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Heme/Onc > Pathoma 5 > Flashcards

Pathoma 5 Flashcards

1
Q

heme = ?

A

iron + protoporhyrin

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2
Q

ddx microcytic anemia

A
  1. iron deficiency
  2. ACD
  3. sideroblastic
  4. thalassemia
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3
Q

Iron absorption occurs?

A

duodenum

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4
Q

Iron from duodenum into enterocyte through?

A

DMT1

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5
Q

iron from enterocyte to blood through

A

ferroportin

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6
Q

iron transporter in blood

A

transferrin

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7
Q

places where iron is stored

A

liver and BM macrophages

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8
Q

stored intracellular iron is bound to?

A

ferritin

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9
Q

normal iron % saturation

A

33% transferrin bound by iron

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10
Q 
microcytic hypochromic RBCs with inc RDW
Dec ferritin
Inc TIBC
Dec serum iron
Dec % saturation
A

iron-deficiency labs

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11
Q

anemia + dysphagia + beefy red tongue

A

Plummer Vinson Syndrome (iron deficiency + esophageal web + atrophic glossitis)

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12
Q

Hepcidin sequesters iron in storage sites by

A
  1. limiting iron transfer from macrophages to erythroid precursor
  2. suppressing EPO
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13
Q 
Inc ferritin
Dec TIBC
Dec serum iron
Dec iron sat
Inc FEP
A

Lab findings ACD

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14
Q

what causes sideroblastic anemia?

A

defective protoporhyrin synthesis

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15
Q

rate limiting step in protoporphyrin synthesis

A

ALAS-catalyzed S CoA –> ALA using vB6 as cofactor

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16
Q

enzymes in protoporphyrin synthesis

A

ALAS (B6) + ALAD (and others)

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17
Q

where is the iron in sideroblastic anemia

A

stuck in the macrophages (seen w/ prussian blue stain)

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18
Q

congenital sideroblastic anemia

A

usually ALAS defect

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19
Q

acquired sideroblastic anemia

A
  1. alcoholism
  2. lead poisoning (inhibits ALAD + ferochelatase)
  3. vB6 deficiency (isoniazid side effect)
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20
Q

inc ferritin
dec TIBC
inc serum iron
inc % sat

A

sideroblastic labs

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21
Q

HbF =

A

alpha2gamma2

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22
Q

HbA =

A

alpha2beta2

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23
Q

HbA2 =

A

alpha2delta2

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24
Q

how many alpha genes present on chromosome 16

A

four total

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25
Q

cis deletion (–> mild anemia w/ inc RBC count) in what population

A

Asian

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26
Q

trans deletion (–> mild anemia w/ inc RBC count) in what population

A

African

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27
Q

HbH =

A

beta4

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28
Q

What happens with three deleted alpha hemoglobin chains?

A

severe anemia; HbF seen on electrophoresis

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29
Q

What happens with four deleted alpha hemoglobin chains?

A

hydrops fetalis; Hb Barts = gamma4

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30
Q

beta thal in what populations

A

Mediterranean + African

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31
Q

alpha hemoglobin gene is on what chromosome?

A

16

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32
Q

beta hemoglobin gene is on what chromosome?

A

11

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33
Q

microcytic hypochromic RBCs and target cells

A

beta thal minor on smear

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34
Q

beta thal minor electrophoresis

A

slightly decreased HbA, increased HbA2 + HbF

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35
Q

beta thal major cause of damage

A

alpha tetramers –> ineffective erythropoiesis + extravascular hemolysis

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36
Q

what ID is really bad if you have beta thal major?

A

parvovirus B19

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37
Q

severe anemia a few months after birth; massive erythroid hyperplasia; extra medullary hematopoiesis w/ HSM

A

beta thal major symptoms

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38
Q

microcytic, hypochromic RBCs w/ target cells + nucleated RBCs

A

beta thal major smear

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39
Q

beta thal electrophoresis

A

HbA2 + HbF + little or no HBA

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40
Q

macrocytic anemia w/ megaloblastic change ddx

A

folate or B12 deficiency

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41
Q

macrocytic anemia w/o megaloblastic change ddx

A

alcoholism, liver dz, drugs (5-FU)

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42
Q

where is folate absorbed?

