Hereditary spherocytosis

Fatigue may be a symptom of hereditary spherocytosis (HS), and is often associated with anemia in affected people. However, splenectomy typically cures the anemia (improving associated symptoms) in people with HS.^[1] Some people with severe HS may remain anemic post-splenectomy, and may need blood transfusions during an infection.^[2] We are not aware of reports in the literature suggesting that splenectomy or gallbladder removal is associated with persistent or excessive fatigue years after undergoing these surgeries.

Fatigue can be an important sign of many different types of illnesses or underlying conditions. We are unable to say whether it may be associated with hereditary spherocytosis, splenectomy, or gallbladder removal in any specific case. Fatigue that impacts daily life activities, or affects emotional or psychological well-being, should be discussed with a healthcare provider, as it may require medical treatment.

Overall, the long-term outlook (prognosis) for people with hereditary spherocytosis (HS) is usually good with treatment.^[1] However, it may depend on the severity of the condition in each person. HS is often classified as being mild, moderate or severe.^[2] People with very mild HS may not have any signs or symptoms unless an environmental "trigger" causes symptom onset.^[3] In many cases, no specific therapy is needed other than monitoring for anemia and watching for signs and symptoms.^[4] Moderately and severely affected people are likely to benefit from splenectomy.^[2] Most people who undergo splenectomy are able to maintain a normal hemoglobin level.^[3] However, people with severe HS may remain anemic post-splenectomy, and may need blood transfusions during an infection.^[2]

Information about life expectancy in the medical literature appears to be limited. However, we are not aware of reports that state that life expectancy is known to be significantly shortened in people without other medical problems who are managed appropriately. In all people who undergo splenectomy, there is a lifelong, increased risk of developing a life-threatening infection (sepsis).^[5] Although most septic episodes have been observed in children whose spleens were removed in the first years of life, older children and adults also are susceptible. Fortunately, taking certain precautions can reduce this risk and can prevent minor infections from becoming life-threatening.^[5] 

Very rarely, hereditary spherocytosis (HS) in people that have not undergone splenectomy has been associated with Moyamoya disease, which can increase the risk of blood clots, strokes, and transient ischemic attacks.^[6] However, because people who are anemic have lower cholesterol and whole blood viscosity than those who are not anemic, it has been suggested that people with HS who have not had their spleen removed should have fewer arteriosclerotic events (such as heart attack or stroke) than unaffected family members. Chronic anemia may slow down the development of arteriosclerosis.^[7]

People with HS who have undergone splenectomy may be at increased risk. Both venous and arterial vascular events have been associated with HS in patients who have undergone splenectomy compared to non-splenectomized patients.^[8] Long-term potential complications of splenectomy in adults may include an increased risk of atherosclerotic heart disease, and many adults who have had a splenectomy are on a low-dose aspirin therapy regimen. More recently, some evidence has pointed to a connection between splenectomy for HS and the development of venous thrombosis (blood clots). In some cases, a high platelet count has been suggested as a contributing factor. In others, the connection is less clear. In some patients, pulmonary hypertension has developed related to blood clots in the lung.^[4]