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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%.
One common definition of thrombocytopenia requiring emergency treatment is a platelet count below 50,000/μL. [5] Thrombocytopenia can be contrasted with the conditions associated with an abnormally high level of platelets in the blood – thrombocythemia (when the cause is unknown), and thrombocytosis (when the cause is known).
Increased deoxygenation causes sickling of red blood cells, which adhere to the spleen wall and splenic macrophages causing ischemia. [2] This ischemia can result in splenic sequestration, where large amounts of blood pool in the spleen but do not flow within vasculature. [11]
The spleen is especially prone to damage in SCD due to its role as a blood filter. A splenic sequestration crisis, also known as a spleen crisis, is a medical emergency that occurs when sickled red blood cells block the spleen's filter mechanism, causing the spleen to swell and fill with blood. The accumulation of red blood cells in the spleen ...
The basic pathology is some kind of obstructive pathology in the portal, hepatic or splenic vein that causes obstruction of venous blood flow from the spleen towards the heart. The cause of such obstruction may be abnormalities present at birth (congenital) of certain veins, blood clots, or various underlying disorders causing inflammation and ...
Acquired asplenia occurs for several reasons: . Following splenectomy due to splenic rupture from trauma or because of tumor; After splenectomy with the goal of interfering with splenic function, as a treatment for diseases (e.g. idiopathic thrombocytopenic purpura, thalassemia, spherocytosis), in which the spleen's usual activity exacerbates the disease
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Other causes of thrombocytopenia which may occur in pregnancy, such as drug induced thrombocytopenia, hereditary thrombocytopenia and pseudothrombocytopenia should also be ruled out. [63] ITP can be difficult to distinguish from gestational thrombocytopenia (which is by far the most common cause of thrombocytopenia in pregnancy). Unlike ITP ...
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