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Heme- Midterm Study
Ch 2, 4, 6, 7, 8
| Question | Answer |
|---|---|
| 1. What is the primary defect in macrocytic anemias?: | Defective nuclear maturation caused by impaired DNA synthesis. |
| 2. What are the two main types of macrocytic anemias?: | Megaloblastic and Non-Megaloblastic. |
| 3. What are common causes of megaloblastic macrocytic anemia?: | Vitamin B12 deficiency, folic acid deficiency, drugs, congenital diserythropoietic anemia (CDA), and myelodysplastic syndrome (MDS). |
| 4. What are common causes of non-megaloblastic macrocytic anemia?: | Alcoholism, liver disease, hematologic diseases, and hemolytic anemia. |
| 5. What is a characteristic feature of polychromatophilic RBCs?: | They indicate ineffective erythropoiesis. |
| 6. What hematologic feature indicates ineffective erythropoiesis?: | Increased erythrocyte precursors in the bone marrow and decreased red cell release into peripheral blood. |
| 7. What is the typical MCV range in macrocytic anemia?: | 100 to 160 fL. |
| 8. What are Howell-Jolly bodies?: | Red cell inclusions often present in macrocytic anemia. |
| 9. What is the role of intrinsic factor in vitamin B12 absorption?: | Intrinsic factor allows absorption of vitamin B12 in the intestines. |
| 10. What are the four stages of vitamin B12 deficiency?: | Stage I: negative vitamin B12 balance; Stage II: vitamin B12 depletion; Stage III: vitamin B12-deficient erythropoiesis; Stage IV: vitamin B12 deficient anemia. |
| 11. What is pernicious anemia?: | An autoimmune disease that is the most common cause of vitamin B12 |
| 12. What are common clinical manifestations of pernicious anemia?: | Pallor, weakness, jaundice, smooth tongue, and neurological signs. |
| 13. What laboratory findings are associated with pernicious anemia?: | Decreased serum vitamin B12, Howell-Jolly bodies, increased MCV, and hypersegmented neutrophils. |
| 14. What is the Schilling test used for?: | To determine the cause of vitamin B12 deficiency, specifically to assess intrinsic factor absorption. |
| 15. What dietary sources are rich in folic acid?: | Green leafy vegetables, fruits, dairy products, cereals, and animal foods. |
| 16. What are common causes of folic acid deficiency? | : Nutritional deficiency, malabsorption, and drug-induced deficiencies. |
| 17. What laboratory findings indicate folic acid deficiency?: | Decreased serum folate, decreased RBC folate, and increased serum homocysteine. |
| 18. What is the recommended daily intake of vitamin B12?: | 5 µg/day. |
| 19. What is the recommended dietary intake of folic acid?: | 50 to 100 µg/day. |
| 20. What is the difference between megaloblastic and non-megaloblastic macrocytic anemia in terms of MCV? | Megaloblastic: MCV > 110 fL; Non-megaloblastic: MCV > 100 but < 110 fL. |
| 21. What is the significance of hypersegmented neutrophils in macrocytic anemia?: | they are seen in 98% of cases and indicate megaloblastic changes. |
| 22. What is the treatment for vitamin B12 deficiency?: | Lifelong vitamin therapy with cyanocobalamin or hydroxocobalamin. |
| 23. What is the treatment for folic acid deficiency?: | 1 to 5 mg/day for 2-3 weeks; lifelong therapy is not required. |
| 24. What is the role of transcobalamin II in vitamin B12 metabolism?: | It is the main transport protein for cobalamin to the tissues. |
| 25. What is the effect of chronic pancreatic disease on vitamin B12 absorption?: | It decreases proteases needed to release B12 from food. |
| 26. What is the significance of increased lactate dehydrogenase in macrocytic anemia? | It indicates hemolysis or ineffective erythropoiesis. |
| 27. What is the typical appearance of bone marrow in megaloblastic anemia?: | Hypercellular with nuclear-cytoplasm asynchrony. |
| 28. What is the primary function of red blood cells (RBCs)?: | To carry oxygen to distal tissues. |
