Heme - Sickle Cell Anemia Flashcards
Biochemical substitution in sickle cell anemia?
Valine for glutamine in the sixth amino acid position of the beta-globin chain
Symptoms of acute chest syndrome?
Fever, tachycardia, chest pain, leukocytosis, pulmonary infiltrates
Conditions that cause polymerization of red blood cells in sickle cell anemia?
- Hypoxia
- Acidosis
- Dehydration
- Variations in body temperature
Pain crises in sickle cell are a consequence of? Precipitated by? Last for?
Microvascular occlusion of bones I by sickled cells cells
- Infection
- Cold
- Dehydration
- Venus stasis
- Acidosis
Last 2 to 7 days
In sickle cell patients auto infarction of the spleen occurs when?
Early childhood (four years)
Things related to sickle cell anemia?
AREAS
Aplastic anemia Renal papillary necrosis Encapsulated bacterial infections Autosplenectomy Splenic sequestration
Acute chest syndrome? Associated with?
vasoocclusive crisis within the lungs associated with infection or pulmonary function.
Chest x-ray of acute chest syndrome chose?
Progressive pulmonary infiltrates
Aplastic crisis in sickle cell anemia results from?
Viral suppression of red blood cell precursors (especially by parvovirus B19)
Complications of sickle cell disease?
- Hemorrhagic or ischemic stroke due to thrombosis
- Pigmented gallstones
- Priapism
- Congestive heart failure
Treat acute chest with?
- Oxygen
- Analgesia
- Antibiotics
Drug used to reduce the occurrence of pain crises? Mech?
Hydroxyurea and decitabine. Stimulates hemoglobin production.
Indications for exchange transfusion?
#Acute chest syndrome #Priapism #Stroke #Visual disturbance from retinal can function
Sickle cell patient presents dropping hemoglobin – Best initial test?
Reticulocyte count (Should be very high. If low, think parvovirus)
The only clinical manifestations of sickle cell trait?
Isosthenuria and hematuria
