Redefining Budd-Chiari syndrome: A systematic review

Table 4 Risk factors and/or etiologies of classical Budd-Chiari syndrome and hepatic vena cava-Budd Chiari syndrome

	Classical BCS														HVC-BCS
	Perellóet al[40]	Smalberg et al[39]	Colaizzo et al[30]	Xavier et al[31]	Sakr et al[22]	Deepak et al[29]	Rautou et al[37]	Raszeja-Wyszomirska et al[45]	Westbrook et al[32]	D'Amico et al[34]	Seijo et al[11]	Harmanci et al[42]	Nozari et al[47]	Pavri et al[38]	Ebrahimi et al[46]	Qi et al[35]	Cheng et al[13]	Qi et al[36]
Country	Spain	Netherlands	Italy	Brazil	Egypt	India	France	Poland	United Kingdom	Italy	Europe	Turkey	Iran	United States	Iran	China	China	China
n (%)	21	40	32	31	94	20	94	20	66	31	157	62	55	47	21	169	145	25
MPN (%)	13 (62)	13 (33)	13 (41)	5 (16)		8 (40)	51 (59)	8 (40)	37 (56)	17 (55)	52 (33)	19 (31)	9 (16)			7 (4)9	5 (5)10
JAK2V617-F	N/A	7 (41)		8 (26)	18 (29)4	8 (40)			34 (52)							4 (2)	5 (5)10	0 (0)
PV	9 (43)										28 (18)			14 (30)		3 (2)	2 (2)10
ET	3 (14)										12 (8)			3 (6)		1 (1)	2 (2)10
FVL	2 (10)	5 (15)	6 (19)	3 (10)	34 (53)5	5 (25)	15 (19)	1 (5)	1 (2)	9 (29)	19 (12)	15 (30)	10 (18)	4 (9)		0 (0)	0 (0)11	0 (0)
PT 20210A		2 (8)	1 (3)	1 (3)	3 (5)6		6 (8)		1 (2)	1 (3)	5 (3)	1 (2)				0 (0)	0 (0)11	0 (0)
Protein C deficiency		2 (7)2			4 (4)	2 (10)	6 (12)	3 (15)	2 (3)		5 (3)	16 (31)	12 (20)	2 (4)			0 (0)12
Protein S deficiency		2 (7)2			1 (1)	1 (5)	5 (9)				3 (2)	5 (10)	3 (6)	1 (2)			0 (0)12
AT deficiency					4 (4)	3 (15)	3 (4)				4 (3)	6 (15)	3 (6)				0 (0)12
PAI-1 (4G-4G)										17 (55)
MTHFRC677T					31 (52)6					8 (26)		19 (39)				96 (71)		18 (72)
HH						2 (10)					29 (18)					64 (50)	30 (21)
PNH	4 (19)	2 (9)	1 (3)		2 (2)	1 (5)	8 (12)	1 (5)	0 (0)		15 (10)	1 (2)		3 (6)		1 (1)	0 (0)13	1 (4)
OCP, pregnancy, or puerperium1		13 (52) (OC)3	1 (4) (OC)3	7 (35)	19 (33)		21 (35) (OC)3			4 (24)	35 (39)	4 (11)	3 (9)	2 (6) (OC)3			2 (4)
Systemic diseases or local factors		2 (5)			12 (13)7				3 (5)7		37 (24)	8 (13)7	5 (9) autoimmune		4 (19)8	2 (1)	1 (1)
NAD/idiopathic	1 (5)				8 (9)			6 (30)		2 (6)		6 (10)	10 (18)		6 (29)		28 (19)
Web/membrane															11 (52)		89 (61)
MOVC																	6 (4)
MOVC + HV	1 (5)																60 (41)
MOHV																	23 (16)

¹Percentage of total female patients;

²Patients on anticoagulation were not tested;

³Number of women on OC only, no specific information on number of pregnant women;

⁴Out of 62 tested;

⁵Out of 64 tested;

⁶Out of 60 tested;

⁷Mainly Behcet’s disease;

⁸Behcet’s disease (2), Hepatitis C (1), Leukemia (1);

⁹2 of these 7 patients were diagnosed with latent MPN;

¹⁰In this study, most (105 patients), but, not all were tested; percentages are out of 105;

¹¹Out of 96 patients were tested;

¹²Out of 80 patients tested;

¹³Out of 83 patients tested. BCS: Budd-Chiari syndrome; HV: Hepatic vein; HVC: Hepatic vena cava; ET: Essential thrombocythemia; PNH: Paroxysmal nocturnal hemoglobinuria; OCP: Oral contraception; MOVC: Membranous obstruction of IVC; NAD: No associated disease/etiology found; MPN: Myeloproliferative neoplasms; FVL: Factor V Leiden mutation; HH: Hyperhomocysteinemia.