| 29. What is the composition of the RBC membrane?: | 52% proteins, 40% lipids, 8% carbohydrates. |
| 30. What are the three layers of the RBC membrane?: | Outer layer (hydrophilic), central layer (hydrophobic), inner layer (hydrophilic). |
| 31. What type of proteins span the entire thickness of the RBC membrane?: | Integral proteins, including glycoproteins like Glycophorin A, B, and C. |
| 32. What is the role of spectrin in the RBC membrane?: | Strengthens the membrane and controls the biconcave shape. |
| 33. What are the characteristics of RBC membrane deformability?: | It is energy dependent (requires ATP) and can undergo reversible and irreversible changes. |
| 34. What ions are freely permeable across the RBC membrane?: | Anions such as chloride (Cl-) and bicarbonate (HCO3-). |
| 35. What is hemoglobin (Hgb)?: | A conjugated globular protein composed of a tetramer of 2 like pairs of globin chains and 4 heme groups. |
| 36. What percentage of hemoglobin synthesis occurs in nucleated RBCs?: | 65% in nucleated RBCs and 35% in reticulocytes. |
| 37. What is required for hemoglobin synthesis?: | Adequate iron delivery, synthesis of protoporphyrins, and adequate globin synthesis. |
| 38. How is iron delivered to the RBC precursor membrane?: | By the protein carrier transferrin. |
| 39. What happens to excess iron in the cytoplasm of RBCs?: | It aggregates as ferritin and can be stored as hemosiderin. |
| 40. What is the process of heme synthesis?: | Iron in the ferric state is delivered to the RBC membrane, enters the cytoplasm, is reduced to ferrous iron in mitochondria, and inserted into protoporphyrin to produce heme. |
| 41. Where does globin synthesis occur?: | On RBC-specific cytoplasmic ribosomes. |
| 42. What is the relationship between globin synthesis and protoporphyrin synthesis?: | The rate of globin synthesis is directly related to the rate of protoporphyrin synthesis. |
| 43. What is the structure of hemoglobin?: | Hemoglobin consists of 4 heme groups and 2 alpha chains plus 2 non-alpha chains. |
| 44. What is the role of cation pumps in the RBC membrane?: | To maintain the shape of the RBC by controlling the concentration of cations like sodium (Na+) and potassium (K+). |
| 45. What is the significance of ATP in RBC membrane function?: | ATP is required for membrane deformability and flexibility. |
| 46. What are the effects of irreversible forces on the RBC membrane? | : They require an increase in surface area and can lead to membrane fragmentation. |
| 47. What is the function of peripheral proteins in the RBC membrane?: | They strengthen the membrane and help maintain its shape. |
| 48. What happens to iron when globin or protoporphyrin synthesis is impaired? | : Iron accumulates in the RBC cytoplasm as ferritin aggregates. |
| 49. What is a sideroblast? | : An iron-laden nucleated red blood cell (nRBC). |
| 50. What is a siderocyte?: | An anucleated form of a sideroblast. |
| 51. What is a ringed sideroblast?: | A nucleated red blood cell with mitochondria encrusted with iron, visualized with Prussian Blue stain. |
| 52. What are the three types of hemoglobin found in normal adult RBCs? | Hgb A[α2β2], Hgb F [α2γ2], and Hgb A2 [α2δ2]. |
| 53. What is the primary function of hemoglobin?: | To deliver and release O2 to tissues and and facilitate CO2 excretion. |
| 54. What is the difference between deoxyhemoglobin and oxyhemoglobin?: | Deoxyhemoglobin has a lower affinity for O2, while oxyhemoglobin has a higher affinity. |
| 55. What physiological factors can adjust O2 affinity of hemoglobin?: | 2,3 DPG levels, pH, and temperature. |
| 56. What does the hemoglobin-oxygen dissociation curve illustrate?: | The relationship between O2 tension and the binding/dissociation of O2 by hemoglobin. |
| 57. What does a 'shift to the right' in the hemoglobin-oxygen dissociation curve indicate?: | Decreased hemoglobin affinity for O2, facilitating increased O2 delivery to tissues. |
