Hematology Week 1: Diminished Erythropoiesis Flashcards
(78 cards)
Hemolytic anemia Structural protein defects in one of these skeletal proteins
- Ankyrin
- Band 3
- Spectrin
- Band 4.2
G6PD function
Important in making the reduced form of glutathione (GSH) which is critical in protecting against oxidant injury to the RBCs
G6PD Deficiency Genetics
X linked
G6PD Deficiency Triggers for hemolysis
3 listed
Infection - most common
Drugs - antimalarial
Food - Fava beans
G6PD Deficiency is most more common in? because?
Males because it is X-linked
G6PD Deficiency forms what kind of cells
Bite cells from the spleen removing precipitated Hb
Hemoglobin S AKA
Sickle Cell Disease
Most common hemoglobinopathy
Hemoglobin S (Sickle Cell Disease)
Hemoglobin S Mutation
Point mutation on Beta Globin gene leads to valine replacing glutamic acid at the 6th amino acid position
Hemoglobin S Prognosis
Often leads to severe lifelong hemolytic anemia
Hemoglobin S etiology
Vascular obstruction -> tissue Infarction -> Sickle Cell Crisis
Hb will form tetramer and will not be flexible and will have difficulty traveling through the vasculature and the spleen
Hemoglobin S Diagonisis
- Gold standard is hemoglobin electrophoresis
- Identify HBS based on mobility
β Thalassemia etiology
- absent or reduced synthesis of β globin chains
- increased α globin chains cause erythroid precursor damage
- causing marrow expansion and extramedullary hematopoiesis
β Thalassemia Mutation
Due to >170 β gene mutations
α Thalassemia
deletion of α gene resulting in a lack of α subunits and β subunits
α Thalassemia Mutations
- Gene deletions are common
- 2 deletions (asymptomatic mild anemia)
- 4 deletions is fatal in utero (hydrops fetalis)
α Thalassemia genes
α 1 and α 2 on chromosome 16
so each person has 4 copies
Hydrops fetalis
α Thalassemia deletion of all 4 copies of α genes
α Thalassemia 3 copies deleted
- soluble tetramers of β globins called H bodies
- H bodies are minimally damaging to RBCs
Characteristic cell type of diminished erythropoiesis
Macroovalocytes
diminished erythropoiesis PBS findings
- macroovalocytes
- reticulocyte count is low
- nucleated Red Cell Progenitors appear when anemia is severe
- neutrophils are larger than normal and show nuclear hypersegmentation (macropolymorphonuclear)
Aplastic Anemia AKA
empty bone marrow
Myelophthisic Anemia
Question 1
B Kidney damage
because EPO is low despite anemia
