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CM 4 > Heme Definitions and Info > Flashcards

Heme Definitions and Info Flashcards

1
Q

rbc appearance

A

no nuclei, appearing as biconcave disks filled with hemoglobin

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2
Q

neutrophil appearance

A

3-5 nuclear lobes with cytoplasm containing pale, iliac colored granules.

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3
Q

neutrophils

A

they are phagocytes and protect against acute protection

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4
Q

life span of neutrophils

A

5 hrs to a few days

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5
Q

basophils appearance

A

lack a nuclear segment

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6
Q

role of basophils

A

similar to eosinophils

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7
Q

eosinophils appearance and role

A

2 nuclear lobes and play a role in chronic immune responses particularly those associated with helminth worm infections, asthma, and allergies

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8
Q

what is largest wbc

A

monocyte

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9
Q

monocyte appearance

A

kidney shaped nuclear and light blue cytoplasm

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10
Q

monocyte role

A

similar to neutrophils and are highly phagocytic but long lived and serve as sentinels detecting danger signals produced by infection or tissue injury

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11
Q

what is the key component in adaptive immune system

A

lymphocytes

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12
Q

lymphocyte appearance

A

condensed nuclei and scant cytoplasm

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13
Q

what may lymphocytes be

A

B cells, T cells, or natural killer cells

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14
Q

B cells are formed where

A

bone marrow

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15
Q

T cells are made where

A

thymus

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16
Q

how many rbcs, platelets and neutrophils does the bone marrow produce every day

A

rbcs -> 200 billion
platelets -> 100 billion
neutrophils -> 60 billion

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17
Q

why is hematopoiesis highly responsive to changes in peripheral counts

A

bc cytopenias and cytoses blood cells have serious even fatal consequences

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18
Q

what is hematopoiesis maintained by

A

hematopoietic stem cells

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19
Q

what is transplanted during a stem cell transplant

A

hematopoietic stem cells

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20
Q

what does the clot formation start with

A

primary platelet plug

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21
Q

what does the activation of platelets also result in and what is it converted to

A

arachidonic acid which is converted to thromboxane a2

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22
Q

thromboxane a2

A

potent inducer of platelet via a series of enzymes including cyclooxygenase

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23
Q

what does aspirin contain

A

cyclooxyrgenase inhibitors which is why aspiring is used to suppress platelet activation

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24
Q

activation of platelets also causes it to release and activation of what

A

proteins that contribute to homeostasis such as platelet factor 4, fibrinogen and factor 5. activates factor xa receptor on the cell membrane of the platelet which joins with factor 5 to generate thrombin.

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25
Q

what does defects in coagulation or thrombolysis lead to

A

hypercoagulability or bleeding disorders

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26
Q

initial tests of blood coagulation cascade

A

platelet count
prothrombin time (pt)
partial thromboplastin time (ptt)

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27
Q

normal platelet count

A

> 100,000 per microliter

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28
Q

what happens if platelet count falls below 100,000

A

takes longer time to clot and usually comes with symptoms like purpura and mucosal bleeding

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29
Q

pt is a test of what pathway

A

extrinsic

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30
Q

what is a normal pt

A

10-13 secs

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31
Q

if pt is high what does it mean

A

deficiency in one or more of clotting factors in extrinsic pathway (VII, X V, prothrombin, fibrinogen).

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32
Q

inr is derived from what

A

pt

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33
Q

inr is the same as pt except

A

it is standardized by taking into account potency of tissues factor used

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34
Q

aptt is a test of what pathway

A

intrinsic

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35
Q

what is a normal ptt

A

25-35 secs

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36
Q

what is added to plasma which activates factor XII and starts intrinsic cascade

A

kaolin

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37
Q

intrinsic cascade

A

factors XII, XI, IX, VIII, X, V, prothrombin and fibrinogen.

