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BloodDiseases
| Question | Answer |
|---|---|
| What percent of the total blood volume is plasma? | 60% |
| What are the contents in plasma? | a pale, aqueous solution of electrolytes, proteins, and small organic molecules like glucose. |
| Where are red blood cells and plasma located once they have settled in a test tube? | Plasma is on top, white blood cells and platelets (buffy coat) are in the middle, and the red blood cells are on the bottom |
| What is the major component of plasma and what is the function of this component? | Water-it functions as a solvent for transport and it also helps regulate body temperature (37 degrees) |
| What makes up 7% of plasma? | Proteins-most of which come from the liver |
| What purpose do electrolytes serve in plasma? | To maintain acid-base balance (pH of 7.6) |
| What are various functions of the proteins in plasma? | Hemostatic (so you don't bleed to death), complement, immunoglobulins (protection), and transport |
| What it hematocrit? | The estimation of packed erythrocytes (normal value is 40-50%) |
| Blood is composed of what two phases? | Liquid (plasma) and solid (cellular) |
| What is the total volume of blood in a 160lb man? | 5 liters |
| What are the bone forming organs? | Bone marrow, spleen, liver, and lymphatics (lymph nodes and lymphatic vessels) |
| Blood is among the _______ organs of the body. | Largest |
| Which blood cells are the most numerous? | Red blood cells (erythrocytes) |
| What is the key function of red blood cells? | to transport oxygen to tissues and to carry carbon dioxide away from these tissues |
| What is the protein that performs the key function of red blood cells? | Hemoglobin (it is also responsible for the characteristic color of blood) |
| Which blood cells are the least numerous? | White blood cells (leukocytes) |
| What 3 categories can white blood cells be divided into? | granulocytes, lymphocytes, monocytes |
| What is another word for platelets? | thrombocytes |
| Granulocytes can be further divided into what? | Neutrophils, Basophils, and eosinophils |
| What are neutrophils? | the most numerous WBC (55-75%) and they are the first line of defense in inflammatory processes. |
| What are basophils? | they contain granules with amines like histamine, which have a role in infection and allergy |
| What are eosinophils? | they are primarily used to attack parasitic worms. |
| What is the second most common group of WBCs? | Lymphocytes (20-35%) |
| What are T-lymphocytes? | They are responsible for cell-mediated immunity, also called T-cells and they come from the thymus. |
| What are B-lymphocytes? | They produce antibodies and support humoral immunity, they are called B-cells for bursa of Fabricius |
| Monocytes make up about ____ percent of WBCs. | 5% |
| Monocytes circulate for about ___ hours and exit to tissues to mature to _________. | 10, macrophages |
| What do monocytes do? | They remove debris, aid the inflammatory response, process aged RBCs, and help in presenting and processing of antigens to T-cells. |
| _________ are the second most numerous blood cell. | Platelets |
| How are platelets produced? | By fragmentation of the peripheral cytoplasm of megakaryocytes in bone marrow. |
| Why are platelets important? | They are important in hemostasis and blood coagulation. |
| What are 5 functions of blood? | Respiratory gas transport (carries oxygen), transport of nutrients (carries food) and waste products, thermoregulation (maintains constant temperature), host defense mechanism (fighting infection), and hemostasis (plugs holes as you puncture vessels) |
| What is a megakaryocyte? | It is the largest cell found in the bone marrow, and it forms platelets by shedding pieces of its cytoplasm. |
| What is the lifetime of a red blood cell? | 120 days |
| What is the first stage in the life cycle of a red blood cell? | Kidneys respond to a lower than normal oxygen concentration in the blood by releasing the hormones erythropoietin (EPO). |
| What happens in the life cycle of a red blood cell after EPO is released? | EPO travels to the bone marrow and stimulates an increase in the production of RBCs. |
| From where does the bone marrow manufacture RBCs? | From stem cells that live inside the marrow. |
| Where do RBCs go after being produced in the bone marrow? | They squeeze through blood vessel membranes to enter the circulation. |
| Which two organs work to supply continuous movement and oxygenation of RBCs? | The heart and lungs. |
| Which organ primarily destroys damaged or old RBCs? | The spleen. |
| What type of cell do all blood cells arise from? | Pluripotent stem cells (PPSC) |
| What do myeloid stem cells require? | A special environment provided by the cellular structure of the bone marrow, and at least 3 hematopoietic growth factors. |
