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red blood disorders Flashcards

1
Q

clinical presentation of anemia

A

weakness, fatigue, dyspnea, pale conjunctiva and skin. angina. HA and lightheadedness.

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2
Q

most common anemia

A

iron-deficient microcytic

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3
Q

lab findings of iron deficient anemia

A

microcytic hypochromic anemia. decreased ferritin, increased TIBC, decreased serum iron, decreased saturation. increased free erythrocyte protoporphyrin

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4
Q

plummer-vinson syndrome

A

esophageal web, atrophic glossitis. presents as anemia, dysphagia, and beefy-red tongue.

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5
Q

anemia of chronic disease

A

associated with chronic inflammation or cancer. most common type in hospital patients. increased acute phase reactants from the liver such as hepcidin.

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6
Q

hepcidin

A

sequesters iron into storage sites. limiting iron from macrophages to erythroid precursors. prevent bacteria from assessing iron which is necessary.

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7
Q

labs for anemia of chronic disease

A

increased ferritin, decreased TIBC, decreased serum, decreased saturation. increased free erythrocyte protoporphyrin

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8
Q

sideroblastic anemia

A

due to defective protoporpyrin synthesis. there will be decreased heme, Hb, and a microcytic anemia

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9
Q

what is classically seen in sideroblastic

A

iron laden mitochondria. forms a ring around nucloid precursors.

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10
Q

congenital sideroblastic anemia

A

deficiency in ALAS

aminolevulinic acid synthase

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11
Q

acquired sideroblastic

A

alcoholism, lead poisoning, vitamin B6 deficiency (required for ALAS) most commonly seen in isoniazid treatment for TB.

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12
Q

lab findings in sideroblastic anemia

A

increased ferritin, decreased TIBC, increased serum iron, increased saturation.

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13
Q

thalassemia

A

anemia due to decreased synthesis of globin chains of Hb. this is an inherited mutation.

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14
Q

clinical findings of anemia

A

anemia, koilonychia (spoon nails), pica

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15
Q

one gene deletion in thalassemia

A

asymptomatic.

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16
Q

two genes deleted in thalassemnia

A

mild anemia with increased RBC count.

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17
Q

cis deletion for thalassemia

A

increased risk for severe thalassemia in offspring. seen in asian

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18
Q

trans deletion of thalassemia

A

africans.

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19
Q

three genes deleted in thalassemia

A

severe anemia. the beta-chains form tetramers and damage RBCs. HbH is seen on electrophoresis.

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20
Q

four gene deletion in thalassemia

A

lethal in utero. hydrops fetalis gamma chains form tetramers Hb barts that damage RBCs.

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21
Q

beta thalassemia

A

seen in african or Mediterranean descent.

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22
Q

beta thalassemia minor

A

mildest form, asymptomatic with increased RBC counts microcytic hypochromic. shows slightly decreased HbA and increased HbA2. there will be HbF

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23
Q

beta thalassemia major

A

most severe form. high HbF (alpha2, gamma2), unpaired alpha chains precipitate and damage the RBC membrane. resulting in ineffective hematopoiesis and extravascular hemolysis.

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24
Q

what does the body do to try and correct for the anemia

A

there is massive extramedullary hemolysis. chipmunk facies, crew cut

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25
Q

how do we treat beta thalassemia major

A

transfusion leads to hemachromatosis

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26
Q

what does the smear look like for beta thalassemia major

A

hypochromic microcytic anemia with target cells.

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27
Q

most common causes of macrocytic anemia

A

folate or vitamin B12 deficiency

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28
Q

most common cause of vit B12 deficiency

A

pernicious anemia. lack of intrinsic factor

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29
Q

parietal cells

A

pink, produce acid, pernicious anemia.

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30
Q

other causes of B12 deficiency

A

pancreatic insuficiency (no enzymes to cleave B12 from R-Binder). damage to ileum (crohn’s) or diphyllobothrium latum.

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31
Q

what causes the glossitis in macrocyctic anemia

A

lack of turnover in the tongue cells

32
Q

what happens to the spinal cord in B12 deficiency

A

subacute combined degeneration of the cord. this is caused by increased methylmalonic acid in the myelin of the spinal cord

33
Q

what is the normal metabolism of methylmalonic acid

A

goes to succinyl CoA

34
Q

reticulocyte

A

blue, slightly larger than RBCs. they are blue due to RNA

35
Q

what happens to the number of reticulocytes during anemia

A

it is falsely elevated. needs to be corrected by multiplying by patients Hct/normal Hct)

36
Q

what system mediates extravascular hemolysis

A

reticuloendothelial system

37
Q

clinical findings of extravascular hemolysis

A

anemia, splenomegaly, jaundice, increased bilirubin gallstones, marrow hyperplasia.