A

jejunum

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43
Q

causes of folate deficiency

A
  1. poor diet
  2. increased demand (pregnancy, cancer, hemolytic anemia)
  3. folate antagonists (methotrexate)
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44
Q

macrocytic RBCs and hypersegmented neutrophils

A

folate or B12 deficiency on smear

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45
Q

dec serum folate
inc homocysteine (inc risk for thrombosis)
nl methylmalonic acid

A

folate deficiency labs

46
Q

how to differentiate folate/b12 def?

A

methylmalonic acid

47
Q

where is B12 absorbed?

A

ileum (complexed w/ IF)

48
Q

absorption of iron, folate, b12

A

duodenum, jejunum, ileum

49
Q

how does b12 get through the stomach?

A

R-binder

50
Q

what detaches b12 and R-binder?

A

pancreatic proteases in duodenum

51
Q

what makes IF

A

gastric parietal cells

52
Q

b12 deficiency takes a long time to develop bc?

A

there are large hepatic stores

53
Q

5 causes of b12 deficiency

A
  1. pernicious anemia (AI destruction of parietal cells)
  2. pancreatic insufficiency
  3. Crohn dz
  4. D latum
  5. veganism
54
Q

what does vB12 do in the body normally?

A

cofactor for conversion of methylmalonic acid to succinyl Coa (important in fatty acid metabolism)

55
Q

B12 def –> inc methylmalonic acid –> impaired myelinization –>

A

subacute combined neurodegeneration (damage to posterior column and lateral corticospinal tract)

56
Q

anemia w/ splenomegaly,
jaundice due to unconjugated bilirubin,
increased risk for bilirubin gallstones (pigmented);
marrow hyperplasia w/ high corrected retic count

A

clinical + lab findings w/ normocytic anemia w/ extravascular hemolysis

57
Q

hemoglobinemia,
hemoglobinuria,
hemosiderinuria,
decreased serum haptoglobin

A

clinical + lab findings w/ normocytic anemia w/ intravascular hemolysis

58
Q

normocytic anemias w/ predominant extravascular hemolysis

A
  1. hereditary spherocytosis
  2. sickle cell anemia
  3. Hemoglobin C
59
Q

normocytic anemias w/ predominant intravascular hemolytis

A
  1. PNH
  2. G6PD deficiency
  3. immune hemolytic anemia
  4. microangiopathic hemolytic anemia (TTP, HUS, DIC, HELLP, mechanical trauma)
60
Q

Inherited defect of RBC cytoskeleton-membrane tethering proteins (ankyrin, spectrin, band 3)

A

hereditary spherocytosis

61
Q

increased MCHC

A

hereditary spherocytosis

62
Q

labs with hereditary spherocytosis

A

increased RDW and MCHC

63
Q

smear with hereditary spherocytosis

A

spherocytes w/ loss of central pallor

64
Q

clinical symptoms of hereditary spherocytosis

A
  1. splenomegaly, jaundice w/ UCB, inc risk for bilirubin gallstones
  2. increased risk for aplastic crisis w/ parvovirus B19
65
Q

Diagnosis for hereditary spherocytosis

A

osmotic fragility test (RBCs break in hypotonic solution)

66
Q

treatment of hereditary spherocytosis

A

splenectomy (then you’ll see howell-jolly bodies!)

67
Q

what causes sickle cell anemia?

A

single AA chain from glutamic acid (hydrophilic) to valine (hydrophobic) in beta chain of hemoglobin

68
Q

treatment for sickle cell anemia?

A

hydroxyurea increases HbF

69
Q

why do sickle cell pts have extravascular hemolysis

A

reticuloendothelial system removes RBCs with damaged membranes

70
Q

why do sickle cell pts have intravascular hemolysis?

A

RBCs w/ damaged membranes dehydrate –> hemolysis

71
Q

irreversible sickling in sickle cell anemia leads to vaso-occlusion, wh/ presents in five ways

A
  1. Dactylitis
  2. Autosplenectomy
  3. Acute chest syndrome
  4. Pain crisis
  5. Renal papillary necrosis
72
Q

consequences of autosplenectomy

A
  1. inc risk of enc org infxn (S pneumo + H flu)
  2. inc risk of salmonella paratyphoid osteomyelitis
  3. Howell-Jolly bodies on smear
73
Q

most common cause of death in adult sickle cell pts

A

acute chest syndrome

74
Q

symptoms of renal papillary necrosis

A

gross hematuria and proteinuria

75
Q

RBCs in sickle cell trait do not sickle except where?

