RED BLOOD CELL AND BLEEDING DISORDERS I

Question 1

effects of ____ are mainly due to the loss of intravascular volume , which if massive can lead to cardiovascular collapse, shock, and death

Answer 1

acute blood loss

Question 2

_________ induces anemia only when the rate of loss exceeds the regenerative capacity of the marrow or when iron reserves are depleted and iron deficiency anemia appears

Answer 2

chronic blood loss

Question 3

_______- anemias share the ff features:
1. a shortened red cell life span below the normal 120 days
2. elevated erythropoeten levels and a compensatory 3. increase in erythropoiesis

Answer 3

hemolytic anemias

Question 4

if persistent extravascular hemolysis leads to a hyperplasia of phagocytes manifested by varying degrees of ________

Answer 4

splenomegaly

Question 5

_________ is most commonly caused by alterations that make red cells less deformable

Answer 5

extravascular hemolysis

Question 6

________ hemolysis of red cells may be caused by mechanical injury, complement fixation, intracellular parasites, or exogenous factors

Answer 6

intravascular hemolysis

Question 7

iron released from hemogloblon can accumulate withink tubular cells giving rise to renal _________

Answer 7

hemosiderosis

Question 8

_________ is an inherited disorder caused by intrinsic defetc in the red cell membrane skeleton that render cells spheroid, less deformable, and vulnerable to splenic sequestration and destruction

Answer 8

hereditary spherocytosis

Question 9

in spherocytosis, _____ occurs in 40%-50% of affected adults , moderate _______ is characteristic (5000-1000 g)

Answer 9

cholelithiasis
splenomegaly

Question 10

hereditary spherocytosis

general stable clinical course is sometimes punctuated by ________, usually triggered by an acute parvovirus infection

Answer 10

aplastic crises

Question 11

hereditary spherocytosis

_______- are produced by intercurrent events leading to increased splenic destruction of red cells

Answer 11

hemolytic crises

Question 12

abnormalities in the _________ shut or glutatathione metabolism resulting from deficient or impaired enzyme function to reduce the ability of RBCs to protect themselves against oxidative injuries and lead to hemolysis

Answer 12

hexose monophosphate shunt

Question 13

G6PD

G6PD is present in about 10% of american blacks, mediterranean is present in middle east

high frequency of these variants in each population is believed to stem from protective effect against __________

Answer 13

plasmodium falciparum

Question 14

G6PD

exposure of G6PD deficient red cells to high levels of oxidants causes the cross linking of reactive sulfhydryl groups on globin chain which become denatured and form membrane bound precipitates known as _____________

Answer 14

heinz bodies

Question 15

__________ is a common hereditary hemoglobinopathy caused by a point mutation in beta globin that promots the polymerization of deoxygenated hemoglobin, leading to red cell distortion, hemolytic anemia, microvascular obstruction, and ischemic tissue damage

Answer 15

sickle cell disease

Question 16

sickle cell disease is a caused by a ______ mutation in the beta globin gene that leads to the replacement of a charged glutamate residue with a hydrophobic ______ residue

Answer 16

missense mutation
valine

Question 17

high prevalence of sickle cell trait in certain African populations stems from its protective effects against ________

Answer 17

falciparum malaria

Question 18

sickle cell anemia

in some individuals HbF expression remains relatively high, a condition known as ___________ in these individuals, sickle cell disease is much less severe

Answer 18

hereditary persistence of fetal hemoglobin

Question 19

Sickle cell anemia

_____________, higher HbS concentrations increase the probabilty that aggregation and polymerization will occur during any given period of deoxygenation

Answer 19

mean cell hemoglobin concentration

Question 20

sickle cell anemia

a ______ in pH reduces the oxygen affinity of hemoglobin, thereby increasing the fraction of deoxygenated HbS at any given oxygen tension and augmenting the tendency for sickling

Answer 20

intracellular pH

Question 21

Sickle cell anemia

________- also are present in red cells due to asplenia

Answer 21

howell jolly bodies

Question 22

sickle cell diseases causes a moderately severe hemolytic anemia (hematocrit 18%-30%) associated with _________, _______, and ________-

