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Flashcards in Heme - Sickle Cell Anemia Deck (16)
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0
Q

Biochemical substitution in sickle cell anemia?

A

Valine for glutamine in the sixth amino acid position of the beta-globin chain

1
Q

Symptoms of acute chest syndrome?

A

Fever, tachycardia, chest pain, leukocytosis, pulmonary infiltrates

2
Q

Conditions that cause polymerization of red blood cells in sickle cell anemia?

A
  1. Hypoxia
  2. Acidosis
  3. Dehydration
  4. Variations in body temperature
3
Q

Pain crises in sickle cell are a consequence of? Precipitated by? Last for?

A

Microvascular occlusion of bones I by sickled cells cells

  1. Infection
  2. Cold
  3. Dehydration
  4. Venus stasis
  5. Acidosis

Last 2 to 7 days

4
Q

In sickle cell patients auto infarction of the spleen occurs when?

A

Early childhood (four years)

5
Q

Things related to sickle cell anemia?

A

AREAS

Aplastic anemia
Renal papillary necrosis
Encapsulated bacterial infections
Autosplenectomy
Splenic sequestration
6
Q

Acute chest syndrome? Associated with?

A

vasoocclusive crisis within the lungs associated with infection or pulmonary function.

7
Q

Chest x-ray of acute chest syndrome chose?

A

Progressive pulmonary infiltrates

8
Q

Aplastic crisis in sickle cell anemia results from?

A

Viral suppression of red blood cell precursors (especially by parvovirus B19)

9
Q

Complications of sickle cell disease?

A
  1. Hemorrhagic or ischemic stroke due to thrombosis
  2. Pigmented gallstones
  3. Priapism
  4. Congestive heart failure
10
Q

Treat acute chest with?

A
  1. Oxygen
  2. Analgesia
  3. Antibiotics
11
Q

Drug used to reduce the occurrence of pain crises? Mech?

A

Hydroxyurea and decitabine. Stimulates hemoglobin production.

12
Q

Indications for exchange transfusion?

A
#Acute chest syndrome
#Priapism
#Stroke
#Visual disturbance from retinal can function
13
Q

Sickle cell patient presents dropping hemoglobin – Best initial test?

A

Reticulocyte count (Should be very high. If low, think parvovirus)

14
Q

The only clinical manifestations of sickle cell trait?

A

Isosthenuria and hematuria

15
Q

Most accurate test for parvovirus B 19? Best therapy?

A

PCR for DNA; IVIg

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