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38
Q

tt

A

test of functionality of fibrinogen and presence of thrombin inhibitors

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39
Q

in inherited bleeding disorders how many deficiencies are present

A

single

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40
Q

in pts w/ acquired bleeding problem such as with liver disease or vitamin k deficiency how many deficiencies are present

A

multiple

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41
Q

labs in factor 5 & 10 or prothrombin defect

A

pt & ptt -prolonged

tt - normal

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41
Q

labs in factor 5 & 10 or prothrombin defect

A

pt -pronlonged

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42
Q

labs in factor 7 defect

A

pt - prolonged

ptt & tt - normal

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43
Q

labs in factor 12,11,9,8 defect

A

pt and tt- normal

ptt - prolonged

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44
Q

labs in fibrinogen and thrombin inhibitor

A

pt and ptt- normal or prolonged

tt- proloned

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45
Q

vitamin k antagonists

A

block or slow down clotting by affecting 7, 9, 10 and prothrombin bc they all need vitamin k for carboxylation

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46
Q

mc vit. k antagonist

A

warfarin

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47
Q

how to monitor pts on heparin and why

A

pt/inr testing to make sure the degree of anticoagulation is within therapeutic range

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48
Q

heparin

A

activates antithrombin and thus inhibits steps in cascade. IV.

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49
Q

low molecular weight heparin

A

sq heparin that does not require intensive monitoring but also doesn’t work as rapidly

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50
Q

direct inhibitors

A

new oral anticoagulants that directly inhibit activated coagulation factors (in contrast to warfarin, which is indirect and affects the cascade)

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51
Q

three defects in clotting cascade

A

platelet function/thrombocytopenia
decrease in clotting factors due to decreased production or excess consumption
decrease in clotting factors because inherited defect

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52
Q

thrombocytopenia

A

too few platelets due to decreased platelet production or enhanced platelet destruction

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53
Q

platelet life span

A

7-9 days

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54
Q

platelet survival with decreased production

A

a week before severe thrombocytopenia occurred

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55
Q

platelet survival with destruction

A

curtails platelet survival suddenly and swiftly and can produce severe thrombocytopenia within hours

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56
Q

what does splenomegaly entail

A

high portion of platelets entrapped in spleen along with red and white blood cells

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57
Q

hypersplenism

A

reduction in one or more peripheral blood counts by splenic sequestration

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58
Q

platelet count of 50,000 to 120,00 which is not low enough to poser serious risk of hemorrhage

A

hypersplenidism

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59
Q

immune thrombocytoenic purpura (ITP)

A

immune mediated isolated thrombocytopenia caused by autoantibodies against platelets which leads to destruction of platelets

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60
Q

primary ITP

A

idiopathic, MC after viral infection

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61
Q

secondary ITP

A

immune mediated but associated w/ underlying disorders such as SLE, HIV, HCV

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62
Q

heparin induced thrombocytopenia

A

acquired within first 5-10 after initiation of heparin caused by autoantibody formation to the hapten of heparin & platelet factor which cause platelet activation and consumption leading to simultaneous thrombocytopenia and thrombosis.

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63
Q

disseminated intravascular coagulation (DIC)

A

activation of coagulation system leads to uncontrolled fibrin production due to tissue factor activation leading to widespread microthrombi which consumes coagulation proteins & platelets.

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64
Q

disseminated intravascular coagulation (DIC) etiologies

A

obstetric
infections (MC is gram negative sepsis)
malignancies (acute myelogenous leukemia, lung, GI or prostate malignancies)

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65
Q

what are bleeding disorders caused by

A

decrease in clotting factors due to decreased production or excess consumption

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66
Q

MCC of bleeding disorders

A

vitamin k deficiency/anticoagulation therapy
coagulopathy associated with liver disease
DIC can be considered excess consumption of clotting factors

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67
Q

what does vit k deficiency lead to

A

decrease in coagulation factors 2, 7, 9, 10 and the coagulation regulators protein c and s.

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68
Q

what is responsible for the carboxylation of factors 2, 7, 9, 10

A

vitamin k

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69
Q

what interferes with activation of vitamin k

A

warfarin

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70
Q

role of the liver

A

production and metabolism of coagulation factors

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71
Q

what can severe liver failure result in

A

bleeding tendency bc of factor deficiency

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72
Q

which factor is preserved in liver disease and where is it produced

A

factor 8 and in the endothelium

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73
Q

bleeding disorders caused by a decrease in clotting factors due to an inherited effect

A

hemophilia A & B

Von Willebrand Disease

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74
Q

hemophilia a

A

inherited blood disorder most often associated with severe morbidity frequently requiring hospitalization

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75
Q

hemophilia a is due to deficiency of what factor

A

8

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76
Q

what kind of disorder is hemophilia a

A

x linked recessive

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77
Q

what % of pts have no family history and hemophilia a is due to spontaneous mutations

