Table 2

Frequency distribution and features of patients with rare bleeding disorders.

Rare bleeding disorders (N=165)	N	%	Median age (yr)	Male (N)	Female (N)	Median factor levels	Disease severity

							Severe	Moderate	Mild
Fibrinogen	35	21.2	12 yr	11	24	0.6 g/L	Undetectable	0.1–1 g/L	>1 g/L
Fibrinogen	35	21.2	12 yr	11	24	0.6 g/L	03	30	02
Factor V	27	16.36	8 yr 06 mo	21	6	<0.1 IU/mL	Undetectable	<0.1 IU/mL	>0.1 IU/mL
Factor V	27	16.36	8 yr 06 mo	21	6	<0.1 IU/mL	02	23	02
Factor VII	46	27.88	11 yr	29	17	0.1 IU/mL	<0.1 IU/mL	0.1–0.2 IU/mL	>0.2 IU/mL
Factor VII	46	27.88	11 yr	29	17	0.1 IU/mL	12	29	05
Factor X	19	11.52	1 yr 6 mo	9	10	<0.1 IU/mL	<0.1 IU/mL	0.1–0.4 IU/mL	>0.4 IU/mL
Factor X	19	11.52	1 yr 6 mo	9	10	<0.1 IU/mL	17	02	-
Factor XI	14	8.48	18 yr	11	3	0.4 IU/mL	-	-	-
Factor XI	14	8.48	18 yr	11	3	0.4 IU/mL	-	-	-
Factor XII	2	1.21	24 yr	2	0	<0.1 IU/mL	-	-	-
Factor XII	2	1.21	24 yr	2	0	<0.1 IU/mL	-	-	-
Factor XIII	19	11.52	2 yr 2 mo	6	13	0.3 IU/mL	Undetectable	0.3 IU/mL	>0.3 IU/mL
Factor XIII	19	11.52	2 yr 2 mo	6	13	0.3 IU/mL	02	15	02
Combined factor V and VIII	3	1.81	21 yr	2	1	0.2 IU/mL	<0.2 IU/mL	0.2–0.4 IU/mL	>0.4 IU/mL
Combined factor V and VIII	3	1.81	21 yr	2	1	0.2 IU/mL	-	3	-