Hemo 2 Flashcards

Question

Anemia  Usually diagnosed based on: Inadequate numbers of erythrocytes (low hematocrit - Inadequate level of hemoglobin –

Answer 1

the ratio of packed red cells to total blood volume) the hemoglobin concentration of the blood

Answer 2

RBC destruction RBC production Blood

Answer 3

Pallor – pale skin and mucosa Lethargy – lack of energy, fatigue, weakness Dyspnea – labored breathing, SOB Tachycardia, arrhythmia, chest pain Koilonychia - spoon-shaped nails Atrophic glossitis Cognitive problems, dizziness Cold extremities Headache

Answer 4

 Fatigue  Increased heart rate  Shortness of breath / increased respiratory rate  Low blood pressure  Pale Skin  Central nervous system  Cardiac failure can develop and compound the tissue hypoxia caused by the deficiency of O2 in the blood

Answer 5

a person with a low hematocrit cannot carry enough oxygen in the blood to meet energy demands. Weakness, malaise, and easy fatigability.

Answer 6

compensates for the low oxygen carrying capacity of the blood

Answer 7

compensates for the poor delivery of oxygen to the tissues. Dyspnea on mild exertion.

Answer 8

a decrease in blood viscosity directly lowers total peripheral resistance to the flow of blood, thus lowering mean arterial blood pressure

Answer 9

hemoglobin is bright red when oxygenated and less red when deoxygenated. Because the redness of skin is due to the redness of blood, the skin of an anemic person (who has less oxygen in the blood) will be less red (paler) than the average person

Answer 10

hypoxia can cause headache, dimness of vision, and faintness

Answer 11

Anemias of Increased Blood Destruction Sickle cell anemia Thalassemia Erythroblastosis fetalis Hereditary spherocytosis G6PD deficiency Paroxysmal nocturnal hemoglobinuria Autoimmune hemolytic anemia Mechanical trauma to red cells Malaria

Answer 12

 A hemoglobinopathy  Inherited, mis-sense mutation of beta chain  A single AA substitution of valine for glutamic acid  Forms a new, abnormal hemoglobin, Hemoglobin S - HbS  Sickle cell disease – homozygous HbS  Sickle cell trait - heterozygous, a less serious condition

Answer 13

 Individuals with sickle cell trait (heterozygous for HbS) have a survival advantage in malaria-endemic areas  About 8% of African Americans are heterozygous (sickle cell trait)  1 in 600 African Americans are homozygous (sickle cell disease)

Answer 14

increased mortality due to malaria

Answer 15

survival advantage

Answer 16

increased mortality due to sickle cell disease

Answer 17

 HbS molecules polymerize when deoxygenated, forming HbS aggregates  Cytosol changes from a freely-flowing liquid to a viscous gel  With continued deoxygenation, HbS aggregates form long, needle-like fibers that distort the red cell shape  Sickle cell trait

Answer 18

HbA interferes with HbS polymerization in the heterozygous condition  Red cells do not sickle except under conditions of profound hypoxia

Answer 19

 Hemolytic anemia  Microvascular occlusions  Vaso-occlusive crises (pain crises)  Commonly involved sites: bone, lung, liver, brain, spleen  Autosplenectomy

Answer 20

chronic hemolysis – and jaundice – phagocytosis in spleen

Answer 21

sickle cells becone arrested during transit through the microvasculature

Answer 22

episodes of hypoxic injury and infarction that cause severe pain in the affected region

Answer 23

Increased susceptibility to infection with encapsulated organisms Pneumococcus pneumoniae Hemophilus influenzae

Answer 24

Thalassos = sea “Mediterranean” anemia Group of inherited diseases Quantitative problem - too few globins synthesized Underproduction of normal globin proteins due to mutations in regulatory genes

Answer 25

 Ineffective production of globin chains -Alpha globin chains (α Thalassemia) -Beta globin chains (β Thalassemia)  Regular transfusions – iron overload – organ damage  Bone deformities – expansion of marrow space  Splenomegaly - splenectomy  Impaired growth  Bone marrow transplantation

Answer 26

Two genes involved in making beta chain (one from each parent) Severity depends on number of affected beta chain genes One gene Two genes

Answer 27

beta-thalassemia minor - beta-thalassemia trait Mild disease

Answer 28

beta-thalassemia major (Cooley’s anemia) Severe disease

Answer 29

 Four genes involved in making alpha chain (two from each parent)  Severity depends on number of affected alpha chain genes

Answer 30

asymptomatic carrier alpha-thalassemia minor - alpha-thalassemia trait - Mild disease hemoglobin H disease -Moderate to severe disease alpha-thalassemia major – (lethal)

Answer 31

ABO mismatch leads to intravascular hemolysis Antibody-coated erythrocytes destroyed by both complement-mediated lysis and by phagocytosis in spleen

Answer 32

45% 42% 10% 3%

Answer 33

A, B, AB, O Rh+, Rh-

Answer 34

A pos, A neg, etc.

