Acholuric Jaundice Syndrome (Abdominal Apoplexy Syndrome), also known as hereditary spherocytosis, is a congenital defect of the red blood cell membrane. It is characterized by chronic hemolytic anemia with acute episodes of jaundice, an increase in spherocytes in the blood, and increased osmotic fragility of red blood cells.

bubble_chart Pathological Changes

During the non-acute stage of attack, hemoglobin levels may be normal. However, during a hemolytic crisis or bone marrow aplastic crisis, hemoglobin levels drop significantly, with reticulocyte counts typically ranging between 5% and 20%. The most prominent feature is an increase in spherocytes, accompanied by markedly elevated red blood cell osmotic fragility. During hemolytic episodes, the serum jaundice index rises, with increased indirect bilirubin but minimal elevation in direct bilirubin. Urine tests show negative bilirubin but increased urobilinogen and urobilin. Red blood cell survival time is notably shortened. The bone marrow exhibits hyperactive erythropoiesis, but during an aplastic crisis, regeneration becomes suppressed.

bubble_chart Clinical Manifestations

It is mainly characterized by anemia, recurrent episodes of jaundice, and splenomegaly. This syndrome often manifests in early childhood, though some patients may not be diagnosed until middle age when symptoms worsen. In childhood, infections frequently trigger episodes of anemia. During the intercritical phase of hemolysis, due to the strong compensatory function of the bone marrow, anemia may be mild or absent, making it easy to overlook. Jaundice may occur during hemolytic episodes, but its severity varies. Almost all patients exhibit splenomegaly, mostly grade I to grade II, while the liver may show grade I enlargement or remain normal. Infections, fatigue, or emotional stress can induce acute hemolysis or even a hemolytic crisis, presenting as sudden high fever, nausea, vomiting, muscle pain in the back and limbs, liver and spleen pain, and worsening anemia. Infections may also lead to sudden bone marrow aplasia, exacerbating anemia. Symptoms of cholelithiasis may occur. Occasionally, chronic ulcers may develop in the ankle area of the lower limbs.

Splenectomy is the most effective treatment for anemia. After splenectomy, excessive hemolysis stops, and the lifespan of red blood cells returns to near normal.

bubble_chart Complications

Gallstones are a common complication.