SPLENIC SEQUESTRATION CRISIS

  • Acute sequestration of RBCs in the spleen

  • Sudden drop in hemoglobin and acute painful splenomegaly

  • 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.

  • Dramatic regression of splenomegaly and increase in Hb level occurs with prompt correction of hypovolemia and blood transfusion.

  • Splenic dysfunction occurs due to obstruction of sinusoidal blood flow, causing diversion of blood through intrasplenic shunts, bypassing phagocytic reticuloendothelial element of the spleen.

  • Children with palpable spleen at 6 months of age are at high risk of pneumococcal septicemia.

  • Emergency condition.

  • To prevent recurrence > splenectomy.

  • Treatment: Transfusion. Sometimes splenectomy