| 58. What causes a 'shift to the left' in the hemoglobin-oxygen dissociation curve?: | Increased affinity for O2, resulting in decreased O2 delivery to tissues. |
| 59. What is carboxyhemoglobin?: | Hemoglobin that is unable to transport O2 due to CO binding, which is tighter than O2. |
| 60. What is methemoglobin?: | Hemoglobin that cannot transport O2 because the iron is oxidized to the ferric (Fe3+) state. |
| 61. What is sulfhemoglobin?: | Hemoglobin that cannot transport O2 due to increased sulfur content, often from sulfur-containing drugs. |
| 62. What are the four metabolic pathways in RBCs?: | Embden-Meyerhof Glycolytic Pathway, Hexose Monophosphate Shunt, Methemoglobin Reductase Pathway, Luebering-Rapaport Pathway. |
| 63. What is the main function of the Embden-Meyerhof Pathway?: | To generate 90% of ATP through anaerobic breakdown of glucose. |
| 64. What is the Hexose Monophosphate Shunt responsible for?: | Generating NADPH and reduced glutathione (GSH) for defense against oxidative injury |
| 65. What does the Methemoglobin Reductase Pathway maintain?: | The reduced ferrous state of hemoglobin to prevent accumulation of methemoglobin. |
| 66. What is the role of the Luebering-Rapaport Pathway?: | To generate 2,3 DPG, which modulates O2 affinity for hemoglobin. |
| 67. What is the reticuloendothelial system (RES) responsible for? | Removing old RBCs from circulation by phagocytosis. |
| 68. What is the difference between intravascular and extravascular hemolysis? | : Intravascular occurs within blood vessels, while extravascular occurs in the spleen and RES. |
| 69. What are the signs of intravascular hemolysis?: | Positive urine hemoglobin, decreased haptoglobin, and hemoglobinemia. |
| 70. What are the signs of extravascular hemolysis?: | Negative urine hemoglobin, absent haptoglobin, and bilirubin presence. |
| 71. What happens to hemoglobin during extravascular hemolysis?: | It is disassembled into heme and globin, with iron returned to the marrow. |
| 72. What is the fate of bilirubin after hemolysis?: | Converted to conjugated bilirubin in the liver and excreted into the intestines. |
| 73. What does normochromic indicate about red blood cells?: | It indicates that the red cell is normal in color. |
| 74. What is hypochromic: | red blood cells that appear paler than normal under a microscope, indicating a reduced concentration of hemoglobin |
| 75. What is hyperchromic?: | high concentration of hemoglobin |
| 76. What does MCHC stand for and what does it measure?: | Mean Corpuscular Hemoglobin Concentration; it measures the average concentration of hemoglobin in a given volume of red blood cells. |
| 77. What is anisocytosis?: | variation in RBC size |
| 78. What is poikilocytosis?: | variation in RBC shape |
| 79. What are microcytes?: | smaller than normal RBCs |
| 80. What conditions can lead to the formation microcytes?: | Conditions include impaired hemoglobin synthesis and ineffective iron utilization. |
| 81. What are macrocytes?: | larger than normal RBCs |
| 82. What can cause a macrocyte to form?: | Causes include impaired DNA synthesis, B12 or folate deficiency, and chemotherapy. |
| 83. What are codocytes?: | Target cells are red blood cells with an increase in surface membrane and are always hypochromic. |
| 84. What conditions are associated with codocytes?: | Conditions include liver disease, hemoglobinopathies, thalassemias, and sideroblastic anemia. |
| 85. What are spherocytes?: | Darkly staining RBCs with reduced or no central pallor |
| 86. What is the most common condition associated with spherocytes?: | Hereditary spherocytosis. |
| 87. What are stomatocytes?: | RBCs with an elongated mouth-like area in center |
| 88. What can induce stomatocytosis?: | Chemical agents like phenothiazine and chlorpromazine. |
