Splenectomy or spleen removal surgery is resorted only when there is no other choice but to remove the infected or damaged spleen. Splenectomy is also resorted to in case of cancers involving the spleen such as leukemia or Hodgin lymphoma, severe lupus or blood disorders such as sickle cell disease or thalassemia. Idiopathic thrombocytopenic purpura is a blood disorder that often necessitates spleen removal. Splenectomy can be done in traditional open style or by laparoscopic method. The spleen functions include making antibodies and removing antibody-coated blood cells. So a Splenectomy leaves a person with a compromised immune system. Post spleen removal, patients are at higher risk for infections and blood vessel complications. Antibiotic prophylaxis and yearly vaccines are prescribed.
Hereditary Spherocytosis is a genetic disorder characterized by red blood cells that are fragile and spherical in shape instead of the normal flat disk shape. The abnormal shape makes it difficult for red blood cells to pass through the spleen. The spleen's function is to purify the blood and safeguard the immune function by fighting against the potentially dangerous bacteria, viruses or other microorganisms in the blood. In case of Spherocytosis, the membrane of the red blood cells is defective lending it a spherical shape. When these defective blood cells pass through the spleen, they break and destroy causing premature death of the red blood cells. The shortage of blood cells gives rise to severe anemia. It is an inherited type of Hemolytic Anemia. A parent with the disease has a 50% chance of having a child with the disease. Spherocytosis is most common in people of northern European descent.
The symptoms can vary from mild to severe. In severe cases, the disorder may be found in early childhood. In mild cases it may go unnoticed until adulthood. Patients with Spherocytosis exhibit all the symptoms associated with anemia such as fatigue, irritability, shortness of breath and muscle weakness. They also appear pale and weak. In addition to these symptoms, Spherocytosis also causes enlarged spleen and jaundice with yellow skin and eyes. Jaundice occurs due to the elevated levels of serum bilirubin as the red blood cells are destroyed within the body. Excess bilirubin, sometimes, also results in in the gallbladder. These stones may cause pain, infection or may block the tubes that lead out of the gallbladder.
Family history is checked for hereditary factor and the abdomen has to be checked for enlarged spleen. Following blood tests may be performed to support the clinical examination:
Splenectomy is the most definitive treatment for hereditary Spherocytosis. By removing the spleen, red blood cells are prevented from damage and are allowed to stay alive for a longer duration. However, removing a spleen makes the patient susceptible to infections for a lifetime. Hence patients need to take penicillin (or another antibiotic) for the rest of their lives. Immunization against pneumococcal and meningococcal infections is also prescribed. In case of severe anemia, blood transfusion is advised.
The spleen is responsible for the production of humoral antibodies and is a reservoir for blood cells. In case of Splenomegaly, the functions associated with the spleen are hampered. Enlarged spleen or Splenomegaly generally indicates the presence of an underlying medical condition which has to be attended to immediately. Physicians palpate the patient and observe the size variation that is clinically correlated with Splenomegaly. This routine examination is followed by other diagnostic tests which include blood, urine and body fluid analysis to identify the presence of infection.
Symptoms of Splenomegaly
The occurrence of Splenomegaly is usually asymptomatic unless there is an underlying factor such as bacterial, viral or parasitic infection. In most cases, patients complain of upper abdominal discomfort or pain, decreased appetite, weakness because of anemia and susceptibility to infections.
Splenomegaly predominantly occurs because of infections. However other metabolic disorders such as Niemann pick and Gaucher's disease can also serve as diagnostic markers. The susceptibility of Splenomegaly is high among children and elderly persons. Enlarged spleen can be life threatening as it may lead to splenic rupture in the abdominal cavity.
Treatment options for Splenomegaly include treating the underlying infection by administration of antibiotics. Post operative patients must be given antibiotics such as penicillin to avoid relapse of infections. In case of splenic enlargement, contact sports such as football, wrestling, boxing must be avoided. Children must be given vaccinations for Pneumococci, Haemophilus influenza (Bacillus Influenzae) on schedule to avoid infections. Splenectomy is the surgical choice in cases of total spleen malfunction or rupture.
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Bibliography / Reference
Collection of Pages - Last revised Date: March 30, 2020