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Old 09-04-2012
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Default Sickle Cell Disease

An 18 yr old man with sickle cell disease is brought to the emergency department because of sudden onset dyspnea,weakness and fatigue.He denies any fever or chest pain.On examination ,he has pallor.His lung are clear to auscultation and percussion.His abdominal examination is within normal limits without any hepatospenomegaly.The chest Xray is normal.Lab results are as follows:
Complete blood count
Hemoglobin 4.5g/dL
Reticulocytes 0.1%
Platelets 300,000cells/uL
Leukocyte count 10,000 cells/uL

Serum chemistry
Sodium 135
Potassium 3.4
Chloride 110
Bicarbonate 18
BUN 16mg/dL
Creatinine 1.0mg/dL
Glucose 18mg/dL

Which of the following is the most likely diagnosis?

A.Hyperhemolytic crisis
B.Splenic sequestration crisis
C.Acute chest syndrome
D.Aplastic crisis
E.Vasoocclusive crisis
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Old 09-04-2012
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I say it's A
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Aplastic crisis due Parvovirus b19 he has extremely low reticuloxytes which
would be high in option A and B, E causes C
D is the best option
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D.Aplastic crisis

The only choice which will cause low reticulocyte count, usually due to parvovirus B19.

All others will show appropriate reticulocytosis.
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Answer D Aplastic crisis

Sickle cell disease is characterized by a chronic ,well compensated hemolytic anemia with appropriate reticulocytosis.An acute drop in hemoglobin is a known complication of a sickle cell disease that occur secondary to a hyperhemolytic crisis, splenic sequestration or an aplastic crisis .An aplastic crisis is characterized by a transient arrest in erythropoieis that results in a sever drop in hemoglobin and virtual absence of reticulocytosis on peripheral smear(reticulocytosis <1%) .It may be caused by infection of parvovirus B19.Treatment consists of blood transfusion.It is important to note that an aplastic crisis in sickle cell disease differs from aplastic anemia , which occurs in patients without sickle cell disease and is characterized by pancytopenia.

Choice A. A hyperhemolytic crisis is a rare complication of sickle cell disease that is charchterized by a sudden ,severe anemia accompanied by an appropriate reticulocytosis.

Rest all options can have anemia but will have reticulocytosis rather than reticulocytopenia

Reference http://www.ncbi.nlm.nih.gov/pubmed/19418550
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Old 09-06-2012
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Quote:
Originally Posted by step_enhancer View Post
An 18 yr old man with sickle cell disease is brought to the emergency department because of sudden onset dyspnea,weakness and fatigue.He denies any fever or chest pain.On examination ,he has pallor.His lung are clear to auscultation and percussion.His abdominal examination is within normal limits without any hepatospenomegaly.The chest Xray is normal.Lab results are as follows:
Complete blood count
Hemoglobin 4.5g/dL
Reticulocytes 0.1%
Platelets 300,000cells/uL
Leukocyte count 10,000 cells/uL

Serum chemistry
Sodium 135
Potassium 3.4
Chloride 110
Bicarbonate 18
BUN 16mg/dL
Creatinine 1.0mg/dL
Glucose 18mg/dL

Which of the following is the most likely diagnosis?

A.Hyperhemolytic crisis
B.Splenic sequestration crisis
C.Acute chest syndrome
D.Aplastic crisis
E.Vasoocclusive crisis
D.) aplastic crisis
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