A

30%

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78
Q

hemophilia occurs in how male births

A

1/5000

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79
Q

pts w/ 1% or less factor 8 are considered what

A

severe and have average of 20-30 episodes per year of either spontaneous bleeding or excessive bleeding after minor trauma

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80
Q

if pt w/ 1-4% factor 8 they’re considered

A

to have moderate hemophilia

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81
Q

if pt w/ 4% ir more factor 8 considered to have

A

mild hemophilia and generally have bleeding episodes only after trauma or surgery

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82
Q

hemophilia a life expectancy

A

60 yrs

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83
Q

leading cause of death among adult hemophilia pts

A

aids

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84
Q

hemophilia b

A

inherited bleeding disorder similar genetic, clinical, and molecular factors as hemophilia a

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85
Q

hemophilia b due to deficiency in

A

factor 9

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86
Q

where is the gene for factor 9 located and how im hemophilia b inherited

A

on X chromosome and inherited in x linked manner w/ female heterozygous carries passing the disease to half of sons

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87
Q

hemophilia b occurs in how many males

A

1/25000-30000

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88
Q

MC inherited bleeding disorder

A

von willebrand disease

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89
Q

von willebrand disease effects how many people

A

1/90 in US or 3 billion

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90
Q

how is von willebrand disease different from hemophilias

A
  1. it is inherited is autosomal dominant way
  2. bleeding is primarily from mm
  3. bleeding time is prolonged
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91
Q

von willebrand disease caused by

A

mutations in the gene encoding von willebrand factor

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92
Q

functions of von willebrand factor protein

A
  1. chaperones factor 8 protecting it from degradation

2. acts as molecular glue that enables platelets to adhere to injured blood vessels

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93
Q

what people have lower levels of plasma vWF and are commonly diagnosed a having von willebrand disease

A

humans with group O blood

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94
Q

type 1 von willebrand disease

A

vWF protein deficiencies and mc form (70%)

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95
Q

type 2 von willebrand disease

A

protein defects (not deficiencies) and accounts for 25% of cases

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96
Q

type 3 von willebrand disease

A

inherited a mutant vWF gene from each parent and proteins are very low so these pts have much more severe bleeding problems

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97
Q

thrombophilia

A

lab abnml that increase rick of venous thromboembolism and pts w/ hx of recurrent VTE, VTE at young age, strong fam hx of VTE or thrombosis at unusual site

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98
Q

virchows triad

A

abnml blood flow (stasis)
vessel injury or inflammation
hypercoagulability

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99
Q

common cause of acquired thrombophilia

A

antiphospholipid syndrome

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100
Q

acquired causes of thrombophilia

A

transient thrombophilia, including taking estrogen based contraception, pregnancy, cancer, surgery and immobilization

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101
Q

mc hereditary cause of thrombophilia

A

factor V leiden mutations and deficient of natural anticoagulants antithrombin, protein c, and protein s

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102
Q

about 1/2 of pts with hereditary thrombophilia have first thrombotic episode in relation to what

A

acquired prothrombotic risk factor

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103
Q

mc inherited cause of hyper coagulability

A

factor v leiden mutation

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104
Q

how much % of population effected by factor v leiden mutation

A

5%

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105
Q

factor v leiden mutation

A

genetic variant that increases activity of factor v bc it is resistant to breakdown by activated protein c

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106
Q

protein c or s deficiency

A

loss of function mutations that interfere w/ activity of inhibitors of coagulation rather than increasing activity if procoagulant factors.

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107
Q

protein c and s are … dependent and produced in the …

A

vit k, liver

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108
Q

anemia

A

lack of healthy rbcs or hemoglobin

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109
Q

causes of anemia

A 
Hypoproliferative = Decreased production of RBCs or their components
Maturation = Inability of components to produce a healthy RBC or hemoglobin, often due to structural abnormality or deficiency of precursors
Hemolytic = destruction of RBCs or their components
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110
Q

how much hemoglobin in every rbc

A

280 mil

111
Q

heme

A

protoporphyrin which binds to iron

112
Q

where is heme synthesized

A

bone marrow (constant) but also liver (highly variable)

113
Q

most famous heme protein synthesized by liver

A

cytochrome 450

114
Q

what is heme synthesis regulated by

A

ALA synthase, prophobilinogen synthase and ferrochelatase

115
Q

ALA synthase is regulated by

A

intracellular iron levels

116
Q

Porphobilinogen and Ferrochelatase are inhibited by

A

Lead

117
Q

each heme is capable of binding to

A

one O2 molecule

118
Q

How many oxygen atoms does each hemoglobin carry?

