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Splenic Sequestration. Tessa Bandhan. Question 1.

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Splenic sequestration

Splenic Sequestration

Tessa Bandhan


Question 1
Question 1

  • A 3 year old girl known to have sickle cell disease (Hb SS) presents to the Emergency Room with a 2 day history of weakness and abdominal pain and recent onset of fever. Examination reveals HR140, RR40 T37.5C, pallor and tender splenomegaly. CBC show Hb4, WBC20, Plt 120. A diagnosis of acute splenic sequestration crisis is made.




Splenic sequestration1
Splenic Sequestration

  • Splenic sequestration is a complication of sickle cell anemia.

  • It occurs when red blood cells sickle within the spleen causing trapping of the blood cells. This leads to a rapid and marked fall in hemoglobin concentration and an enlarging spleen.



  • Clinical manifestations: adulthood, but most first attacks occur between 3 months and 5 years.

    • Pallor

    • Sudden weakness

    • Tachycardia

    • Tachypnea

    • Abdominal fullness

  • There is a risk of hypovolemic shock and death. Splenic sequestration crisis is associated with a 10-15% mortality rate, occurring before transfusions can be given.


  • Treatment adulthood, but most first attacks occur between 3 months and 5 years.

    • Immediate treatment involves urgent RBC transfusion. This corrects the hypovolemia and causes mobilization of the blood cells trapped in the spleen. Splenomegaly begins to resolve and the hemoglobin level begins to rise (autotransfusion). The decrease in the size of the spleen and relief of abdominal pain may occur within hours to days after the transfusion.




Question 2
Question 2 transfusions over a prolonged period may reduce the risk of acute sequestration.

  • An 8 year old boy with HbSC disease presents to the ER with pallor, weakness and abdominal pain. The differential diagnosis includes acute splenic sequestration crisis and aplastic crisis.



Question 21
Question 2 transfusions over a prolonged period may reduce the risk of acute sequestration.

  • What laboratory test would best identify his condition?

  • ESR

  • Hemoglobin level

  • WBC

  • Platelet count

  • Reticulocyte count



  • Aplastic include:anemia is as an acute illness with a Hb below the patient’s baseline with a decreased reticulocyte count. It is usually associated with parvovirus B19 infection.


Resources
Resources include:

  • Splenic sequestration and transient aplastic crises. Winfred C. Wang, MD. St Judes Children’s Research Hospital.

  • Splenic complications of sickle cell anemia and the role of splenectomy. Al-Salem, Ahmed. ISRN Hematology. Vol 2011, Article ID 864257


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