What is an autosplenectomy?
Autosplenectomy denotes spontaneous infarction of the spleen with resulting hyposplenism.
What does Hyposplenism mean?
Hyposplenia is the reduced or absent function of the spleen, impairing the capacity to prevent bacterial infections.
What does it mean to be Asplenic?
Asplenia means the absence of a spleen. Asplenia can occur in a variety of clinical settings, and it can refer to an anatomic absence of the spleen or functional asplenia secondary to a variety of disease states.
At what age does autosplenectomy occur in sickle cell?
The age range of the study subjects was 10-52 years, while that of the control group was 20-24 years. The mean age of the sickle cell patients was 23.2 ±5.3 years, while that of the control group was 22.7 ±12.4 years….Splenic size in the study subjects.
Splenic size | Frequency (%) n=74 |
---|---|
Autosplenectomy | 41 (55.4) |
At what age does autosplenectomy occur?
The demographics thus match those of sickle cell disease, usually occurring gradually in childhood with complete autosplenectomy achieved by the age of 8 4.
What is Dactylitis sickle cell?
Dactylitis is severe pain that affects the bones of the hands, the feet, or both. It’s often the first symptom of sickle cell disease in babies. Dactylitis is caused by blocked blood circulation. Symptoms include extreme pain and tenderness, usually with swelling. An episode may last 1 to 4 weeks.
Why do you get hyposplenism in Coeliac?
It is thought that hyposplenism in coeliac disease is the result of 2 distinct mechanisms: functional hyposplenism and splenic atrophy. Evidence to support these distinct mechanisms comes from the association between splenic atrophy and mesenteric lymph node cavitation (MLNC) syndrome.
How do you assess hyposplenism?
Functional hyposplenism is diagnosed by the presence of Howell-Jolly bodies and pitted erythrocytes in the peripheral blood smear, and by nuclear imaging modalities such as spleen scintigraphy with the use of Technetium-99m and/or spleen scintigraphy with the use of heat-damaged Technetium-99m labeled erythrocytes.
What causes asplenia?
However, the most common cause of asplenia is secondary to trauma, infarction, or surgery. Regardless of etiology, the most important consequence of asplenia is increased susceptibility to infection by encapsulated organisms, most commonly Streptococcus pneumoniae.
Is asplenia primary immunodeficiency?
Asplenia is a form of immunodeficiency, increasing the risk of sepsis from polysaccharide encapsulated bacteria, and can result in overwhelming post splenectomy infection (OPSI), often fatal within a few hours. In particular, patients are at risk from Streptococcus pneumoniae, Haemophilus influenzae, and meningococcus.
What is autosplenectomy caused by?
The most frequent cause of autosplenectomy is sickle cell anemia which causes progressive splenic hypofunction over time. Increased deoxygenation causes sickling of red blood cells, which adhere to the spleen wall and splenic macrophages causing ischemia.
What is sickle cell anemia and why should it result to the so called autosplenectomy?
The spleen may have to be removed due to complications of sickle cell disease in an operation known as a splenectomy. Some sickle cell patients will sustain enough damage to their spleen that it becomes shrunken and ceases to function at all. This is called autosplenectomy.
Who is most at risk for autosplenectomy?
Autosplenectomy is most frequently encountered in patients with homozygous sickle cell disease, although it has also been reported in pneumococcal septicemia 1, and systemic lupus erythematosus 2.
What does autosplenectomy stand for in medical terms?
autosplenectomy [ô′tōsplinek′təmē] a progressive shrinking of the spleen that may occur in sickle cell anemia.
When to have An autosplenectomy for sickle cell disease?
Autosplenectomy is most frequently encountered in patients with homozygous sickle cell disease, although it has also been reported in pneumococcal septicaemia, and SLE. The demographics thus match those of sickle cell disease, usually occurring gradually in childhood with complete autosplenectomy achieved by the age of 8
How does autosplenectomy increase susceptibility to infection?
Asplenia can increase susceptibility to infection. Autosplenectomy can occur in cases of sickle-cell disease where the misshapen cells block blood flow to the spleen, causing fibrosis and eventual atrophy of the organ.