A

8?

119
Q

hemoglobin is made from

A

heme and globin (chains)

120
Q

4 polypeptide globin chains

A

2 alpha chains and 2 non alpha chains

121
Q

how many times of globin chains and what are they

A

alpha (α), beta (β), gamma (γ) and delta (δ)

122
Q

alpha globin aa and chromosome

A

141 amino acids

Coded on chromosome 16

123
Q

beta globin aa and chromosome

A

146 amino acids

Coded on chromosome 11

124
Q

delta globin

A

small amounts in adults

125
Q

gamma globin

A

found in fetal hemoglobin

126
Q

Hemoglobin A (HgbA)

A 
adult hemoglobin (aka hemoglobin α2β2)
97% of adult hemoglobin contains 2 α chains and 2 β chains.
127
Q

Hemoglobin A2 (HgbA2)

A

less common form of adult hemoglobin

1-3% of adult hemoglobin contains 2 α chains and two delta (δ) chains.

128
Q

Fetal hemoglobin (HgbF)

A

Has 2 α chains and 2 gamma (γ) chains that, by 6 months after birth, the γ chains evolve to β chains.

129
Q

Hemoglobin S

A

predominant hemoglobin in people with sickle cell disease. The alpha chain is normal, the beta chain has a mutation (2 alpha chains, 2 beta S chains)

130
Q

Hemoglobin H

A

a tetramer composed of four beta chains; seen in three-gene alpha thalassemia

131
Q

Hemoglobin Barts

A

develops in fetuses with four-gene deletion alpha thalassemia

132
Q

electrophoresis

A

A test to measure and identify the different types of hemoglobin in the blood. An electrical current separates normal and abnormal hemoglobin.

133
Q

Hemolytic anemia

A

anemia caused by increased rbc destruction at rate that exceeds bone marrows ability to replace destroyed cells

134
Q

what happens when there is rbc lysis

A

an intracellular enzyme called lactate dehydrogenase or LDH spills into plasma and builds up in blood
hemoglobin spills out of cell and breaks up into globin and heme
heme converted to bilirubin which is taken up by liver cells and secreted out with bile

135
Q

inherited (intrinsic) hemolytic anemia

A 
abnml rbcs
sickle cell
thalassemia
G6P6 deficiency 
hereditary spherocytosis
136
Q

extrinsic (acquired) hemolytic anemia

A 
cause by things outside rbc
autoimmiune hemolytic anemia (AHA)
thrombocytopenic purpura (TTP)
hemolytic uremic syndrome (HUS)
paroxysmal nocturnal hemoglobinuria
137
Q

hemoglobinopathies

A

defects in genes that control expression of hemoglobin proteins that produce abnormal hemoglobins and anemia

138
Q

Structural defects in the hemoglobin molecule

A

alterations in the gene for one of the two hemoglobin subunit chains (alpha or beta) are called mutations. These mutations change a single amino acid in the subunit. These changes are often innocuous, but occasionally these disturb the behavior of the hemoglobin molecule and produce a disease state like Sickle Cell Anemia/Disease.

139
Q

Diminished production of one of the two subunits of the hemoglobin molecule

A

Mutations that produce this condition are called “thalassemias.” Equal numbers of alpha and beta chains are necessary for normal function, but chain imbalances damage and destroy red cells and thereby produce anemia.

140
Q

sickle cell trait homo or hetero

A

heterozygous

141
Q

sickle cell disease

A

inherited disorder affecting beta globin gene leading to production of rbcs that sickle causing hemolysis and vase occlusive diease

142
Q

cause of sickle cell anemia

A

replacement of glutamic acid w/ valine at position 6 on beta globin chain.

143
Q

result of sickle cell disease

A

change in chains solubility. Deoxygenated hemoglobin S polymerizes to form twisted rope-like fibers that align and deform the RBC, producing the sickle shaped hemoglobin (HbgS).

