Splenic-Liver Syndrome refers to a group of symptoms characterized by splenomegaly, anemia, and liver cirrhosis. It is also known as thrombophlebitic splenomegaly syndrome, splenic anemia, congestive splenomegaly syndrome, chronic congestive splenomegaly, hepatosplenic fibrosis, or fibrotic congestive splenomegaly syndrome. First described by Banti in 1883, it is also called Banti syndrome. Initially, the spleen was considered the primary site of the disease, and the syndrome was clinically divided into the anemic phase, transitional phase, and terminal cirrhotic phase. Later, many scholars opposed classifying Banti disease as an independent condition. Currently, it is believed that the syndrome results from recurrent inflammation of the portal vein leading to thrombosis and occlusion of the portal or splenic veins, or cirrhosis. Therefore, many advocate discontinuing this diagnostic term.

bubble_chart Pathological Changes

The pathological changes mainly include hepatosplenomegaly, thickened and often hardened capsules presenting a pale grayish-red color, and histological fibrosis with dilated hepatic sinusoids and hyaline degeneration of Malpighian corpuscles. There is hemorrhage around the stirred pulse, with small iron-deposited nodules surrounding the stirred pulse. The spleen is often significantly enlarged, and the degree of enlargement is proportional to hypersplenism, but it shows no clear correlation with the duration of the disease. Due to fibrous tissue proliferation, the longer the disease course, the harder the spleen becomes.

bubble_chart Clinical Manifestations

Most patients are under 35 years old, with the onset often insidious. The main clinical manifestations include significant splenomegaly (mostly grade III), anemia and/or portal hypertension, while liver function is generally good with few symptoms. Symptoms such as lack of strength, digestive disturbances like abdominal distension and fullness, anorexia, nausea, and diarrhea may occur. Some cases present with sudden massive gastrointestinal bleeding. Occasionally, grade I jaundice and skin pigmentation are observed, and the liver is mostly grade I enlarged.

1. Laboratory tests showed decreased counts of red blood cells, white blood cells, hemoglobin, and platelets (pancytopenia). Bone marrow examination revealed active proliferation of nucleated cells with maturation arrest. Serum iron may be decreased, while total iron-binding capacity may be increased. Liver function may show varying degrees of impairment.

2. X-ray examination may reveal esophageal or gastric varices.

bubble_chart Diagnosis

1. The spleen is significantly enlarged.

2. Pancytopenia (bone marrow shows maturation arrest of nucleated cells) and manifestations of portal hypertension, while liver function impairment is generally mild.

3. It is necessary to exclude other diseases causing massive splenomegaly, such as post-hepatitis cirrhosis, chronic granulocytic leukemia, black Rebing, Niemann-Pick syndrome, subacute bacterial endocarditis, etc.

bubble_chart Treatment Measures

The main treatments are splenectomy and shunt or devascularization; symptomatic and supportive therapy. For those with impaired liver function, liver-protecting treatment is given. To prevent upper gastrointestinal bleeding caused by portal hypertension, calcium channel blockers such as nifedipine, 10-20mg each time, three times a day, or beta-blockers such as propranolol, 10-30mg each time, three times a day, can be used to reduce the heart rate by about 25%.