Authors: Papadaki D, et al.
Cureus 17(10): e95701. DOI: 10.7759/cureus.95701
Summary
A 25-year-old primigravida with aplastic anemia–paroxysmal nocturnal hemoglobinuria (AA-PNH) on eculizumab and cyclosporine required a scheduled cesarean delivery at 33+3 weeks. Despite near-daily transfusion support late in gestation, her preop platelets were 3 ×10³/µL; conventional coagulation tests were normal. To balance profound thrombocytopenia with PNH-related hypercoagulability, the team used serial rotational thromboelastometry (ROTEM) to individualize hemostatic therapy.
Because platelets remained critically low, general anesthesia was chosen over neuraxial. Pre-incision ROTEM showed delayed/weak clot formation on EXTEM with normal FIBTEM, prompting an additional platelet unit. After induction, ongoing bleeding and anemia led to 1 unit PRBCs and calcium. Repeat ROTEM showed improving EXTEM kinetics but early low fibrin contribution (low FIBTEM A5); with high thrombotic risk in mind, the team administered only tranexamic acid 1 g and deferred fibrinogen concentrate. Uterotonics (carbetocin 100 mcg; ergometrine 200 mcg) were given prophylactically. A male infant (2,375 g) had Apgars 8 and 9. Estimated blood loss was ~1.1 L; hemodynamics remained stable; the patient was extubated in the OR.
In recovery, labs showed Hgb ~9–10 g/dL and platelets ~52 ×10³/µL; ROTEM largely normalized aside from a slightly low EXTEM A5. Given thrombosis risk, no further factors were given. Enoxaparin 40 mg daily was started the day of delivery. The postoperative course was uncomplicated, and both mother and neonate did well.
What You Should Know
• Why ROTEM mattered: Standard labs were “normal” but ROTEM exposed platelet-driven weakness (low EXTEM firmness) and later a transient fibrin contribution deficit (FIBTEM A5), allowing targeted, conservative transfusion/antifibrinolytic choices in a prothrombotic disease.
• Technique selection: Profound thrombocytopenia precluded neuraxial; GA with invasive monitoring and multimodal analgesia minimized stressors that can trigger complement activation in PNH.
• Hemostasis strategy: Platelets first (to correct the ROTEM-identified platelet deficit), cautious TXA (1 g) given competing thrombosis risk, and avoidance of empiric fibrinogen concentrate once FIBTEM trended acceptable.
• PNH-specific periop points: Continue complement inhibition (eculizumab schedule maintained), prevent triggers (hypoxia, acidosis, hypothermia), and initiate early postpartum anticoagulation despite low platelets due to high thrombotic risk.
• Outcome: Stable intraop course, guided transfusion, timely extubation, and uneventful recovery for mother and infant.
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