The spleen is a vascular lymphoid organ that filters the blood and traps any foreign invaders in the white blood cells in the spleen. The spleen also produces the lymphocytes needed by our immune system to initiate a proper attack against invading pathogens in addition to destroying, recycling, and filtering old and damaged red blood cells. Metabolic disorders such Gaucher's disease, viral infections or genetic disorders such as hereditary spherocytosis can negatively effect the spleen and its ability to filter the blood and produce white blood cells. The recommended treatment for any of these spleen issues will be dependent on the severity and prognosis of the underlying disease.
Antimalarial Drugs
Hypersplenism is an idiopathic disorder characterized by an enlargement of the spleen as well as an increase in the spleen's filtration, and destruction of red and white blood cells. A prescribed treatment plan for hypersplenism will always target the underlying disorder, thus treatment options could range from antiviral drugs for infections such as hepatitis, to a spleenectomy for cirrhosis of the liver. Hypersplenism is often associated with repeated malaria infections and thus antimalarial drugs, such as chloroquine and progeny, are often prescribed, and have proven to be effective clearing the antigen buildup from repeated infections that results in elevated IgM levels and subsequently, an enlarged spleen.
Enzyme Replacement Therapy
Gaucher's disease is an inherited, metabolic disorder in which there is a deficiency in the enzyme glucocerebrosidase, a catalyst in he biodegradation of glucocerebroside, an intermediate in the glycolipid metabolic pathway. This enzyme deficiency results in the accumulation of the lipid glucocerebroside, which causes the liver and spleen to swell, in addition to causing anemia and lowering blood platelet counts. Enzyme replacement therapy with Ceredase, a recombinant human glucocerebrosidase, has proven to be an effective treatment for Gaucher's disease, reversing the visceral and hematologic manifestations of the disease. According the National Institute of Neurological Disorders and Strokes, a 1991 study comprised of 12 patients with type 1 Gaucher's disease were treated with Ceredase for a period of 9 to 12 months. By the end of that 9 to 12 month period, all the patients had increased hemoglobin levels, boosted platelet counts, decreased spleen enlargement by an average of one-third and reduced liver size in 5 of the patients.
Splenectomy
Hereditary spherocytosis is a congenital disorder affecting the integrity of red blood cell membranes, and results in fragile, spherical-shaped red blood cells that due their fragility, are unable to travel through smaller blood vessels and thus accumulate in the spleen causing splenomegaly and jaundice. The treatment of choice for hereditary spherocytosis is splenectomy because it significantly increases the survival rate of red blood cells. Total splenectomy, although effective, is often associated with surgical sepsis and increased vulnerability to infectious pathogens. An article in the January 2010 issue of "Touch Oncology" states that partial splenectomy is a better option since splenic function is preserved and the immune system is not completely compromised.
References
- National Institute of Neurological disorders and strokes: Seizures and Epilepsy: Hope Through Research:Treatment with Enzyme Replacement Therapy Reverses Symptoms in Patients with Type 1 Gaucher's Disease
- Touch Oncoology:Partial Splenectomy for Hereditary Spherocytosis--- What Do We Know and What Do We Need to Know?