| What are 3 hematopoietic growth factors? | Interleukin 3 (IL-3), Granulocyte-macrophage colony stimulating factor (GM-CSF), and Erythropoietin (EPO) |
| _____ and_____ are essential to the earliest stages of stem cell proliferation and differentiation. | IL-3, GM-CSF |
| _____ is essential for the successful proliferation and maturation of the CFU-E to adult RBCs. | EPO |
| 1 ___________ makes 16 RBCs and is committed to becoming an erythrocyte. | Pronormoblast |
| Progeny of each CFU-E stem cell enter a programmed sequence of development that involves __________. | 4 cell divisions |
| Where is hemoglobin formed? | Within the cell cytoplasm |
| What happens to the nucleus after the fourth division in RBC development? | It becomes pyknotic (gets dense and black and then get "kicked out" of the cell) and it lost from the cell |
| ___________ is an early red blood cell with RNA and mitochondria that continues to produce Hb for 2-3 days. | Marrow reticulocyte |
| After the marrow reticulocyte completes its synthesis of hemoglobin, what does it do next? | It crosses the sinusoidal wall to enter the circulation. |
| Blood reticulocytes still contain a small amount of what? | Residual RNA (can e identified as a reticulocyte for approx. 24 hrs) |
| The adult red blood cell lacks a ________ and _________. | Nucleus and mitochondria |
| What is the shape of an adult RBC? | biconcave disc |
| When you see __________ red blood cells on a blood smear, you know there is something wrong with that person. | Nucleated |
| Is an adult RBC a high or low energy cell? | Low energy |
| Is an adult RBC capable of extreme shape changes? | Yes. |
| An adult RBC is designed to survive what? | Many high-speed trips through the microvasculature. |
| A RBC, having engaged in oxygen and CO2 transport during its lifetime, beings to undergo __________ with time. | Senescence (still alive and functioning-just old) |
| What is the result of an aging RBC? | Less ATP generation by glycolysis, decreased amounts of cholesterol and phospholipids in the membrane, and loss of selective permeability that results in increased Na and decreased K from the cell. |
| What change in the RBC begins to attract IgG? | A change in shape-spherical rather than biconcave |
| When Methemoglobin reductase decreases what happens? | Methemoglobin accumulates (which is a non-functional form of iron in hemoglobin-cannot bind O2) |
| Erythrocyte disorders are traditionally divided into which two groups? | Anemia (too few RBCs or RBCs aren't working very well) and Polycythemia (erythrocytosis-too many RBCs) |
| Anemia is best characterized by what? | A hemoglobin concentration below normal. |
| Erythrocytosis is best characterized by what? | A hematocrit above normal. |
| Patients with anemia suffer from tissue _______, the consequence of a low-oxygen carrying capacity of blood. | Hypoxia |
| Anemia is defined as a clinical condition with a ______ in the total number of RBCs, a ________ in the concentration of hemoglobin, and a _______ in the hematocrit. | Decrease, decrease, decrease |
| Environmental causes of anemia include: | ingestion of toxic substances, radiation (like working in a dental office for example), or lack of essential nutrients |
| A pathological cause of anemia is: | From an infectious organism-like malaria |
| An iatrogenic cause of anemia is: | From the side-effects of legitimate medical treatment. |
| What is included in a CBC? | A Hb, HCT, RBC count, WBC count, and platelet count |
| In a CBC, are the WBCs differentiated? If so, what are their names? | Yes, neutrophils, lymphocytes, and eosinophils |
| What unit is hemoglobin measured in? | Grams/dL |
| How is HCT expressed? | As a percent (describes volume of packed RBCs that occupies a volume of blood) |
| What is a MCV? | Mean Corpuscular Volume-describes the volume of an average RBC in a specimen-this is essential in evaluating the cause of a particular anemia. |
| What is the formula for calculating the MCV (in fL)? | MCV = HCT(%) x 10/RBC count(x10^6/uL) |
| What is the normal MCV value in adults? | 80-96 fL |
| What is a MCH? | Mean Corpuscular Hemoglobin-describes the average amount of Hb in a single RBC-this is also helpful in evaluating the cause of a specific anemia. |
| What is the formula for calculating the MCH (in pg)? | MCH = HB(g/dL) x 10/RBC count(x10^6/uL) |
| What is the normal MCH value for adults? | 27-33pg |
| What is a MCHC? | Mean Corpuscular Hemoglobin Concentration-describes the average amount of Hb as a % of the volume of the RBC-this corrects for the size of the RBC in reporting the amount of cellular Hb. It is expressed in g/dL. |
| What are the two equations that can be used to find the value of MCHC? | MCHC(in g/dL) = MCH/MCV x 100 MCHC = Hb(g/dL)/HCT(as a decimal) |
| What are the normal values for MCHC? | 33-36g/dL |
| Normocytic anemias have: | normal size RBCs and a normal Hb content |
| Microcytic anemias have: | both the size and Hb content of these RBCs are smaller than normal |