38
Q

haptoglobin

A

binds Hb that is free in the blood. low haptoglobin is a response due to binding

39
Q

intravascular hemolysis

A

hemaglobinemia, hemoglobinuria, hemosiderinuria, decreased serum haptoglobin

40
Q

low haptoglobin

A

tells us there is some intravascular hemolysis

41
Q

hereditary spherocytosis

A

inherited defect in the cytoskeleton membrane tethering proteins. membrane blebs are lost over time causing the cells to become round. consumed by the spleen.

42
Q

tethering proteins in hereditary spherocytosis

A

ankryin, spectrin, band 3.1.

43
Q

findings of spherocytosis

A

splenomegaly (hypertrophy), jaundice with uncongugated bilirubin. risk of gallstones.

44
Q

treatment for hereditery spherocytosis

A

splenectomy. the spherocytes will persist and there will be howel jolly bodies

45
Q

sickle cell anemia gene and protein consequences

A

AR mutation in the beta chain of globin. normal glutamic acid is replaced by valine (hydrophobic).

46
Q

who gets sickle cell

A

10% of africans. occurs when there is >90% of HbS

47
Q

HbS does what?

A

aggregates into needle-like structures that cause deformation of the cell.

48
Q

when does HbS polymerize

A

acidosis, dehydration, deoxygenation

49
Q

what is protective against sickling

A

HbF. this is why children dont get sickling until later in life

50
Q

dactylitis

A

swollen hands and feet due to vasoocculsive infarcts of the bone. common presenting sign in infants.

51
Q

autosplenectomy

A

due to vsoocclusive disease in sickle cell. shrunken fibrotic spleen. more susceptible to encapsulated organisms

52
Q

acute chest syndrome

A

vaso-occlusion in pulmonary microcirculation. chest pain, shortness of breath, lung infiltrates and pneumonia. most common cause of death in adults with sickle cell.

53
Q

sickle cell kidney manifestations

A

renal papillary necrosis. due to vaso-occlusive

54
Q

what percentage of HbS is necessary to sickle

A

50%. this is why people with trait dont usually have sickling.

55
Q

how do we get sickle cell trait to sickle

A

test with metabisulfite screen.

this causes any amount of HbS to sickle.

56
Q

HbC

A

has a lysine mutation in AR in beta chain Hb. this is less common than sickle cell. anemia due to extravascular hemolysis.

57
Q

what is the hallmark of HbC

A

there will be crystals on blood smear.

58
Q

paraxosymal nocturnal hemaglobinuria

A

acquired defect. absent GPI renders RBC susceptible to complement destruction. (there is no MIRL or DAF attached to the RBC). hemolysis occurs at night due to normal respiratory acidosis causing activation of complement.

59
Q

paraxosymal nocturnal hemaglobinuria presents

A

hemoglobinuria, hemoglobinemia, hemosiderinuria

60
Q

screening for PNH

A

lack of CD55, sucrose test that activates complement, acidified serum test

61
Q

main cause of death of PNH

A

thrombosis because the platelets also lack GPI

62
Q

what are two common associations with PNH

A

iron deficiency anemia, AML (10% of patients)

63
Q

G6PD deficiency

A

X-linked recessive disorder resulting in reduced half-life of G6PD renders cells susceptible to oxidative stress.

64
Q

what is required to regenerate glutathione

A

NADPH. G6PD generates NADPH. this is the etiology of intravascular hemolysis of G6PD def.

65
Q

african variant of G6PD def

A

mild form.

66
Q

Mediterranean form of G6PD def.

A

markedly reduced half-life and marked hemolysis.

67
Q

what are the causes of oxidative stress in G6PD

A

infections, drugs (primaquine, dapsone, sulfa), fava beans.

68
Q

what does the oxidative stress produce in G6PD def.

A

heinz bodies. leads to intravascular hemolysis. forms bite cells

69
Q

immune hemolytic anemia

A

can be IgG or IgM

70
Q

IgG mediated hemolytic

A

warm agglutinin, central body. extravascular hemolysis. results in spherocytes. associated with lupus, CLL and certain drugs (methyldopa).

71
Q

IgM mediated disease

A

extravascular hemolysis. colder extremities. fixes complement (MAC), associated with mycoplasma pneumoniae and infectious mono.

72
Q

direct coombs test to diagnose hemolytic anemia

A

looks for RBC that are bound by IgG agglutination.

73
Q

indirect coombs test

A

are there antibodies in the serum.

74
Q

microangiopathic hemolytic anemia

A

schistocytes. seen in TTP (ADAMTS13), HUS, DIC, HEELP. also in aortic stenosis or prosthetic heart valves.

75
Q

HEELP

A

hemolysis elevated liver enzymes and low platelet

76
Q

what transmits malaria

A

anopheles misquito. organisms in the RBC. RBC will rupture during the disease

77
Q

why does renal failure cause anemia

A

low erythropoietin

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