| 89. What are elliptocytes/ovalocytes?: | Elliptocytes are red blood cells that are oval in shape and associated with hereditary elliptocytosis. |
| 90. What are sickle cells (drepanocytes)?: | Sickle cells are crescent or sickle-shaped red blood cells with rigid, inflexible hemoglobin. |
| 91. What are schistocytes?: | Schistocytes are fragmented pieces of red blood cells resulting from trauma to the cell membrane. |
| 92. What are helmet cells/keratocytes?: | Helmet cells are altered membrane red blood cells that may represent macrophages clearing out damaged cells. |
| 93. What are echinocytes?: | RBC with evenly spaced projections |
| 94. What are acanthocytes?: | Acanthocytes have 3 to 12 spicules of uneven length and are associated with excess cholesterol-phospholipid ratio. |
| 95. What are dacrocytes?: | Teardrop cells are pear-shaped or tear-shaped red blood cells associated with severe anemia. |
| 96. What does polychromasia indicate?: | Polychromasia indicates the presence of larger red blood cells with residual RNA, often seen in conditions like acute and chronic hemorrhage. |
| 97. What are Pappenheimer bodies?: | Pappenheimer bodies are siderotic granules containing ribosomes and associated with excess iron |
| 98. What are Heinz bodies?: | Heinz bodies are denatured hemoglobin seen with supravital stain, associated with G-6-PD deficiency. |
| 99. What is agglutination?: | Agglutination occurs when RBC antibodies and antigens are present, leading to clumping at room temperature. |
| 100. What is rouleaux?: | Rouleaux refers to red blood cells appearing as stacks of coins due to increased globulins or fibrinogen in plasma. |
| 101. What protozoan is associated with malaria?: | Plasmodium species, including Plasmodium vivax, Plasmodium malaria, Plasmodium falciparum, and Plasmodium ovale |
| 102. What is Babesia?: | Babesia microti is a protozoan transmitted by tick bites that invades blood circulation. |
| 103. What is anemia?: | A decrease in RBC count, hemoglobin (Hgb), or hematocrit (Hct), often associated with an underlying disease. |
| 104. What are the signs and symptoms of sudden anemia?: | Shock or shutdown due to a sudden loss of 30% or more of blood volume. |
| 105. How does slow blood loss affect symptoms of anemia?: | Fewer symptoms due to adaptation, including changes in heart and respiration rates. |
| 106. What are the classifications of anemias based on morphology?: | Normochromic, hypochromic, normocytic, microcytic, and macrocytic. |
| 107. What is a hypoproliferative anemia?: | Anemia caused by decreased production of red blood cells due to factors like iron deficiency or bone marrow failure. |
| 108. What causes maturation disorders in anemias?: | Abnormalities in nuclear or cytoplasmic development, such as B12 deficiency or thalassemia. |
| 109. What is hemolytic anemia?: | Anemia resulting from the destruction of red blood cells, which can be intravascular or extravascular. |
| 110. What are the key tests used in diagnosing anemia?: | RBC count, red cell morphology, red cell indices, hemoglobin (Hgb), hematocrit (Hct), reticulocyte count, and differential. |
| 111. What does the hemoglobin (Hgb) test measure?: | The main component of RBCs that carries oxygen to tissues and acts as a buffer for CO2 |
| 112. What is the reference range for hematocrit (Hct) in men?: | 42-52% |
| 113. What is the reference range for hematocrit (Hct) in women?: | 37-47% |
| 114. What does MCV measure in RBC indices?: | The average volume of red blood cells, with a reference range of 80-100 femtoliters. |
| 115. What is the MCH reference range?: | 27-31 picograms. |
| 116. What is the MCHC reference range?:. | 30-36% |
| 117. What characterizes microcytic/hypochromic anemia?: | MCV < 80 femtoliters MCH < 25 picograms MCHC < 30%. |
| 118. What can a peripheral blood smear reveal?: | Size, shape, and hemoglobin content of RBCs, as well as morphological abnormalities. |
