RBC Anemias Patho Test 3
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Created by:
monkey93063 on October 7, 2010
Classes:
Clin Med, Principles of Clinical Medicine I - k, Medical Physiology/ Pathophysiology, Penn College PA program 2012
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162 terms
Terms | Definitions |
|---|---|
 Normal Erythropoiesis path | kidney secretes erythropoietin and liver secretes iron, then the bone marrow secretes RBC that help with O2 and energy |
| Hemoglobin molecule consists of | alpha chain, beta chain, iron, and a heme group |
| reduction in oxygen carrying capacity of the blood (results from reduction of total circ. Red cell mass) | Anemia |
| anemia results from | massive bleeding, inc. RBC destruction, decreased RBC production |
| anemia due to acute bleeding or increased RBC destruction | hemolysis |
| anemia due to decreased RBC | reticulocytopenia |
| 3 cell sizes | normocytic, microcytic, macrocytic |
| 2 degrees of hemoglobinization | normochromic, hypochromic (lacking) |
| RBC morphology correlates directly with | etiology of deficiency |
| MCV is | mean cell volume average volume per red cell |
| MCH is | average content of hemoglobin per red cell |
| MCHC is | mean cell hemoglobin concentration average concentration of hemoglobin in a given VOLUME of packed RBC's |
| RDW is | red cell distribution width (coefficient of variation of red cell volume) |
| common clinical features of all RBC anemias | pallor, fatigue, weakness |
| Common symptoms in hemolytic anemias * | hyperbilirubinemia, jaundice, pigment, gallstones |
| elevated iron levels and premature death of erythroid progenitor cells in bone marrow are significant of ______ | anemia from inadequate hematopoesis |
| In acut hemorrhagic anemia you must ____ | worry about hypovolemia (shock) first then the anemia |
| acute hemorrhagic anemia is | normocytic/normochromic |
| acute hemorrhagic anemia you see _____ | an inc. in erythropoietin level as RBC production inc. (reticulocytosis in marrow) |
| in chronic hemorrhagic anemia there is | a gradual loss of iron stores |
| iron is needed for | hemoglobin synthesis and erythropoiesis |
| chronic hemorrhagic anemia is | a chronic anemia of underproduction |
| Types of hemolytic anemia | hereditary spherocytosis, sickle cell anemia, thalassemia, G6PD Deficiency, immunohemolytic anemia, trauma |
| RBC life span is appox. | 120 days |
| accelerated destruction of RBC's is | hemolytici anemia |
| All hemolytic anemias have the following | inc rate of RBC destruction, increase in erythropoiesis, retention of RBC breakdown material (iron, etc.) |
| hemolysis causes_____ | hemoglobinemia, homoglobinuria, hemosiderinuria |
| hemolytic anemias convert heme of pigment to | bilirubin creating unconjugated hyperbilirubinemia and jaundice |
| intravascular hemolytic anemia is often the result of | mechanical trauma or physical agents (heart valve, heat) |
| extravascular hemolytic anemia is often the result of | phagocytotic cells in liver and spleen (more common than intravascular) |
| Most hemolytic anemias cause _____ | splenomegaly |
| inherited, intrinsic, defect in RBC membrane causing spherical shape | hereditary spherocytosis |
| hereditary spherocytosis is vulnerable to | sequestration by the spleen, phagocytosis/destruction |
| hereditary spherocytosis is an _______ trait | autosomal dominant |
| 25% of pts with hereditary spherocytosis have a ______ | different more severe autosomal recessive trait |
| mutation that weakens the vertical interactions of the membrane skeleton of RBC's and the membrane proteins | hereditary spherocytosis |
| hereditary spherocytosis has mutations in | ankyrin, band 3 and spectrin |
| _____ is primary destructor of spherocytes in hereditary spherocytosis | spleen |
| why does the spleen destroy RBC's in hereditary spherocytosis | abnormally shaped cells can't change shapes to move through splenic sinusoids thus are destroyed by phagocytes |
| with hereditary spherocytosis anemia corrects with | a splenectomy (though red cell defects and spherocytes persist |