A

renal medulla –> microinfarction –> microscopic hematuria + decreased ability to concentrate urine

76
Q

screen for sickle cell

A

metabisulfite

77
Q

90% HbS,
8% HbF,
2% HbA2
(no HbA)

A

labs in sickle cell dz

78
Q

55% HbA,
43% HbS,
2% HbA2

A

labs in sickle cell trait

79
Q

glutamic acid replaced by lysine in beta globin

A

HbC

80
Q

symptoms of HbC

A

mild anemia due to extravascular hemolysis

81
Q

Acquired defect in myeloid stem cells resulting in absent GPI –> cells susceptible to destruction by complement

A

PNH (NB: deficiency of CD55 + CD59 = PNH!!)

82
Q

DAF (CD55) normally protects RBCs from complement by

A

inhibiting C3 convertase

83
Q

what secures DAF to RBC?

A

GPI (so in PNH, no GPI –> no DAF)

84
Q

PNH screening

A

sucrose test; confirmatory test is acidified serum or flow cytometry for DAF

85
Q

cause of death in PNH

A

thrombosis of hepatic, portal, or cerebral veins (bc lysed platelets release their contents)

86
Q

complications of PNH

A

iron deficiency anemia + AML

87
Q

x linked recessive disorder that renders cells susceptible to oxidative stress

A

G6PD deficiency

88
Q

pathophys of G6PD deficiency

A

dec G6PD –> dec NADPH –> dec GLUTATHIONE –> oxidative injury of H2O2 –> intravascular hemolysis

89
Q

there are two variants of G6PD - what are they and which is worse?

A

Mediterranean variant is more severe than African variant

90
Q

dark inclusions in RBCs when stained by crystal violet

A

Heinz bodies (oxidative stress precipitates Hb)

91
Q

causes of oxidative stress in G6PD deficiency

A
  1. infections
  2. drugs (primaquine, sulfa drugs, dapsone)
  3. fava beans
92
Q

hemoglobinuria and back pain hours after exposure to oxidative stress

A

presenting symptoms of G6PD deficiency

93
Q

bite cells! (splenic macrophages remove heinz bodies)

A

smear w/ G6PD

94
Q

IgG mediated destruction of RBCs

A

warm agglutinin (immune hemolytic anemia), primarily extravascular hemolysis

95
Q

causes of IgG-mediated IHA

A

SLE, CLL, drugs (penicillin + cephalosporin)

96
Q

treatment of IgG-mediated IHA

A
  1. remove the offending drug
  2. steroids
  3. IVIG
  4. splenectomy
97
Q

IgM mediated destruction of RBCS

A

cold agglutinin, primarily intravascular hemolysis

98
Q

causes of IgM-mediated IHA

A

mycoplasma pneumonia + infectious mono

99
Q

direct coombs test confirms presence of antibodies where?

A

On the RBCs

100
Q

indirect coombs test confirms presence of antibodies where?

A

in the plasma

101
Q

what are you mixing if you do a direct coombs test?

A

Anti-IgG + patients RBCs

102
Q

what is mixed in indirect coombs test?

A

Anti-IgG + test RBCs + patients serum

103
Q

causes of microangiopathic hemolytic anemia (schistocytes!)

A

TTP, HUS, DIC, HELLP, prosthetic heart valves, aortic stenosis

104
Q

how is malaria transmitted?

A

female anopheles mosquito

105
Q

what produces EPO?

A

peritubular interstitial cells in the kidney

106
Q

Infects progenitor red cells and temporarily halts erythropoiesis; significant anemia in setting of preexisting marrow stress (sickle cell)

A

Parvovirus B19

107
Q

what type of virus is parvovirus B19? (i.e. RNA vs DNA, ss vs ds, env vs nonenv)

A

Non-enveloped ssDNA

108
Q

anemia + thrombocytopenia + leukopenia + low retic count

A

pancytopenia

109
Q

possible treatments for aplastic anemia?

A 
EPO, 
GM-CSF, 
G-CSF, 
immunosuppression, 
BMT
110
Q

pathologic process (e.g. metastatic cancer) that replaces bone marrow –> impaired hemopoiesis –> pancytopenia

A

myelophthisic process

111
Q

deficiency of CD55 + CD59 =

A

PNH!!

112
Q

what sequesters iron in storage sites?

A

hepcidin

Heme/Onc (6 decks)

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