The phenomenon of splenomegaly is associated with congenital hemolytic anemia, spontaneous bleeding, leukopenia, and more. Splenectomy can resolve this syndrome.
The spleen is responsible for producing blood cells during the fetal stage; it serves as a storage site for blood cells, releasing them into peripheral circulation when needed. It destroys aged blood cells, especially red blood cells; it is involved in phagocytosis and immune responses, producing antibodies to combat infections.
Splenomegaly is characterized by four basic features: one or all three blood cell lines are reduced; bone marrow activity is increased or normal; splenomegaly is present; and the condition improves rapidly after splenectomy.
What diseases are classified as splenomegaly?
Congenital hemolytic anemia: Also known as Minkowski-Chauffard disease, it occurs in children and young adults. Symptoms include anemia, pallor, slight jaundice, yellowing of the eyes, and dark-colored urine. The spleen may not be greatly enlarged. Blood tests are needed for diagnosis. The disease is caused by structural abnormalities in the red blood cell membrane.
Spontaneous bleeding: Also known as thrombocytopenic purpura, it typically occurs in individuals aged 10-40, predominantly in females. Symptoms include varying degrees of skin hemorrhage from petechiae to larger bruises; nosebleeds; and prolonged menstrual bleeding. The spleen is not enlarged, and if it is, it is only moderately so. Anemia may vary depending on the bleeding status. Blood tests show reduced red blood cells and platelets. Bleeding time is prolonged. Coagulation time is normal, but clots do not completely retract. If medication is used for three courses lasting over 12 months without improvement, splenectomy is indicated.
Leukopenia due to splenic issues: Patients experience fatigue, weakness, pallor, anemia, and bleeding. The spleen is markedly enlarged. There may be signs of infection: high fever, oral mucosal ulcers. The acute form may resolve spontaneously, while the chronic form typically requires splenectomy.
Disease affecting all three blood cell lines due to splenic issues: Patients exhibit anemia, skin hemorrhages, and oral mucosal ulcers. The spleen is enlarged. Blood tests reveal reductions in white blood cells, platelets, and red blood cells. Bone marrow activity is increased. This condition can be challenging to differentiate from acute leukemia at onset.
Banti’s syndrome: This condition is characterized by hepatomegaly, signs of increased splenic function, and cirrhosis; it typically occurs in individuals aged 15-35. There are four main signs: anemia, reduction in one or all three blood cell lines (red blood cells, white blood cells, or platelets); marked splenomegaly with a smooth, firm surface. Initially, adrenaline injection may cause the spleen to shrink, but if splenomegaly has persisted for a long time and fibrosis has developed, it may not shrink significantly. Splenectomy alleviates hematological symptoms. In the late stages, patients may experience cirrhosis with atrophy, ascites, hepatomegaly, vomiting, or hematochezia. At this stage, splenectomy is no longer indicated.
Many scientists believe that splenomegaly occurs due to the spleen sequestering blood cells and then phagocytosing them, leading to hemolysis within the spleen. Some suggest that the spleen secretes substances that inhibit bone marrow function and releases blood cells into peripheral circulation. Recent studies have identified antibodies against white blood cells and platelets in the serum of individuals with splenomegaly syndrome.
Splenectomy is the primary treatment method, indicated for all cases of splenomegaly, regardless of spleen size. Treatment with medication and blood transfusions often proves ineffective, and the condition worsens over time. The mortality rate can reach 50%.
Medical management is applied in mild cases using corticosteroids. In severe cases, while the effects may be beneficial, they are usually temporary. Corticosteroids may also be used while patients are being prepared for splenectomy to improve their condition.
Dr. Dao Ky Hung, Health & Life