| spherocytes in hereditary spherocytosis lack _____ | central pallor |
| You will have anemia, splenomegaly, jaundice, and you may see aplastic crisis. A marrow infection causes transient pause in RBC production. What disease causes this? | Hereditary spherocytosis |
| What is the primary treatment for hereditary spherocytosis? | Splenectomy |
| What is the most common hemoglobinopathy? | Sickle cell anemia |
| What diease causes a mutation in B-globin chain gene that creates HbS? | Sickle cell anemia |
| What happens to the HbA in homozygotes for SCA in sickle cell anemia? | All of the HbA is replaced by HbS |
| What happens to the HbA in heterozygotes for SCA in sickle cell anemia? | only half of the HbA is replaced by HbS |
| 8% of _______ are heterozygous for HbS ( 1:600) | American blacks |
| Why is the presence of sickle cell anemia so predominant in blacks? | Because of areas with high spread of malaria like in africa |
| What is HbS slightly protective against? | Plasmodium falciparum malaria |
| What is the most common form of familial hemolytic anemia? | Sickle cell anemia |
| What happens when HbS is exposed to deoxygenation? | The polymer particles distort the shape of the RBC into a sickle shape |
| What happens when sickling is reoxygenated | it is irreversible |
| For sickle cell anemia what do the cells accumulate and what other thing do the cells lose? | They accumulate calcium and you lose potassium |
| What is so different about some stages of sickle cell? | Some stages are reversible! |
| Sickle cells are more _____? | STICKY |
| What affects sickling of RBC's? | The concentration of HbS |
| Where is sickling ususally confined to? | Sluggish blood flow |
| What are the major consequences of sickle cell? | Membrane damage and dehydration of the RBCs |
| What removes the damaged cells? | Mononuclear phagocytes |
| Microvascular obstruction is _____? | Diffuse |
| At what time are the homozygous cases usually noticed? | After 6 months of age |
| What are some problems that come along with sickle cell anemia? | Marked reticulocytosis, hyperbilirubinemia, vaso-occlusive pain crisis, and aplastic crisis |
| Most common in bone marrow > infarction and necrosis. You need to worry about acute chest syndrome, pulmonary infections or fat emboli from marrow necrosis. You also need to worry about pulmonary and systemic hypoxemia or CNS stroke. What are we discussing here? | Vaso-occlusive pain crisis |
| What is aplastic crisis? | Severe anemia due to temporary cessation of erythropoiesis is seen mostly with parvovirus B19 infections. |
| What do most patients need to have done in adulthood if they have sickle cell anemia? | Autosplenectomy |
| What are sickle cell anemia patients more prone to? | Infections |
| How is the diagnosis of sickle cell anemia official? | Hemoglobin electrophoresis (+) for HbS |
| Mutations lead to decreased rate of a- or B- globin chain synthesis. What disease causes this? | Thalassemia |
| What 4 chains is adult HbA made up of? | 2 a- chains (chromosome 11) and 2 B- chains (chromosome 16) |
| What are the 3 types of Thalassemia? | Major, Minor, and Intermedia |
| This type of thalassemia inherits only one abnormal allele what type is it? | Thalassemia minor |
| This type of thalassemia inherits both abnormal alleles what type is it? | Thalassemia major |
| What is rarely associated with gene deletions? | Thalassemia |
| What are the 2 conditions that cause the pathogenesis of beta thalassemia? | Reduced synthesis of B-globin > inadequate HbA, and red cell hemolysis |
| What causes the reduced synthesis of B-globin > inadequate HbA? | MCHC low, microcytic/hypochromic |
| What causes RBC hemolysis? | Unbalanced rates of B-globin and a-globin chain synthesis. The excess a-chains cause RBC damage. |
| What clincally happens to patients with B-Thalassemia Major? | Children have growth retardation, and all patients live only with repeated blood transfusions. |
| What clinically happens to patients with B-Thalassemia Minor? | Mild microcytic, hypochromic anemia. These pts live normal life spans. You need to rule out Iron Deficiency Anemia |
