SPLENIC SEQUESTRATION CRISIS
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Acute sequestration of RBCs in the spleen
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Sudden drop in hemoglobin and acute painful splenomegaly
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The event is characterized by sudden trapping of large amounts of blood in spleen or less commonly in liver. Presents with profound anemia and shock.
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Dramatic regression of splenomegaly and increase in Hb level occurs with prompt correction of hypovolemia and blood transfusion.
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Splenic dysfunction occurs due to obstruction of sinusoidal blood flow, causing diversion of blood through intrasplenic shunts, bypassing phagocytic reticuloendothelial element of the spleen.
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Children with palpable spleen at 6 months of age are at high risk of pneumococcal septicemia.
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Emergency condition.
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To prevent recurrence > splenectomy.
- Treatment: Transfusion. Sometimes splenectomy