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Congenital hemolytic anemia
Terms in this set (26)
What is characterstic of all congenital hemolytic anemias?
normocytic anemias with elevated retic count due to RBC destruction
What is mutated in Sickle Cell disease?
point mutation on Beta hemoglobin gene where glutamic acid is replaced with a valine
What are some causes of sickling of RBC?
dehydration, becoming cold, hypoxia, infection due to WBC clogging up the capillaries
What must be done to decrease the rate of stroke in sickle cell patients?
the HbS must be rapidly reduced to less than 30% by exchange transfusion
How do you distinguish between aplastic crisis and splenic sequestration crisis in SCD?
aplastic has a retic count of 0; sequestration will have enlarged and TENDER spleen on palpation. Both present with rapid occuring anemia
MOA of hydroxyurea
inhibits ribonucleotide reductase
What needs to be monitored when taking hydroxyurea?
WBC count b/c it will decrease it, which is good in certain SCD with high WBC
What are the major proteins afftected in Hereditary Spherocytosis?
AD defect in ankyrin, Band 3 and Beta- spectrin
Is the level high or low for Hered. Spherocytosis on EMA binding test?
LOW, it is more sensitive and specific than osmotic-fragility testing
When you see spherocytes on blood smear how can you differentiate between autoimmune hemolytic anemia vs. hereditary spherocytosis?
positive direct Coomb's test detects antibodies on RBC which is positive in AIHA
Is hereditary elliptocytosis or spherocytosis a defect in the horizontal or vertical protein dimers?
Ellip - horizontal (alpha spectrin); Sphero - vertical
How does G6PD deficiency cause anemia?
reduced glutathione is depleted and oxidative stress causes hemoglobin to become denatured and falls out of solution damaging the RBC
What are the 4 common types of sickle cell disease?
SS: sickle cell disease
SC: one sickle cell Beta globin and one C beta globin (less severe)
SB naught: one sickle cell beta globin and one thalassemic beta globin; severity equal to SS
SB(+): one sickle beta globin and on thalassemic that produces less HbA. varied severity
What medications should be started when a newborn is positive for Sickle Cell disease?
penicillin for prophylaxis; folate to prevent megaloblastic anemia and keep homocysteine levels reduced. Hydroxyurea - check WBC, can drop it.
What should be given to a sickle cell pain crisis patient who is NOT hypoxic?
hydration-have hyposthenuria and not drinking due to pain; IV narcotics; NSAIDs; incentive spirometry to prevent ACS. Oxygen if hypoxic; RBC transfusions if Hb<6 or hypoxic.
What commonly precipitates splenic sequestration crisis in patients with sickle cell?
What labs differentiate aplastic anemia from splenic sequestration crisis?
both have severe anemia; AA-LOW retic; SSC-HIGH retic
Do you need to give folate in AA or splenic sequestration crisis?
AA due to low retic that when treated will have a large reticulocytosis
What hemoglobin level should patients with AA or splenic sequestration crisis be transfused to?
What is the most common cause of death in a patient with Sickle cell disease?
Clinical definition of ACS
lung infiltrate taking up at least 1 lobe + fever, tachypnea, wheeze, or cough
What tests should be ordered for a patient suspected of having acute chest syndrome?
chest x-ray; CBC with retic count; blood cultures; pulse ox although ABG is better for O2 level
What are the most common sites for pain in a patient with ACS due to fat embolism?
pelvis and femur
What are the 3 main causes of ACS in a patient with sickle cell disease?
pneumonia; fat emboli; pain crisis causing splinting and hypoventiliation
Treatment for ACS
oxygen; azithromycin+cefuroxime; incentive spirometry; RBC transfusion to Hb of 10; pain control; IV hydration
What produces impotence in sickle cell patients?
THIS SET IS OFTEN IN FOLDERS WITH...
Acquired hemolytic anemia
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