3 year old with sickle cell anemia is dying

[usmlethinking]

A 3yr old girl with sickle cell anemia pesents with pallor, hypotension, tachycardia, and massive splenomegaly. What is the most likely explanation?

A. hemohagic shock
B. septic shock
C. splenic sequestation
D. hemolytic crisis

[dradi]

is it splenic sequestration ?

[draashna]

B Septic shock??

[Floater]

D.

I think this is Sickle Cell Crisis brought on by some stressor.

[usmlethinking]

I think the answer is splenic sequestration. Septic shock is not a bad answer because most acute splenic sequestration resembles septic shock. Here is a portion of an article in Medscape: http://www.medscape.com/viewarticle/475043_3

This article "Acute Splenic Sequestration Crisis Resembling Sepsis in an Adult" from medscape

ASSC is a common complication of sickle cell anemia in children with the SS genotype. It is less common in patients with the SC genotype. In one large series of children with hemoglobin SC disease, only 5% had episodes of acute splenic sequestration.[8] ASSC is even rarer in adults; when it does occur, it is often in the setting of low ambient oxygen tension, as occurs at high altitudes.[5,9]

Treatment of the acute crisis involves transfusion of blood products and supportive care with intravenous fluids, oxygen, and pain control. Given the potential for mortality, and because sequestration recurs in the majority of cases,[3,10] splenectomy is generally recommended after the acute event remits,[3,6,10] provided that the patient is an appropriate surgical candidate. Pathologic examination of the spleen during ASSC reveals marked splenomegaly, with weights reported up to 1,870 g.[4] Microscopic examination shows extensive pooling of red blood cells within the splenic cords, many of which are sickled. Numerous areas of necrosis and infarction may also be identified.[4]

Diagnosis is made on the basis of the clinical presentation. Scanning with 99mTc/sulfur colloid during the sequestration crisis classically shows almost complete lack of splenic uptake. CT scan may reveal multiple, peripheral, nonenhancing, low-density areas or large, diffuse areas of low density in the majority of the splenic tissue.[11] Although splenic infarcts may be apparent radiographically, marked splenomegaly, decreasing hemoglobin, hemodynamic instability, and multiorgan failure should alert the clinician to the possibility of splenic sequestration. Needle biopsy is not indicated to confirm the diagnosis of ASSC and should be discouraged, given the theoretical risk of splenic rupture. Fortunately, our patient did not suffer adverse consequences from this unnecessary diagnostic procedure.

[deoriveraMD]

A 3yr old girl with sickle cell anemia pesents with pallor, hypotension, tachycardia, and massive splenomegaly. What is the most likely explanation?

A. hemohagic shock
B. septic shock
C. splenic sequestation
D. hemolytic crisis

its splenic sequestration