Term
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Definition
G-CSF increases Neutrophils
Erythropoietin increases Erythrocytes
Thrombopoietin increases Megakaryocytes |
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Term
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Definition
Later stage cytokine.
Increases Neutrophil production.
Released by endothelial cells and macrophages due to inflammation.
Useful for Chemotherapy patients due to neutropenia. |
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Term
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Definition
Normoblasts and Reticulocytes.
BFU-E lineage.
Not in RBCs because no nucleus. |
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Term
| Heme Synthesis Rate-limiting enzymes |
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Definition
| ALA-Synthase (first) and Ferrochelatase (last) |
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Term
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Definition
Rate limiting enzyme in Heme synthesis, first enzyme.
Vitamin B6 is cofactor.
IRE binds the mRNA unless iron is there. |
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Term
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Definition
Causes Sideroblastic Anemia
ALA-dehydratase and Ferrochelatase are affected in heme synthesis.
Causes increased ALA and Protoporphryn IX levels. |
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Term
| Congenital ALA Synthase Deficiency |
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Definition
causes Sideroblastic Anemia.
X-linked inheritance. |
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Term
| Acute Intermittent Porphyria |
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Definition
due to inhibition of PBG deamination.
Reduced heme production and increased ALA-Synthase levels. |
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Term
| Congenital Erythropoietic Porphyria |
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Definition
Accumulation of heme synthesis intermediates.
They become oxidized and can cause scarring especially with sun exposure. |
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Term
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Definition
| On Chromosome 16. Have Hypersensitivity Domain (HS) |
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Term
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Definition
| Chromosome 11. Have Locus Control Region (LCR). Highly coordinated with Alpha-like genes. |
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Term
| Regulation of Globin synthesis |
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Definition
Occurs at Translational level.
Heme-Control Inhibitor (HCI) binds mRNA. Heme binds HCI to release it and allow translation of Globin. |
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Term
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Definition
Less Alpha Globin Produced.
Due to gene deletions.
4 genes (two on each chromosome) - varying severities of the disease. |
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Term
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Definition
Less Beta Globin Produced.
Due to Point Mutations.
The excessive alpha chains precipitate forming Heinz bodies.
Only 2 genes.
Homozygotes are often fatal. |
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Term
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Definition
Ferrous Iron converted to Ferric Iron in Hemoglobin. Irreversibly Oxidized. Curve shifts far left because it won't give up the O2.
Cyanosis, black tongue, Heinz bodies. |
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Term
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Definition
Anaerobic Glycolysis to make ATP (no mitochondria).
Rapaport Luebering Pathway to make 2,3BPG.
HMP Shunt uses G6PD to make Glutathione (the RBC antioxidant).
Methemoglobin Reductase Pathway to revert methemoglobin back to hemoglobin. |
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Term
| Neutralizing Reactive Oxygen Species (ROS)... |
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Definition
| Superoxide Dismutase converts radicals to O2 or H2O2, Glutathione Peroxidase breaks H2O2 into water and O2. |
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Term
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Definition
Intrinsic Defect that shortens RBC lifespan.
Most common RBC Metabolic disorder.
X-Linked Inheritance.
Hemolytic Anemia (episodic) in response to oxidants because Glutathione isn't made.
"Bite Cells" on blood smear. |
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Term
| Pyruvate Kinase Deficiency |
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Definition
Most common defect of glycolytic pathway.
Autosomal recessive inheritance.
Reduced ATP production.
2,3BPG accumulation decreases O2 carrying capacity of hemoglobin.
Causes Hemolytic Anemia. |
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Term
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Definition
Uptaken by Enterocytes in intestinal mucosa.
1. Ferric form reduced to Ferrous form.
2. Brought in with H+ using DMT-1
3. Either stored in Ferritin or moved to blood through Ferroportin then transported using Transferrin. |
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Term
| Hereditary Hemochromatosis |
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Definition
Mutations that affect Ferritin, Ferroportin, and accessory receptor proteins.
Autosomal Recessive |
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Term
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Definition
Binds to Cytochrome Oxidase (Complex IV) in the ETC.
Severe metabolic Acidosis/CNS poisoning.
Nitrite is used to make MetHb that picks up the CN-. |
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Term
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Definition
| % whole blood occupied by RBCs |
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Term
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Definition
"Mean Corpuscular Volume"
Average volume of RBCs.
Either Microcytic, Normocytic, or Macrocytic. |
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Term
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Definition
"Red Cell Distribution Width"
Measures homogeneaity of RBC size on bell curve. |
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Term
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Definition
| Measures production of new RBCs by the bone marrow. |
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Term
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Definition
-Excessive RBC Loss
-Shortening of RBC Lifespan
-Decreased RBC Production |
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Term
| Signs of Shortened RBC lifespan... |
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Definition
Increased Reticulocyte Count.
Increased EPO levels.
Increased LDH levels due to RBC lysis.
Increased Bilirubin levels due to metabolism of dead RBCs.
Decreased Haptoglobin because it is used up clearing heme out of the blood. |
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Term
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Definition
Intrinsic Defect that shortens RBC lifespan.
Mutations in Ankyrin and Band-3 cause damage to RBC membrane.
Elevated Reticulocyte count.
Splenomegaly.
Spherocytes in blood smear. |
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Term
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Definition
Intrinsic Defect that shortens RBC lifespan.
Episodic Painful Crises.
Chronic Erythroid Hyperplasia of Bone Marrow, "Crew Cut" X-Ray appearance.
Normocytic Anemia, sickle cells in blood smears, high reticulocyte count.
Parvovirus causes "Aplastic Crisis".
HbF causes less sickling. |
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Term
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Definition
| An alkylating agent used in chemotherapy and to increase HbF to help sickle cell patients. |
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Term
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Definition
Physical mediators of hemolysis that decrease RBC survival time.
"DAMM"
-Disseminated Intravascular Coagulation
-Arteritis
-Malignant Hypertension
-Mechanical Heart Valves |
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