An unusual presentation and treatment of a hemorrhaging plexiform neurofibroma

NF manifests as characteristic cutaneous pigmentation (café au lait lesions and freckles in groin and axillary regions), optic gliomas, osseous lesions, and benign peripheral nerve tumors [3]. Initial workup revealed a systolic blood pressure in the 60s–80s mmHg, Hemoglobin of 1.3 g/dL, D-dimer 11,710 ng/mL, fibrinogen of 74 mg/dL, Lactate of 7.7 mmol/L, pH of 7.157 and prolonged coagulation studies. Intraoperatively the patient underwent massive transfusion protocol and received an additional 25 units of packed red blood cells, 1 unit of fresh frozen plasma, 3 units of platelets, and 1 unit of cryoprecipitate.