Autosomal recessive inherited bleeding disorders in Pakistan: a cross-sectional study from selected regions

Table 1 Frequency and severity of bleeding

ARBD (N)	Gum Bleeding	Hemarthrosis	Hematoma	Epistaxis	Menorrhagia^c x/y	Umbilical Cord Bleed	Traumatic Bleeding	Bruises	ICH	BG^a	BS^b
VWD-3 (95)	51	8	15	31	11/19	10	-	12	-	II	13.5
Fib. Def. (34)	2	6	17	9	-	-	23	15	-	II	12.5
GT (27)	23	-	4	19	8/9	16	27	19	-	II	11
FXIII Def. (13)	11	-	6	9	6/6	11	5	9	1	II	11.5
FVII Def. (12)	9	8	7	11	6/9	-	11	9	2	III	12
FV Def. (9)	7	2	6	5	5/7	-	4	5	1	II	11
Vit K Def. (8)	7	-	5	2	3/3	5	7	5	-	II	12
BSS (7)	7	-	7	7	-	-	7	7	-	II	11
FX Def. (2)	1	2	2	-	2/3	2	-		-	II	12
FII Def. (2)	2	1	2	1	1/2	-	-	1	-	II	10
FXI Def. (1)	1	-	1	1	-	-	1	1	-	II	10
FV & FVIII Def. (1)	1	1	1	-	-	-	-	1	-	II	11.5

ARBD autosomal recessive bleeding disorders, BG bleeding grade, BS bleeding score, BSS Bernard Soulier syndrome, Def. deficiency, Fib. fibrinogen, FII Factor II, FV Factor V, FVII Factor VII, FX Factor X, FVIII Factor VIII, FXI Factor XI, FXIII Factor XIII, GT Glanzmann Thrombasthenia, ICH intracranial hemorrhage, N number of patients, No. number, Vit vitamin, VWD-3 von Willebrand Disease type 3, x affected females, y females at risk
^aCalculated on the basis of WHO bleeding grades
^bbased on Tossetto et al bleeding score calculation scale
^cReported in adult female patients only

ISSN: 1750-1172