Case 1.2 Flashcards
(29 cards)
An autosomal recessive disorder of HgbSS is?
Sickle Cell Disease
Sickle cell trait is classified as?
Heterozygous HgbS (AS)
What do signs of sickle cell disease begin as early as 6 months of age?
HgbSS starts to replace fetal hemoglobin instead of adult hemoglobin.
What is the most common first sign of sickle cell disease between the ages of 6-9 months?
Dactylitis (swelling of the digits)
What pathogen causes osteomyelitis in sickle cell patients?
Salmonella.
Most sickle cell patients have functional asplenia, this puts them at an increased risk of infection from what type of organisms?
Encapsulated organisms.
Sickle cell aplastic crisis is most associated with?
Parvovirus B19 infection.
Painful occlusive crises in sickle cell patients are triggered by?
Cold weather, hypoxia, infection, dehydration, EtOH, and pregnancy.
A common finding in male patients with sickle cell during an occlusive crisis is?
Priapism
Expected hemoglobin and hematocrit levels for a patient with sickle cell disease are?
Both will be low
Hemoglobin between 5-9g/dL
Hematocrit between 17-29%
Peripheral smear for sickle cell patients will show?
Sickled Erythrocytes
Target Cells
Howell-Jolly Bodies (aspenia)
Diagnosis of sickle cell disease is done with?
Hemoglobin electrophoresis
Which drug should be avoided for sickle cell patients?
Meperidine (can cause seizures)
Pain control for sickle cell patients includes what three things?
- Oxygen therapy
- IV hydration
- Narcotics for adequate pain control
What medications would you expect a sickle cell patient to be on and why?
Hydroxyurea - reduces frequency of pain crises, increases RBC water levels, decreases sickling deformity, and increases HgbF population.
Folic Acid - to help maintain RBC production and DNA synthesis.
Prophylactic Penicillin - reduce chance of infection due to compromised spleen.
Children with sickle cell disease should be vaccinated with what vaccines?
SHiN
- S. pneumococcus
- Haemophilus Influenzae type B
- N. Meningococcus
Which thalassemia is more common among the Southeast Asia population?
alpha-Thalassemia
Which thalassemia is more common among the Mediterranean population?
beta-Thalassemia
Which alpha and which beta Thalassemia present most similarly?
alpha-Thalassemia Intermedia (HgbH Disease) and beta-Thalassemia Major.
beta-Thalassemia is diagnosed with?
Hemoglobin electrophoresis
Describe Hereditary Spherocytosis.
An autosomal dominant intrinsic hemolytic anemia.
What are the common clinical manifestations of hereditary spherocytosis?
- Anemia
- Jaundice
- Splenomegaly
- Pigmented black gallstones
Blood smear will show spherocytes, describe what a spherocyte is.
Round RBCs lacking central pallor
Hereditary Spherocytosis is diagnosed using what two tests?
- Osmotic Fragility Tests (positive)
- Coombs Tests (negative)
