How should a 2‑month‑old infant with severe anemia, markedly prolonged prothrombin time and activated partial thromboplastin time, increased intracranial pressure, and an acquired vitamin‑K‑dependent clotting factor deficiency secondary to sepsis be treated?

Treatment of a 2-Month-Old Infant with Severe Anemia, Prolonged PT/aPTT, Increased Intracranial Pressure, and Acquired Vitamin K Deficiency Secondary to Sepsis

This infant requires immediate intravenous vitamin K administration (1 mg IV slowly over several minutes), urgent blood product replacement (fresh frozen plasma to correct coagulopathy and packed red blood cells to target hemoglobin 10 g/dL during shock resuscitation), aggressive sepsis management with antibiotics and cautious fluid resuscitation, and neurosurgical consultation for increased intracranial pressure management. 1, 2

Immediate Coagulopathy Correction

Administer vitamin K 1 mg intravenously immediately, given very slowly (not exceeding 1 mg per minute) to correct the acquired vitamin K-dependent clotting factor deficiency. 2 In infants with hemorrhagic disease and prolonged PT/aPTT, vitamin K administration typically shortens the prothrombin time within 30 minutes to 2-4 hours and is diagnostic if bleeding ceases. 2, 3, 4

Simultaneously infuse fresh frozen plasma to immediately correct the prolonged PT/aPTT and halt progression of intracranial hemorrhage. 1 The Surviving Sepsis Campaign specifically recommends plasma infusion in children with thrombocytopenia-associated multiple organ failure and progressive purpura, as fresh frozen plasma contains protein C, antithrombin III, and other anticoagulant proteins that are critically consumed in sepsis-associated disseminated intravascular coagulation. 1 Plasma should be infused with the goal of correcting prolonged prothrombin/partial thromboplastin times and halting purpura progression. 1

Critical Monitoring Point

Recheck PT/aPTT 6-12 hours after vitamin K administration to confirm correction (target PT <15 seconds, aPTT <35 seconds). 5 If coagulation parameters do not correct adequately within 6-8 hours, repeat the vitamin K dose. 2

Blood Transfusion Strategy

Transfuse packed red blood cells to target hemoglobin of 10 g/dL during active shock resuscitation, as recommended by the Surviving Sepsis Campaign for pediatric septic shock with low superior vena cava oxygen saturation (<70%). 1, 6 This contrasts with the lower threshold of 7 g/dL used after stabilization and recovery from shock. 1

Prioritize blood transfusion over aggressive crystalloid fluid boluses in this infant with profound anemia and septic shock. 6 The American College of Critical Care Medicine explicitly states that blood transfusion is superior to crystalloid or albumin bolusing in children with severe anemia and septic shock. 6

Transfusion Timing

Do not delay blood transfusion while waiting for coagulation correction—transfuse concurrently with fresh frozen plasma and vitamin K administration, as whole blood or component therapy is indicated for excessive bleeding and does not correct the underlying coagulopathy that vitamin K addresses. 2

Fluid Resuscitation in Context of Severe Anemia

Administer cautious fluid boluses of 10 mL/kg over 5-10 minutes rather than standard 20 mL/kg boluses to avoid hemodilution and pulmonary edema in this profoundly anemic infant. 6 Reassess after each bolus for positive response (improved capillary refill, stronger peripheral pulses, increased urine output, improved mental status, decreased heart rate) versus negative response (hepatomegaly, rales, increased respiratory distress). 6

Stop fluid administration immediately if hepatomegaly or rales develop, and initiate inotropic support instead. 6 Early and aggressive fluid loading is recommended in pediatric sepsis, but must be balanced against the risk of fluid overload in severe anemia. 1

Sepsis Management

Initiate broad-spectrum antibiotics immediately after obtaining blood cultures, as prognosis in pediatric sepsis depends on prompt diagnosis and intervention with aggressive fluid therapy and early antibiotics. 1

Use norepinephrine as first-line vasopressor if mean arterial pressure cannot be maintained above 65 mmHg despite adequate fluid resuscitation. 1 Early vasopressor use reduces organ failure incidence. 1

Consider stress-dose hydrocortisone (50 mg/m²/24h) if the infant has fluid-refractory, catecholamine-resistant shock, as approximately 25% of children with septic shock have absolute adrenal insufficiency. 1 Obtain serum cortisol level before administering empiric hydrocortisone. 1

Increased Intracranial Pressure Management

Obtain urgent neurosurgical consultation given the combination of acquired coagulopathy and increased intracranial pressure, which suggests intracranial hemorrhage. 1, 5 In vitamin K deficiency bleeding, intracranial hemorrhage is the most dangerous complication with high mortality (18-33%) even with surgical intervention. 7, 5

Do not perform neurosurgical evacuation until PT, prothrombin activity, and international normalized ratio are corrected, as premature surgery risks catastrophic intraoperative bleeding. 5 Surgical evacuation should only proceed after coagulation parameters normalize following vitamin K and fresh frozen plasma administration. 5

Warning Signs to Monitor

Watch for focal seizures, disturbed consciousness level, tense anterior fontanel, and respiratory distress—these are major presenting signs of intracranial hemorrhage in vitamin K deficiency bleeding. 5 Bulging fontanel occurs in 63% of cases. 7

Common Pitfalls to Avoid

Do not attribute the coagulopathy solely to sepsis-induced DIC without addressing vitamin K deficiency. While rapid resuscitation reverses most disseminated intravascular coagulation, purpura progresses in some children due to critical consumption of antithrombotic proteins, requiring plasma infusion. 1

Do not delay vitamin K administration while waiting for laboratory confirmation. In a bleeding infant with prolonged PT and normal fibrinogen/platelet count, vitamin K deficiency is almost diagnostic, and empiric administration should not be delayed. 3, 4

Do not use large-volume plasma infusions without concomitant diuretics or continuous renal replacement therapy, as this risks greater than 10% fluid overload. 1

Do not use propofol for sedation in this 2-month-old infant due to the association with fatal metabolic acidosis in children younger than 3 years. 1

Underlying Cause Investigation

Investigate for cholestatic liver disease or malabsorption as the underlying cause of acquired vitamin K deficiency in this 2-month-old, as secondary vitamin K deficiency bleeding most commonly occurs with hepatobiliary disease and poor vitamin K absorption. 3, 4, 8 Prolonged jaundice and failure to thrive are warning signs that should prompt investigation. 3