Consumptive coagulopathy and fatal outcome in hepatic hemangioma: a rare adult case of Kasabach–Merritt syndrome
Anas Derkaoui, Tarik Deflaoui, Intissar Aissi, Abdelali Guellil, Rachid Jabi, Mohammed BouzianeIntroduction:
Kasabach–Merritt phenomenon (KMP) is a life-threatening consumptive coagulopathy that occurs in association with vascular lesions. Although classically described in children with kaposiform hemangioendothelioma, its occurrence in adults with giant hepatic hemangiomas is exceedingly rare and carries a high mortality rate.
Case presentation:
A 42-year-old female presented with acute right upper quadrant pain. Triple-phase CT revealed an 11 cm giant hepatic hemangioma involving segments VII and VIII with classical peripheral nodular enhancement. Laboratory investigations showed critical thrombocytopenia (platelets: 6000/mm 3 ), anemia (Hb: 7 g/dL), prolonged coagulation (INR: 1.9), hypofibrinogenemia (fibrinogen: 0.9 g/L), and markedly elevated D-dimer (>8000 ng/mL), confirming consumptive coagulopathy. Despite aggressive transfusion, thrombocytopenia remained refractory. The patient developed a fatal spontaneous cerebral hemorrhage prior to any surgical intervention.
Clinical discussion:
Giant hepatic hemangiomas may act as sequestration reservoirs, producing a systemic consumptive coagulopathy analogous to KMP. The failure of transfusion in this case illustrates the “consumptive sink” effect. Early surgical or endovascular intervention represents the only definitive approach and should not be deferred in favor of hematological stabilization alone.
Conclusion:
Adult KMP in the setting of a giant hepatic hemangioma is a surgical emergency. The consumptive sink effect renders preoperative stabilization alone insufficient, underscoring a critically narrow window for intervention.