| What treatments are given to pts with b-thalassemia major? | Blood transfusions and bone marrow transplants |
| Most caused by genetic deletions that remove the a-globin gene loci. This is DIFFERENT FROM B-THALASSEMIA!! The disease severity correlates with number of deletions. What disease are we talking about? | A-Thalassemia |
| The G6PD Gene is on X Chromosome. THe deficiency leads to reduced ability of RBC's to protect themselves from oxidative injury, and there are over 400 varieties. What disease are we discussing? | Glucose-6-Phosphate Dehydrogenase Deficiency |
| What disease is asymptomatic until exposed to factor that leads to increased oxidative stress? | Glucose-6-phosphate dehydrogenase deficiency |
| What can cause the symptoms to occur with G6PD deficiency? | Drugs and Infections |
| What kind of drugs cause G6PD deficiency? | Antimalarials, sulfonamides, nitrofurantoin, aspirin, vitamin K products |
| What kinds of infections cause G6PD deficiency? | Phagocytes at work produce free radicals (I.e. hydrogen peroxide) |
| What causes oxidized Hb to become denatured and form precipitates? | G6PD deficiency |
| Carrier females of G6PD are ____? | Asymptomatic |
| What happens when the drug induced G6PD occurs? | Development of hemolysis evidence after 2-3 days and all male cells affected as gene on X chromosome |
| Antibodies bind to RBC surface antigens. What diseases are being described here? | Immunohemolytic anemias |
| What diseases are classified by warm and cold antibody types? | Immunohemolytic anemias |
| Repeated physical blows like marathon running, bongo drumming, and mechanical heart valves all can affect the body because of what disease? | Traumatic hemolytic anemia |
| What blood disorder causes DIC, and malignant hypertension? | Microangiopathic hemolytic anemia |
| What blood disorder has burr cells, helmet cells, triangle cells? | Traumatic hemolytic anemia |
| Iron Deficiency Anemia, Anemia of Chronic Disease, Megaloblastic Anemia, Folic Acid Deficiency, Vitamin B12 Deficiency, and Aplastic Anemia are all types of ______? | Anemias of diminished erythropoiesis |
| What is the most common form of nutritional deficiency? | Iron Deficiency Anemia |
| 80% of functional iron is found in what? | hemoglobin |
| Iron storage pool contains 15-20% of total body iron, Name the tissues/organs? | Liver, spleen, bone marrow, skeletal muscle |
| Serum Ferritin (blood test) is used to determine what? | Represents storage pool supply |
| What's the function of transferrin? | Transports iron in plasma |
| Iron balance maintained mostly by ___________? | Dietary Intake |
| Iron is absorbed in which part of the intestines? | Duodenem |
| What are the 4 causes (etiologies) of Iron Deficiency Anemia | 1. Low Dietary Intake 2.Malabsorption 3. Increased demand (matched w not enough intake) 4. Chronic Blood Loss |
| IRON DEFECIENCY ANEMIA (Fill in the Blanks) COURSE of Disease: Insidious, First__________Decreased ______ _______ (blood test) Then see, Decrease in ______ _______ | ... |
| Rise in serum iron binding capacity | ... |
| Typically asymptomatic | ... |
| May see weakness, pallor, fatigue | ... |
| Spooning of nails, Pica | First iron storage pool depleted Decreased |
| serum ferritin | ... |
| Insidious | ... |
| First iron storage pool depleted | ... |
| Decreased serum ferritin | ... |
| Then see, | ... |
| Decrease in serum iron | ... |
| Rise in serum iron binding capacity | ... |
| Typically asymptomatic | ... |
| May see weakness, pallor, fatigue | ... |
| Spooning of nails, Pica | ... |
| MOST OF THE TIME...in a reasonably nourished person -Iron deficiency anemain this anemia is a symptom of another ______ _______. | underlying cause |
| Most common form of anemia in hospitalized patients | Anemia of Chronic Disease |
| What are some chronic microbial diseases that can cause "Anemia of Chronic Disease"? | Osteomyelitis, bacterial endocarditis, lung abscess |
| What type of anemia looks like Iron deficiency disease BUT Comes from inflammation-induced sequestration of iron within cells of mononuclear phagocytic system | Anemias of Chronic Disease |