Answer 35

 Rh- mom  Rh+ fetus  Fetal RBCs cross the placenta and enter the maternal circulation during birth trauma  Prophylactic anti-Rh (D) immune globulin (Rhogam) within 72 hours of delivery  Rhogam lyses fetal RBCs in the maternal circulation – effectively removing any available antigen, so the mom doesn’t develop anti- Rh antibodies

Answer 36

 Rh- mom, with anti-Rh from prior pregnancy  Rh+ fetus  Anamnestic response rapidly produces anti-Rh (IgG)  Anti-Rh IgG crosses placenta and lyses fetal RBCs  Rh-mediated hemolytic disease

Answer 37

Immunoglobulin Administered to Rh-negative women after pregnancies in which they carried Rh-positive fetuses Anti-D antibodies

Answer 38

 Rh incompatibility  Hemolytic anemia in utero  Rh-negative mother develops antibodies against Rh-positive erythrocytes of fetus  Antibodies cross placenta and hemolyze fetal erythrocytes  High levels of bilirubin and biliverdin  Deposition in developing teeth  Only primary teeth affected

Answer 39

 Anemia caused by immune destruction of erythrocytes  Erythroblasts in peripheral blood  Hyperbilirubinemia  Kernicterus (bilirubin encephalopathy) if bilirubin reaches a high levels  Developmental dental defects reported - Atasu M, Genc A, Ercalik S, Enamel hypoplasia and essential staining of teeth from erythroblastosis fetalis, J Clin Pediatr Dent., 22(3):249-52, 1998

Answer 40

 X-linked disease; most common human enzyme defect (African-American male population)  Most are asymptomatic; at risk for non-immune hemolytic anemia upon exposure to oxidative stress  Oxidative stress: infections, drugs (aspirin)  G6PD / NADPH / Glutathione pathway - maintains supply of reduced glutathione to scavenge free radicals (anti-oxidant)  Red cells sustain damage from oxidizing free radicals (phagocytosed in spleen)  All individuals with Favism (hemolysis due to Broad Beans(fava)) are G6PD deficient  Survival advantage in Malaria endemic environments

Answer 41

Protozoal disease – primarily Plasmodium falciparum Female Anopheles mosquito vector, human reservoir Reproduction in red cells – showers of organisms produce shaking, chills and fever Hemolytic anemia High morbidity, mortality

Answer 42

Iron deficiency anemia - microcytic, hypochromic Anemia of chronic disease Sideroblastic anemia Pernicious anemia (B12 deficiency) - macrocytic Folic acid deficiency anemia - macrocytic Aplastic anemia Myelophthisic anemia

Answer 43

 Most common anemia in US  Lack of Fe most common nutritional deficiency in the world  Microcytic, hypochromic  Seen most often in females – more iron lost in menses than replaced by nutrition  Treated with iron supplements  When in males, suspect internal bleeding

Answer 44

6.2 (nl: 12-16 g/dl)

Answer 45

Dietary lack Impaired absorption Increased requirement Chronic blood loss

Answer 46

breast feeding

Answer 47

Males - peptic ulcer disease Females - menorrhagia or pregnancy

Answer 48

Colonic polyps / colon adenocarcinoma in Western world Hookworm infection in developing world

Answer 49

 Malnutrition, malabsorption, gastrectomy

Answer 50

reflects iron stores in bone marrow macrophages and liver

Answer 51

measure of transferrin molecules in blood

Answer 52

percent of transferrin molecules bound by iron (nl = 33%)

Answer 53

measure of iron in the blood

Answer 54

Scandinavian, Northern European women Severe Fe-deficiency anemia Mucosal atrophy - atrophic glossitis Esophageal webs - dysphagia Increased risk for squamous cell carcinoma

Answer 55

Esophagus Oropharynx Posterior Oral Cavity

Answer 56

Pernicious anemia (B12 deficiency) Folic acid deficiency anemia

Answer 57

intrinsic factor, which is secreted by the parietal cells of the stomach

Answer 58

Intrinsic Factor complex absorbed in the ileum

Answer 59

Autoimmune disease Not due to dietary deficiency of B12 A form of megaloblastic anemia caused by autoimmune gastritis and failure of intrinsic factor production leading to vitamin B12 deficiency Loss of ability to absorb Vitamin B12 Vitamin B12 (cobalamin) required for normal folate metabolism and DNA synthesis

Answer 60

Megaloblastic anemia Dietary deficiency of folic acid Folate required for DNA synthesis

Answer 61

 Marrow aplasia secondary to supression of multipotent myeloid stem cells (erythrocyte, leukocyte and thrombocyte series), resulting in pancytopenia  May be caused by known myelotoxic agents (eg. whole body radiation)  Pathogenesis may involve T cell attack on myeloid stem cells  Prognosis unpredictable  Transfusion, bone marrow transplant

Answer 62

 Antineoplastic drugs (alkylating agents, antimetabolites)  Benzene  Chloramphenicol

Answer 63

Gastrointestinal bleeding Hemoptysis (coughing up blood) Epistaxis (nosebleed) Hematuria (blood in urine) Menstrual blood loss

Answer 64

Hematemasis (vomiting blood) Melena (black stool) Hematochezia (red blood in feces).

Answer 65

Menorrhagia (excessive bleeding) Metrorrhagia (irregular bleeding).

Answer 66

Screening test for occult bleeding in GI tract

Answer 67

 An increase in the RBC mass  Relative polycythemia – dehydration – decreased plasma volume with normal red cell mass  Absolute polycythemia – a true increase in red cell mass

Answer 68

 Erythropoietin-independent  Acquired, clonal stem cell disorder (a chronic myeloproliferative disorder)

Answer 69

 Erythropoietin-dependent  Compensatory response to tissue hypoxia

Hemo 2 Flashcards

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