| Macrocytic anemias have: | larger than normal RBCs, but the Hb content is normal |
| Iron is the _____ most common element on this planet. | 4th |
| What purpose does iron serve? | It catalyzes a number of enzymatic reactions involving electron transfer. |
| What is the predominant form of iron? | Insoluble, Fe(III) |
| Why must iron be compartmentalized? | Because it is very toxic (damages cell membranes and DNA) |
| At birth, how much iron have infants acquired from their mothers? | 350 mgs |
| Iron is distributed into what 4 main compartments at birth? | Operational iron, storage iron, labile iron, transport iron |
| Is there a physiological mechanism for iron excretion? | NO. |
| There is obligatory iron loss from: | Shedding in the intestinal and urinary tracts and by menstruation in women |
| What 3 factors help to maintain balance and availability of iron? | Reuse of erythrocyte iron, minimization of blood loss, and uptake and adequate quantities of iron from the diet (most meats, many vegetables and fruits, beans, and eggs) |
| Operational Iron: | (69%); Largest compartment, consists of iron attached to Hb and Mb |
| Storage Iron: | (30%); second largest compartment; mostly found in hepatocytes (liver), macrophages in bone marrow and spleen; iron is stored in ferritin |
| Labile Iron: | (1%); poorly defined compartment, iron bound to cell membranes, other coenzymes |
| Transport Iron: | (less than 1%); almost exclusively transferrin bound iron |
| Vertebrates have what 2 mechanisms for supplying their cells with a continuous and adequate flow of oxygen? | 1. Circulation system to deliver oxygen to cells/tissues and 2. Oxygen-carrying molecules to overcome the limitations imposed by the low solubility of oxygen in water |
| 98.5% of oxygen in blood is bound to _______. | Hemoglobin |
| What is the major function of erythrocytes? | To carry blood gases!! |
| Heme belongs to the class of pigments known as __________. | Porphyrins (cyclic tetrapyrroles) |
| Heme is composed of ___ pyrroles linked by _______ bridges each bound to a central _______ iron. | 4, methylene, ferrous |
| The heme group is called a _______. | Prosthetic Group |
| A protein without a prosthetic group is called an _____. | Apoprotein |
| Fe2+ in heme binds to 4 _______ in the center of the porphyrin ring. It can form _______ additional bonds. | nitrogens, 2 |
| Proteins consist of _______ chains of amino acids. | Linear |
| The synthesis of a peptide bond, the joining of two amino acids, is actually a _________ step, why? | Dehydration, because you lose a water molecule. |
| Primary structure of a protein: | number and sequence of amino acids |
| Secondary structure of a protein: | regular configuration of the chain, common secondary structures are alpha-helices and beta-strands |
| Tertiary structure of a protein: | overall shape of #D structure |
| Quaternary structure of a protein: | proteins with two or more chains |
| All proteins have which levels of structure? | primary, secondary, and tertiary |
| Myoglobin is a ______. It has how many oxygen(s)? | Monomer, 1 oxygen, no allosteric effect |
| Hemoglobin is a protein with ____ subunits (______ protein). | 4, tetrameric, it is the prototype of an allosteric protein |
| Myoglobin in muscle: | Acts as an oxygen-storing protein, it releases oxygen under SEVERE oxygen deprivation conditions such as during strenuous exercise. |
| Due to its allosteric nature, hemoglobin: | binds oxygen in lung and releases oxygen in capillaries of tissues as the oxygen tension changes. |
| The first hematopoietic organ in the human embryo is the: | yolk sac |
| The primary factor that regulates erythropoietic activity and production is: | oxygen |
| What is the composition of normal adult hemoglobin? | 96-98% HbA1; 1.5-3% HbA2; 0.5-0.8% HbF |
| What are the most common clinical signs of anemia? | fatigue, weakness, pallor, dyspnea, inability to concentrate |
| What hereditary X-linked disease affects >10% of the African-American male population, expressed as a hemolytic anemia especially during an infection/illness? | glucose 6-phosphate dehydrogenase (G6PD) deficiency |
| What is the primary risk to a thalassemia patient (patients with HbH disease or beta thalassemia major) who is regularly being transfused with RBCs? | Iron overload |
| What is the most common cause of iron deficiency in adults in the US? | Chronic blood loss |
| Ionizing radiation, viruses, antibiotic drugs and chemicals, and autoantibodies are all associated with what type of anemia? | Acquired type of aplastic anemia |
| What is the diagnostic value of a reticulocyte count in the evaluation of anemia? | determines response and potential of the bone marrow |
| A normal, functional hemoglobin molecule is composed of: | four heme rings, and four globin chains |
| MCV, MCHC, and Reticulocyte values for sever iron-deificiency anemia: | MCV=60, MCHC=30, Reticulocyte count=0.5 |
