Asplenia - A complication In Sickle Cell Disease

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Dysfunction and absence of the spleen and/ or its functions describe asplenia. Increased and progressive deoxygenation of blood continues the process of sickling. In sickle cell anemia patients, asplenia increases the susceptibility to infection. 100% of sickle cell patients are asplenic, this applies to sickle cell disease caused by hemoglobin SS.

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The spleen is the body’s largest blood filter, because it aids in the removal of old red blood cells RBC, from the circulation that aids removal of microorganisms that are bloodborne. Asplenia can be reduced functionally and is acquired. Causes of asplenia may include gastrointestinal disorder, infectious disease, hepatic disorders, and rheumatologic conditions. 

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One of its major causes is the continuous, recurrent, persistent sequestration of red blood cells, which leads to the trapping of the red blood cell in the spleen leading to spleen enlargement, and finally ends in atrophy. Asplenia can also be a result of direct infiltration of the malignant cells into the parenchyma of the spleen. Patients with sickle cell disease have asplenia which could be asymptomatic, while others might show fatigue, fever, and/ or encephalopathy. It is evaluated by abdominal ultrasound, computed tomography scan, magnetic resonance imaging, Howell – Jolly bodies, or spleen scintigraphy. 

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The most common complication of asplenia and hyposplenia is the overwhelming post-splenectomy infection of OPSI. Up to 60 – 70% rate of mortality due to Overwhelming post-splenectomy infection, which is characterized by the encapsulation of microorganisms caused by bacteremia.  

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Parasitic infections, septicemia (blood poisoning) are common in asplenic patients. It can be managed with the use of antibiotics and vaccination. The vaccinations to be given are pneumococcal polysaccharide vaccine, Hemophilus influenza type b vaccine, meningococcal conjugate vaccine, influenza vaccine. 

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The antibiotics which can be administered to patients with sickle cell disease are amoxicillin, penicillin, erythromycin, phenoxymethylpenicillin. With the loss of lymphoid tissue in the spleen, the immune system fends off infection like a handicap. 

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