measures alone when coagulation studies show severe abnormalities 3, 7
• The reported INR of 1 is likely erroneous given the PT of 40 seconds; repeat the test and do not let this falsely reassure you 1

Expected Outcomes

• Bleeding should stop within hours of appropriate factor replacement 5, 6
• Two to three maintenance doses over 2-3 days are typically required 5
• Failure to respond suggests either inadequate dosing, inhibitor development, or incorrect diagnosis 4, 1