Splenic sequestration crisis

Splenic sequestration crisis is a major cause of mortality in young patients with SCD. The sequestration of RBCs in the spleen may result in a rapid drop of hematocrit, leading to hypovolemia, shock, and death. Immediate treatment is red cell transfusion to correct hypovolemia. Broad-spectrum antibiotic therapy, which includes coverage for pneumococci and H. influenzae, may also be beneficial, because infection may precipitate crises. " ... 

Treatment of SCD is aimed at the preventing and/or minimizing acute and chronic complications, including infection, acute chest syndrome, neurologic damage, and the various forms of sickle cell crises, including vaso-occlusive pain, splenic sequestration, and aplastic crisis. The acute and chronic complications are summarized in Tables 65-2,65-3, and 65-4. 

RBC transfusions are indicated for acute exacerbation of baseline anemia (e.g., aplastic crisis, hepatic or splenic sequestration, severe hemolysis), severe vasoocclusive episodes, and procedures requiring general anesthesia or ionic contrast. Transfusions might be beneficial in patients with compli-... 

Treatment options for splenic sequestration include observation alone, especially for adults because they tend to have milder episodes chronic transfusion to delay splenectomy and splenectomy after a life-threatening crisis, after repetitive episodes, or for chronic hypersplenism. 

Indications for red cell transfusions include (1) acute exacerbation of baseline anemia, such as aplastic crisis if the anemia is severe, hepatic or splenic sequestration, or severe hemolysis (2) severe vaso-occlusive episodes, such as ACS, stroke, or acute multiorgan failure and (3) preparation for procedures that require the use of general anesthesia or ionic contrast. Other patients in whom transfusions may be useful include patients with complicated obstetric problems, refractory leg ulcers, or refractory and protracted painful episodes or severe... 

Recurrent episodes are common approximately 50% in one study, and are associated with mortality. Options for management of recurrence include observation, chronic transfusion, and splenectomy. Observation is common in adults because they tend to have milder episodes. Increased risk of invasive infection after splenectomy is a concern in young children. Chronic transfusion may allow delaying splenectomy and temporarily restore splenic function, but it is associated with its own risks. Splenectomy is probably indicated, even after a single sequestration crisis, if that event is life-threatening. Splenectomy should be considered after repetitive episodes, even if they are less serious. For children less than 2 years of age, chronic blood transfusions have been recommended to prevent sequestration and to delay splenectomy until the age of 2, when the risk of postsplenectomy septicemia is less. Finally, splenectomy should also be considered for patients with chronic hypersplenism. " ... 

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