| What are some Neoplasms that can cause "Anemias of Chronic Disease"? | Hodgkin's lymphoma, carcinomas of lung and breast |
| What Chronic Immune Disorder can result in anemia? | Rheumatoid Arthritis |
| In anemias of chronic disease you have Increased iron storage in??? | bone marrow |
| Anemias of chronic disease, Increased serum ferritin And Reduced >>> what? | total iron-binding capacity |
| NAME THAT ANEMIA! Transfer of iron to erythroid precursors is blocked by secondary effects of inflammatory cytokines | Anemias of Chronic Disease |
| Which anemia has a deficiency in Folate & B12 | Megaloblastic Anemia |
| Folate and B12 are both required for what? | Both required for DNA synthesis, thus effects on hematopoiesis are similar |
| enlarged megaloblasts (erythroid precursors) abnormally large red cells. This is the hallmark sign of what type of anemia? | Megaloblastic anemia |
| In which anemia do most patients develop pancytopenia ? | Megaloblastic anemia |
| as other myeloid lineages are affected -> granulocyte precursors -> hypersegmented neutrophils. This is called what? | Pancytopenia |
| What 3 things does pancytopenia cause? | Anemia, thrombocytopenia, granulocytopenia |
| Folate is transported in blood as what? | monoglutamate |
| A metabolite, tetrahydrofolate, is an integral building block for DNA synthesis, what is being described? | monoglutamate |
| PRESENTATION: Insidious, non-specific symptoms. May see other dietary deficiencies - esp. in alcoholics. NO NEUROLOGIC ANOMALIES! What deficiency results in the presentation described above? | Folic Acid Deficiency |
| Macrocytic anemia can be caused by what deficiency? | Folic Acid Deficiency |
| What refers to defective production of Intrinsic Factor from stomach, Intrinsic Factor is necessary for Vit. B12 absorption | Pernicious Anemia |
| Demyelination of posterior and lateral columns of spinal cord. This can occur in what type of deficiency? | Vitamin B 12 deficiency |
| Vitamin B 12 can cause which two anemias (just like Folate deficiency)? | May cause megaloblastic, macrocytic |
| Autoantibodies to parietal cells and intrinsic factor itself, resulting in Gastral Mucosal Atrophy, What type of anemia is this? | Pernicious Anemia |
| Vitamin B12 is needed to "recycle" what? | tetrahydrofolate |
| a deficiency in B12 means a deficiency in the form of ________ needed to build DNA | folate |
| Vit. B12 deficiency anemia improves with _______ administration | folate |
| administration of folates in the setting of Vit B12 deficiency may exacerbate __________ involvement | neurological |
| Neurologic symptoms include: Symmetric numbness, tingling, burning in feet/hands & | ... |
| Unsteadiness of gait and loss of position sense, these are the neuro symptoms for what deficiency? | Vitamin B 12 deficiency |
| Besides neuro symptoms, give me the 3 "clinical features" of Vitamin B 12 deficiency. | Fatigue, pallor, weakness |
| Whats the treatment for vitamin B 12 deficiency, what doesn't resolve regardless of treatment? | Anemia responds to parenteral Vit. B12; but neuro symptoms won't resolve. |
| Low serum B12 | ... |
| Normal/elevated Folate levels | ... |
| Serum antibodies to intrinsic factor | ... |
| Moderate to severe megaloblastic anemia | ... |
| Leukopenia with hypersegmented granulocytes | ... |
| Dramatic reticulocyte response to parenteral Vit. B12 | ... |
| These are the diagnostic features for what? | Vitamin B 12 deficiency |
| Multipotent Myeloid Stem Cell are suppressed | ... |
| Leading to marrow failure and Pancytopenia, what anemia is this? | Aplastic Anemia |
| Pathogenics not known very well for aplastic anemia, it's idiopathic, etc. but what do we know happens, considering pathogenics for aplastic anemia? | Autoreactive T cells attack marrow stem cells |
| Anemia primarily identified by what lab results? | decreased Hgb and Hct |
| Several classifications; look for mis-shapened erythrocytes; this is true for what type of anemia? | Aplastic Anemia |
| Shistocytes, spherocytes, sickles this are types of misshapened erythrocytes seen in what type of anemia? | Aplastic anemia |
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