Intraoperative thromboelastography-guided transfusion in a patient with factor XI deficiency: A case report

Table 1 Mixing study

APTT (normal)	APTT (normal-2 h)	APTT (patient)	APTT (patient-2 h)	APTT (1:1)	APTT (1:1-2 h)
26.1 s	27.4 s	84.2 s	83.1 s	29.8 s	31.3 s

APTT: Activated partial thromboplastin time. APTT reference 23.3-32.5 s.

Table 2 Genetic and clinical features of different types of hemophilia and their management

	Hemophilia A	Hemophilia B	Hemophilia C
Genetics	X-linked	X-linked	Autosomal
Pathophysiology	FVIII deficiency	FIX deficiency	FXI deficiency
Clinical manifestations	Bleeding of variable severity correlated with factor levels	Bleeding of variable severity correlated with factor levels	Variable
Routine management	Prophylactic factor replacement	Prophylactic factor replacement	None
Perioperative management	Factor replacement, Cryoprecipitate. The goal is to keep the levels of FVIII > 50% for major surgery	Factor replacement, Prothrombin complex concentrate. The goal is to keep the levels of factor IX > 50% for major surgery	Controversial. May include: FFP, antifibrinolytics, TPE, factor replacement. Optimal FXI level unclear

FVIII: Factor VIII; FIX: Factor IX; FFP: Fresh frozen plasma; TPE: Therapeutic plasma exchange.