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Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders that are typically inherited. [2] The most common type is known as sickle cell anemia. [2] Sickle cell anemia results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. [2]
Splenic infarction can also result from a sickle cell crisis in patients with sickle cell anemia. Both splenomegaly and a tendency towards clot formation feature in this condition. In sickle cell disease, repeated splenic infarctions lead to a non-functional spleen (autosplenectomy).
The most frequent cause of autosplenectomy is sickle cell anemia [10] 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. [2]
Splenic sequestration crisis is a life-threatening illness common in pediatric patients with homozygous sickle cell disease and beta thalassemia. Up to 30% of these children may develop splenic sequestration crisis with a mortality rate of up to 15%.
A sickle cell health crisis can escalate into life-threatening complications, but patients still struggle to get seen quickly in emergency rooms and also to get pain medicine.
Hyposplenism is used to describe reduced ('hypo-') splenic functioning, but not as severely affected as with asplenism. Functional asplenia occurs when splenic tissue is present but does not work well (e.g. sickle-cell disease, polysplenia) – such patients are managed as if asplenic – while in anatomic asplenia, the spleen itself is absent.
A complete blood count (CBC) may show increased reticulocytes, a sign of increased red blood cell production, and decreased hemoglobin and hematocrit. The term "non-hereditary spherocytosis" is occasionally used, albeit rarely. [2] Lists of causes: [3] Warm autoimmune hemolytic anemia; Cold autoimmune hemolytic anemia/paroxysmal cold hemoglobinuria
Spleens are also removed for therapeutic purposes in conditions like hereditary spherocytosis, trauma to the spleen, and autosplenectomy caused by sickle cell anemia. Other causes are radiation therapy involving the spleen, such as that used to treat Hodgkin lymphoma. Howell–Jolly bodies inside of two normoblasts (center) in bone marrow.