Answer 22

reticulocytosis
hyperbillirubinemia
presence of irreversibly sickle cells

Question 23

clinical features of sickle cell disease

_______ also called pain crises are episodes of hypoxic injury and infarction that causes severe pain in the affected region

these frequently manifest as the ______ or dactylitis of the bones of the hands and feet,

_________– is a particularly dangerous type of vaso-occlusive crisis involving the lungs that typically presents with fever, cough, chest pain, and pulmonary infiltrates

Answer 23

vaso-occlusive crises
hand foot syndrome
acute chest syndrome

Question 24

Sickle cell anemia

__________- stem from the infection of red cell progenitors by parvovirus

Answer 24

aplastic crises

Question 25

________ is a genetically heterogenous disorder caused by germline mutations that decrease the synthesis of either alpha globin or beta globin leading to anemia, tissue hypoxia, and red cell hemolysis related to the imbalance of globin chain synthesis

Answer 25

thalassemia

Question 26

Three major classes of beta thalassemia

1._________ - most common cause of beta thalassemia
2._________- mutations reduce transcription by 75%-80%
3.________ - most common cause of beta0 thalassemia

Answer 26

splicing mutations
promoter region mutations
chain terminator mutations

Question 27

beta thalassemia

it is estimated that 70%-85% of red cell precursors suffer this fate, which leads to _____________-

Answer 27

ineffective erythropoeisis

Question 28

beta thalassemia

the marked expansion of erythroid precursors leads to increased absorption of iron from the gut and this together with repeated blood transfusions inevitably lead to severe iron accumulation _____________

Answer 28

secondary hemochromatosis

Question 29

________- is most common in mediterranean countries. the red cells may completely lack HbA (Bo/Bo genotype) or contain small amounds of (B+/B+ or Bo/B+ genotypes_

Answer 29

B thalassemia major

Question 30

blood smears show severe red cell abnormalities, including marked variation in size ___________- and shape __________, ________, and hypochromia

Answer 30

anisocytosis
poililocytosis
microcytosis
hypochromia

Question 31

_________- thalassemia is caused by inherited deletions that result in reduced or absent synthesis of alpha globin chains

Answer 31

alpha thalassemia

Question 32

alpha thalassemia

________- is associated with deletion of a single alpha globin gene, which causes a barely detectable reduction in alpha globin chain synthesis

Answer 32

silent carrier state

Question 33

alpha thalassemia

caused by the deletion of two alpha globin genes from a single chromosome or the deletion of one alpha globin gene from each two chromosomes

Answer 33

alpha thalasemia trait

Question 34

alpha thalassemia

caused by deletion of three alpha globin genes

Answer 34

hemoglobin H disease

Question 35

alpha thalassemia

most severe
deletion of all four alpha globin genes

Answer 35

hydrops fetalis

Question 36

____________ is a disease that results from acquired mutations in the phosphatidylinositol glycan complementation group A gene (PIGA) an enzyme that is essential for the synthesis of certain membrane associated complement regulatory proteins

Answer 36

paroxysmal nocturnal hemoglobinuria

Question 37

________– is caused by antibodies that recognize red cells and lead to their premature destruction

Answer 37

immunohemolytic anemia

Question 38

immunohemolytic anemia type

constitutes approximately 80% of cases

caused by antigenic drugs and tolerance braking drugs

Answer 38

warm antibody type

Question 39

immunohemolytic anemia type

caused by IgM antibodies that bind to red cells avidly at low temperatured 0-4 degrees Celsius but not at 37 degrees Celsius

Answer 39

cold agluttinin type

Question 40

immunohemolytic anemia type

______ are autoantobodies responsible for unusual entity known as paroxysmal cold hemoglobinuria

Answer 40

cold hemolysin type

Question 41

_______ most significant hemolysis caused by trauma to red cells is seen in individuals with cardiac valve prosthesis and microangiopathic disorders

Answer 41

hemolytic anemia resulting from trauma to red cells