| MCV, MCHC, and Reticulocyte values for alpha-thalassemia with 3 gene deletion: | MCV=65, MCHC=30, Reticulocyte count=5.0 |
| MCV, MCHC, and Reticulocyte values for folic acid deficiency anemia: | MCV=104, MCHC=35, Reticulocyte count=1.0 |
| MCV, MCHC, and Reticulocyte values for acquired auto-immune hemolytic anemia: | MCV=94, MCHC=34, Reticulocyte count=7.0 |
| Macrocytic-normochromic corresponds to what type of anemia? | Megaloblastic anemia |
| Normocytic-normochromic corresponds to what type of anemia? | Cold agglutinin disease |
| Microcytic-hypochromic corresponds to what type of anemia? | Sideroblastic anemia |
| Involved in transporting of iron from digestive tract to bone marrow: | Transferrin |
| Involved in storing of iron in developing RBC in bone marrow: | Ferritin |
| What protein is being measured when total iron-binding capacity is increased in serum of IDA patients? | Transferrin |
| Incompatible with lief (that is, you are not born) | Neither sickle cell trait not beta-thalassemia minor |
| Considered a hemolytic anemia: | Both sickle cell trait and beta-thalassemia minor |
| Caused by mutations affecting transcription of the gene: | beta-thalassemia minor |
| What hematologist first described sickle cell anemia? | Herrick |
| What term best describes the volume of a red blood cell? | MCV |
| What term best describe tongue atrophy in a patient with a megaloblastic anemia? | glossitis |
| What term best describes the continued existence of sickle cell anemia and malaria? | Balanced polymorphism |
| What condition is a hallmark of aplastic anemia? | Panycytopenia |
| What is the final structure of the heme ring prior to the insertion of iron? | Protoporphyrin IX |
| Which of the following findings is the most specific for a diagnosis of alpha-thalassemia (3-gene deletion) in a patient who is symptomatic and anemic? | Detection of hemoglobin H (HbH) by electrophoresis |
| Interleukin-3: | is a hematopoietic growth factor that is non-lineage specific and would induce the formation of progenitor cells. |
| Oxygen binds normal hemoglobin (HbA1) because: | heme ring is surrounded mainly by hydrophobic AA, heme ring is derived from a porphyrin structure, by definition the heme group is termed a prosthetic group, and oxygen binding is between the distal histidine and iron in heme |
| Order of maturation in normal hematopoiesis goes from what cell type to what cell type? | Stem cell to progenitor cell to precursor (blast) cell to mature cell |
| What is true when comparing Hb to Mb? | Hb shows positive cooperativity of binding to oxygen, Mb is a monomer, Hb is a tetramer, Halene effect is where High O2 levels in lungs unloads CO2 and H from Hb, and Bohr effect shows that CO2 decreases the affinity of Hb for O2 |
| Red blood cells: | have a biconcave shape, does not have a nucleus, has a life span of 120 days, last immature cell is reticulocyte |
| 8-yr-old male with a persistent bacterial infection and frequent bleeding episodes. Played everyday this summer in the shed his father uses to store open containers of insecticides. A bone marrow biopsy is ordered, what anemia is suspected? | Aplastic anemia and hypoplastic marrow |
| 23-yr-old black female, history of lifelong anemia, non-healing ulcers, hand-foot syndrome, recurrent bouts of ab and chest pain. Which abnormality? | Sickle cell on peripheral blood smear |
| What are the AA substitutions found in sickle hemoglobin and in hemoglobin C respectively? | beta chain 6th position Glu-Val, and beta chain 6th position Glu-Lys |
| What is the hemoglobin defect found in both alpha and beta thalassemias? | Defective production of one of the globin chains |
| Hb carries O2 from _____ to ______, and CO@ in the reverse direction. | lungs, capillaries |
| Binding of oxygen to Hb ________ the binding of additional oxygen to the same Hb molecule. | Enhances. (in other words, oxygen binds cooperatively to Hb) |
| Hb binds ___ while it is delivering ___, and releases ___ when it is binding ___. | CO2, O2, CO2, O2 |
| Bohr Effect: | Hb binds oxygen efficiently in a high-pH environment and releases it when it encounters lower pH. |
| Oxygen affinity is modulated by: | 2,3-diphosphoglycerate (2,3-DPG), which results in Hb with a lower affinity for oxygen |
| Mb stores oxygen in ________ tissue, while Hb delivers oxygen throughout the body. | muscle |
| Mb has _____ of the regulatory properties of Hb. | None |
| Human myoglobin has ____ amino acid residues. | 153 |
| What else does Hb transport other than O2? | CO2 and H+ |
| Human Hb is comprised of 2 alpha chains and 2 beta chains, how many residues do each of these make up? | The 2 alpha chains are made up of 141 residues and the 2 beta chains are made up of 146 residues. The alpha and beta subunits associate more strongly with each other than similar subunits. |
| Each of Hb's subunits are structurally ________ to Mb. | Similar |