| 119. What does an increased reticulocyte count indicate?: | Increased bone marrow activity in response to anemia. |
| 120. What is the significance of a bone marrow smear and biopsy?: | Evaluates RBC and WBC maturation, presence of megakaryocytes, and overall cellularity. |
| 121. What is iron deficiency anemia (IDA)?: | The most common type of anemia caused by a deficiency of iron affecting hemoglobin synthesis. |
| 122. What is the total body iron content?: | 3500-4000 mg 2/3 found in hemoglobin 1/3 stored in ferritin and hemosiderin. |
| 123. How is iron absorption regulated?: | Occurs in the intestinal mucosa, with uptake rates depending on the body's requirements. |
| 124. What is the average breakdown rate of RBCs?: | 1% of RBCs break down daily. |
| 125. What is the daily requirement of iron to replace lost iron from senescent RBCs?: | 20 mg of Fe |
| 126. What is the role of transferrin in iron transport?: | Transferrin binds ferric iron and assists in its delivery to erythroblasts in the bone marrow. |
| 127. How many atoms of ferric iron can bind to one transferrin molecule?: | Two atoms of ferric iron. |
| 128. What are the two forms of iron storage in the body?: | Ferritin and hemosiderin. |
| 129. What is ferritin?:. | The major storage form of iron that is water-soluble and easily mobilized |
| 130. What is hemosiderin?: | A less readily available storage form of iron that is not water-soluble. |
| 131. What does Total Iron Binding Capacity (TIBC) measure?: | The total amount of iron that can be bound by transferrin in plasma or serum. |
| 132. What is the normal range for serum iron levels?: | 65-170 µg/dL. |
| 133. How is transferrin saturation calculated?: | % Saturation = (serum iron x 100%) / TIBC. |
| 134. What is the significance of serum transferrin receptors (sTfRs)?: | They are inversely proportional to the amount of body iron. |
| 135. What are common causes of Iron Deficiency Anemia?: | Increased demand for iron, poor diet, increased blood loss, and malabsorption. |
| 136. What characterizes Stage 1 of Iron Deficiency Anemia?: | Iron depletion with normal RBC morphology and decreased hemosiderin content. |
| 137. What happens in Stage 2 of Iron Deficiency Anemia?: | Iron stores become exhausted, ferritin levels decrease, and TIBC increases. |
| 138. What are the laboratory findings in Stage 3 of Iron Deficiency Anemia?: | Decreased Hgb and Hct, microcytic/hypochromic RBC morphology. |
| 139. What is Anemia of Inflammation (AOI)?: | The second most prevalent anemia, often found in hospitalized patients. |
| 140. What are common causes of Anemia of Inflammation?: | Chronic diseases such as infections, neoplasms, and autoimmune disorders. |
| 141. What is Sideroblastic Anemia?: | Anemia caused by defects in the enzymes regulating heme synthesis. |
| 142. What are the laboratory findings in Sideroblastic Anemia?: | Microcytic/hypochromic morphology, increased saturation, and ringed sideroblasts. |
| 143. What is Hemochromatosis?: | A clinical disorder resulting from excess iron accumulation leading to tissue damage. |
| 144. What are the stages of Hereditary Hemochromatosis?: | Genetic predisposition, iron overload without symptoms, early symptoms, and organ damage. |
| 145. What characterizes Secondary Hemochromatosis?: | Can be acquired or secondary to inherited hemolytic anemias, with anemia and iron overload. |
| 146. What is the relationship between Free Erythrocyte Protoporphyrin (FEP) and iron levels? | FEP correlates inversely with ferritin levels. |
| 147. What is the significance of Zinc Protoporphyrin (ZPP)?: | It is a heme precursor that incorporates iron into hemoglobin. |
| 148. What is the typical hemoglobin level in Iron Deficiency Anemia?: | Less than 10 g/dL |
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