RBC S/F App Flashcards
microcytic (smaller than normal), hypochromic (enlarged area of central pallor)
spaghettios
Case 1: Where is the underlying
primary defect?
- Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
hemoglobin synthesis, main component of eythrocytes is hemoglobin so smaller and less pigmented makes sense
Case 1: What is the most likely specific defect?
1. Qualitative globin chain synthesis
2. qualitative heme synthesis
3. quantitative globin chain synthesis
4. quantitative heme synthesis
(4) quantitative heme synthesis, but could also be 3 (quantitative globin chain synthesis)
Case 2: How would you describe the
abnormal erythrocytes shown?
hyperchromic (lack central pallor), spherocytes
spherocytes tend to be normal size (normocytic)
Case 2: Where is the underlying
primary defect?
1. Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
- Membrane/cytoskeleton
Case 2: What is the most likely specific defect?
1. altered membrane lipids
2. altered membrane proteins
3. .qualitative defect in the membrane cytoskeleton
4. quantitative defect in the membrane cytoskeleton
(4) quantitative defect in the membrane cytoskeleton, spherocytosis, loss of spectrin usually
pool ball
Case 3: How would you describe the
abnormal erythrocytes shown?
They have a semicircular piece taken out of them
“bite” cells or degmacytes
Case 3: Where is the underlying primary defect?
1. Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
(3) energy metabolism
Case 3: What is the most likely specific defect?
hexose monophosphate subunit, G6PD defect, no reduced glutathione (antioxidant), ROS causes denatured hemoglobin called Heinz Bodies, splenic macrophages removes these heinz bodies causing bites
Case 4: How would you describe the
abnormal erythrocytes shown?
The red blood cells appear broken or fragmented: Schistocytes (split, divided)
Case 4: Where is the underlying
primary defect?
- Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
(4) Schistocyte formation occurs as a result of mechanical destruction (fragmentation hemolysis) of a normal red blood cell
Case 4: What is the most likely specific defect?
1. abnormal blood flow patterns
2. decrease in pH
3. Bone marrow fibrosis
4. increase in temperature
5. infectious agent
- abnormal blood flow patterns: heart issues, thrombi in microcirculation, DIC
Case 5: How would you describe the
abnormal erythrocytes shown?
elongated with tapered ends, hyperchromic
Sickle cells or drepanocytes
Case 5: Where is the underlying
primary defect?
1. Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
(2) hemoglobin synthesis
Case 5: What is the most likely specific defect?
1. Qualitative globin chain synthesis
2. qualitative heme synthesis
3. quantitative globin chain synthesis
4. quantitative heme synthesis
(1) qualitative globin chain synthesis, point mutation in position 6 of the beta globin gene, glutamic acid for valine substitution
Case 6: How would you describe the
abnormal erythrocytes shown?
hyperchromic with irregular spines, acanthocytes or spur cells
Case 6: Where is the underlying
primary defect?
- Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
- membrane/cytoskeleton
Case 6: What is the most likely specific basis for the defect?
1. altered membrane lipids
2. altered membrane proteins
3. qualitative defects in the membrane cytoskeleton
4. quantitative defects in the membrane cytoskeleton
- altered membrane lipids, excess cholesterol in the outer leaflet of the lipid bylayer, restricts membrane movement and causes the spiny folds
Case 7: How would you describe the
abnormal erythrocytes shown?
elliptical, elongated with rounded ends, elliptocytes
Case 7: Where is the underlying
primary defect?
- Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
- membrane/cytoskeleton
Case 7: What is the most likely specific basis of the defect?
1. altered membrane lipids
2. altered membrane proteins
3. qualitative defects in the membrane cytoskeleton
4. quantitative defects in the membrane cytoskeleton
(3) qualitative defect in the membrane cytoskeleton, involves structurally abnormal spectrin that does not interact as well
Case 8: How would you describe the
abnormal erythrocytes shown?
normocytic, normochromic, dacryocytes, teardrop cells
Case 8: Where is the underlying
primary defect?
- Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
(4) extrinsic to eythrocyte, dacrocytes are associated with myelofibrosis, they are also theorized to be formed due to mechanically squeezing out from the bone marrow as a result of the infiltrative process
Case 8: What is the most likely specific basis for the defect?
bone marrow fibrosis
Case 9: How would you describe the
abnormal erythrocytes shown?
spiculated with central pallor (acanthocytes do not have central pallor or regularly spaced spines), Burr cells or echinocytes
Case 9: Where is the underlying
primary defect?
- Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
- Energy metabolism
Case 9: What is the most likely specific basis of the defect?
1. aerobic respiration
2. hexose monophosphate shunt
3. primary glycolysis
4. rapaport-luebering shunt
- primary glycolysis, it lacks atp to power its sodium potassium pump so it becomes hypotonic and water leaves the cell through osmosis
Case 10: How would you describe the
abnormal erythrocytes shown?
target cells, codocytes, dark center in the area of pallor
Case 10: Where is the underlying
primary defect?
- Membrane/cytoskeleton
- Hemoglobin synthesis
- Energy metabolism
- Extrinsic to erythrocyte
(2) hemoglobin synthesis or (1) membrane/cytoskeleton
Case 10: What is the most likely specific basis of the defect?
1. Qualitative globin chain synthesis
2. qualitative heme synthesis
3. quantitative globin chain synthesis
4. quantitative heme synthesis
could be any technically
