Red Blood Cell Disorders
Review on high yield concepts in Red Blood Cell Pathology. Designed for flashcard mode.
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| 🇬🇧 | 🇬🇧 |
| Due to decresed hemoglobin production | Microcytic anemia |
| Hemoglobin composition | Heme + globin |
| Heme composition | Iron+ protophorphrin |
| Most common type of anemia | Iron deficiency anemia |
| Most common nutritional deficiency in the world | Iron deficiency |
| Transports iron in the blood and delivers it to liver and bone marrow macrophages for storage | Transferrin |
| Binds intracellular stored iron and prevents iron from forming free radicals via the Fenton reaction | Ferritin |
| Primary cause of iron-deficiency anemia among elderly in the developing world | Ancyclostoma duodenale and Necator americanus |
| Primary cause of iron-deficiency anemia among elderly in the Western world | Colon polyps/carcinoma |
| Most common type of anemia in hospitalized patients | Anemia of chronic disease |
| Anemia due to defective protoporphyrin synthesis | Sideroblastic anemia |
| Cells with iron-laden mitochondria forming a ring around the nucleus of erythroid precursors | Ringed sideroblasts |
| Most commonly seen side effect of isoniazid leading to sideroblastic anemia | Vitamin B6 deficiency |
| Major cause of α-thalassemia | Gene deletion |
| Major cause of β-thalassemia | Gene mutation |
| Two most common causes of macrocytic anemia | Folate or Vitamin B12 deficiency |
| This drug is a folate antagonist whose main mechanism of action is to inhibit dihydrofolate reductase | Methothrexate |
| Detaches vitamin B12 from the R-binder | Pancreatic proteases |
| Binds Vitamin B12 in the small intestine | Intrinsic factor |
| Cells that produce intrinsic factor | Gastric parietal cells |
| Most common cause of Vitamin B12 deficiency | Pernicious anemia |
| Autoimmune destruction of parietal cells | Pernicious anemia |
| Inherited defect of RBC cytoskeleton-membrane tethering proteins | Hereditary spherocytosis |
| Test which reveals increased spherocyte fragility in hypotonic solution | Osmotic fragility test |
| Fragments of nuclear material in RBCs which emerges after splenectomy | Howell-Jolly bodies |
| Autosomal recessive mutation in beta chain of hemoglobin | Sickle cell anemia |
| Acquireid defect in myeloid stem cells resulting in absent glycosylphosphatidylinositol (GPI) | Paroxysmal nocturnal hemoglobinuria |
| Normal hemoglobin levels in males | 13.5mg-17.5 mg/dL |
| Normal hemoglobin levels in females | 12.5-16.0 mg/dL |
| Normocytic MCV values | 80-100 um^3 |
| Laboratory measure of iron in the blood | Serum Iron |
| Laboratory measure of transferrin molecules in the blood | Total Iron-binding capacity |
| Reflects iron stores in macrophages and liver | Serum Ferritin |
| Syndrome characterized by iron deficiency anemia with esophageal web and atrophic glossitis | Plummer-Vinson Syndrome |
| Enzyme that catalyzes the conversion of ALA to porphobilinogen | Aminolevulinic acid dehydrogenase (ALAD) |
| Enzyme that attaches protoporphyrin to iron to make heme | Ferrocheletase |
| Chromosome that codes for globin alpha genes | Chromosome 16 |
| Chromosome that codes for globin beta genes | Chromosome 11 |
| Folate circulates in the serum in what form? | Methyltetrahydrofolate |
| Methyl THF tranfers its methyl group of what molecule? | Vitamin B12 or Cobalamin |
| This molecule receives methyl from Vitamin B12 | Homocysteine |
| Impaired division of granulocytic precursors leads to what kind of neutrophils? | Hypersegemented neutrophils |
| Parasite that causes damage to the terminal ileum which may then lead to vitamin B12 deficiency | Diphyllobothrium latum |
| Cofactor tor the conversion of methylmalonic acid to succinyl CoA | Vitamin B12 or Cobalamin |
| Two main causes of normocytic anemia | Peripheral destruction and underproduction |
| Corrected reticulocyte count formula | RC x Hct/45 |
| Indicates good marrow response | Corrected RC >3% |
| Indicates poor marrow response | Corrected RC <3% |
| Laboratory findings when Storage Iron is depleted | Decresed ferritin, increased TIBC |
| Laboratory findings when serum iron is depleted | Decreased serum iron, decreased % saturation |
| Why is it necessary to sequester iron during chronic inflammation | To prevent bacteria from accessing iron which is necessary for their survival |
| Lab findings in anemia of chronic desease (ferritin, TIBC, serum iron, % saturation) | Increased ferritin, decreased TIBC, decreased serum iron, decreased % saturation, increased FEP |
| Rate limiting step in protoporphyrin synthesis | Aminolevulinic acid synthetase (ALAS) converts succinyl CoA to aminolevulinic acid (ALA) |
| Laboratory findings of sideroblastic anemia | Increased ferritin, decreased TIBC, increased serium iron and increased % saturation (iron overload state) |