Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome

Table 1 Additional risk factors for thrombosis[6]

Age (> 55 in men, > 65 in women)

Risk factors for CVD

Hypertension

Diabetes mellitus

Elevated LDL or low HDL - cholesterol

Smoking

Family history of premature CVD

BMI ≥ 30 kg/m2

Microalbuminuria

Estimated GFR < 60 mL/min

Inherited thrombophilias

Oral contraceptives

Nephrotic syndrome

Malignancy

Immobilization

Surgery

LDL: Low density lipoprotein; HDL: High density lipoprotein; BMI: Body mass index; GFR: Glomerular filtration rate; CVD: Cardiovascular disease.

Table 2 Summary of the abdominal manifestations associated with the antiphospholipid syndrome[12]

Abdominal organ	Manifestations
Liver	Budd-Chiari syndrome:
	Hepatic veno-occlusive disease and occlusion of small hepatic veins
	Nodular regenerative hyperplasia
	Hepatic infarction
	Cirrhosis
	Portal hypertension
	Autoimmune hepatitis
	Biliary cirrhosis
	Liver transplantation
Intestine	Mesenteric ischemia (acute-chronic)[13,14]
	Peptic ulcer disease[15,16]
	Bowel ischemia and perforation[17]
	High prevalence of aPL but no increased vascular thromboses in inflammatory bowel disease
Spleen	Splenic infarction
	Autosplenectomy or functional asplenia
Pancreas	Acute pancreatitis

aPL: Antiphospholipid antibodies.

Table 3 Patient S's inherent thrombophilia markers

Mutation	Result
FV Leiden	-/-
G20210A in prothrombin gene	+/-
C10034T in γ-phibrinogen gene	-/-
Methylene tetrahydrofolate reductase 677TT polymorphism	+/-
4G/5G plasminogen activator inhibitor 1	+/+
G29926C in THBS gene (thrombospondine-4 gene)	-/-
G10976A in VII factor gene	-/-
С807Т in GpIa gene	-/-
Т1565С in GpIIIa gene	-/-
CYP2C9*2 ( cytochrome Р450 gene)	-/-
CYP2C9*3 (cytochrome Р450 gene)	-/-
G1639A in VKORC 1 gene (vitamin K hypoxide reductase gene)	-/-
I/D-polymorphism in ACE gene (anghiotensin-converting ferment gene)	-/-

-/-: No mutation; +/-: Heterozygous mutation; +/+: Homozygous mutation. In PAI-1 case: +/+ = 4G/4G, -/- = 5G/5G; in ACE case: +/+ = D/D, -/- = I/I. PAI-1: Plasminogene activator inhibitor 1; FV: V factor gene; GpIa: Glycoprotein I gene; GpIIIa: Glycoprotein III gene.