Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature

Table 1 Classification of Budd-Chiari syndrome

Ref.	Type	Site of obstruction
Chaubal et al[46]	I	Obstruction of IVC with or without secondary hepatic vein occlusion
	II	Obstruction of major hepatic veins
	III	Obstruction of small centrilobular veins (considered by some as veno-occlusive disease)
Patil et al[48]	I	Lesions of the IVC
	IIa	Short segment (< 4 cm) lesion of the hepatic vein
	IIb	Diffuse lesion of the hepatic vein
	III	Mixed type with lesions of IVC and the hepatic vein
Bansal et al[47]	I	Hepatic vein obstruction or thrombosis without IVC obstruction or compression
	II	Hepatic vein obstruction or thrombosis with IVC obstruction or thrombosis
	III	Isolated hepatic venous webs
	IV	Isolated IVC webs

IVC: Inferior vena cava.

Table 2 Acute-on-chronic manifestation of Budd-Chiari syndrome classification

Type	Description of pathology
A	Acute hepatic vein thrombosis or stent block precipitates ACLF in a BCS
B	Non-thrombotic acute insult precipitates ACLF in a chronic BCS
C	Acute hepatic vein thrombosis precipitates ACLF in a non-vascular chronic
	Liver disease

ACLF: Acute-on-chronic liver failure; BCS: Budd-Chiari syndrome.

Table 3 Clinical characteristics of Budd-Chiari syndrome based on geographic region of the studied population[15,58-62]

Clinical characteristics	Percentage in various geographic regions
	Egypt	France	Sweden	China	Japan	United States	Algeria
Ascites/Abdominal distension	82	74.4	88	51.9	31.2	29.9	74.8
Abdominal fullness	-	-	-	-	26.1	-	-
Abdominal discomfort	-	-	-	-	17.8	-	-
Abdominal pain	96	72.4	81	-	2.5	-	42.6
Cirrhosis	-	-	-	-	-	18.6	-
Hepatomegaly	50	70.1	72	-	54.7	-	-
Splenomegaly	42	49.1	66	-	-	-	-
Esophageal varices	-	54.8	67	-	-	7.4	-
Hematemesis/Variceal bleed	36	-	9	16	8.3	3.2	-
Melena	-	-	-	-	2.5	-	-
Jaundice	16	20.3	29	-	5.7	-	13.9
Fever	-	20.1	27	-	-	-	15.7
Hydrothorax	-	13.0	-	-	-	-	-
Acute kidney injury	-	-	-	-	-	18.8	-
History of recurrent abortions	6	-	-	-	-	-	-
History of previous thrombosis	10	-	-	-	-	7	-
Recurrent orogenital ulcers	4	-	-	-	-	-	-
Leg ulcers	2	-	-	-	3.8	-	-
Lower limb edema	68	-	-	58.9	31.8	-	13
Dilated abdominal veins	40	-	-	57	27.3	-	-
Hepatic encephalopathy	36	-	12	0.7	-	9.5	5.2
Ileus	-	-	21	-	-	-	-
General malaise	-	-	-	-	12.1	-	-
Acute respiratory failure	-	-	-	-	-	7	-