144
Q

sickle cells are destroyed by

A

spleen

145
Q

what does sickle shape cause

A

vaso occlusion and hypoxia

146
Q

when does clinical phenotype thalassemia become most apparent

A

at 6-9 months bc evolution from the gamma chains to adult chains occurs at around 6 months

147
Q

alpha thalassemia mcc

A

gene deletions

148
Q

Four phenotypes of alpha thalassemia

A 
Silent Carrier (1 deletion) -> asymptomatic
Alpha Thalassemia Minor (2 deletions) -> causes mild microcytic anemia
Alpha Thalassemia Intermedia (3 deletions) -> presence of Hemoglobin H; Heinz bodies 
Hydrops Fetalis (4 deletions) -> BART gammas; associated with stillbirth or death shortly after birth
149
Q

beta thalassemia

A

decrease beta chain synthesis but most common are mutations that result in defective gene transcription or translation

150
Q

three phenotypes of beta thalassemia

A

Beta Thalassemia Minor (1 mutation) -> asymptomatic or mild anemia
Beta Thalassemia Intermedia (mild homozygous form) -> mild anemia
Beta Thalassemia Major [AKA Cooley’s Anemia] (2 mutations) -> severe anemia

151
Q

beta thalassemia major (Cooley’s Anemia) risk factor

A

mediterranean descent

152
Q

G6PD Deficiency

A

x linked recessive enzymatic disorders of rbcs that can cause hemolytic anemia

153
Q

how much percent of african Americans have g6pd deficiency

A

10-15%

154
Q

when is g6pd deficiency tested for

A

at birth

155
Q

g6pd deficiency

A

G6PD activity is decreased during oxidative stress results in an oxidative form of Hb. The denatured hemoglobin precipitates as Heinz bodies.
RBC membrane damage and fragility causes extravascular RBC destruction in the spleen and liver.

156
Q

G6PD deficiency exacerbated by

A

infections, fava beans, and some meds like (dapsone, Nitrofurantoin, “sulfa” drugs).

157
Q

Hereditary spherocytosis (HS)

A

Autosomal dominant hereditary intrinsic hemolytic anemia caused by a deficiency in RBC membrane and cytoskeleton (spectrin/ankyrin). Most people with HS have a “silencing” of the ankyrin gene.
This leads to increased RBC fragility and sphere-shaped RBCs, which are detected and destroyed by the spleen (hemolysis).

158
Q

extrinsic hemolytic anemias

A

antibodies and complement are directed against RBCs, leading to their destruction

159
Q

extrinsic hemolytic anemias intravascular hemolysis

A

In some of these disorders, there’s excessiveclot formationso when normal RBCs flow through these blood vessels, they get banged up and damaged, leading to intravascular hemolysis.

160
Q

autoimmune hemolytic anemia can be divided into

A

IgG, also called warm antibody hemolytic anemia.

IgM, also called cold agglutinin disease.

161
Q

Extrinsic types can be further classified as ….. and ….

A

intravascular, meaning RBCs are destroyed within the vasculature, or extravascular, meaning that they are removed by macrophages in the spleen and liver.

162
Q

Intravascular hemolysis

A

Hemoglobin that is released inside the vessels gets bound by a protein called haptoglobin and because they’re removed together, haptoglobin decreases.
When the haptoglobin gets consumed, the remaining hemoglobin goes via the blood through the kidneys and into the urine resulting in hemoglobinuria.

163
Q

Extravascular hemolysis

A

RBCs are destroyed outside the vessels and so, haptoglobin is normal and there’s no hemoglobin or hemosiderin in the urine.
RBCs are usually destroyed in the spleen causing splenomegaly or the liver causing hepatomegaly.

164
Q

Autoimmune hemolytic anemia

A

Acquired hemolytic anemia due to autoantibody production against RBCs.

165
Q

Warm autoimmune hemolytic anemia and etiologies

A

IgG antibodies activated by protein antigens on the RBC surface at body temperature.

idiopathic (most common); medications (Penicillin, Cephalosporins, Rifampin, Phenytoin); autoimmune, viral infections, malignancy

166
Q

cold autoimmune hemolytic anemia and etiologies

A

IgM antibodies against polysaccharides on the RBC surface induces intravascular, complement-mediated RBC lysis, especially at colder temperatures.

infection (EBV, HIV, Mycoplasma pneumoniae), malignancy, Waldenstrom macroglobulinemia

167
Q

thrombotic thrombocytopenia purpura (TTP)

A

Thrombotic microangiopathy resulting from ADAMTS13 deficiency.

168
Q

what is ADAMTS13

A

von Willebrand factor-cleaving protease.

169
Q

what does ADAMTS13 deficiency lead to

A

to large vWF multimers that cause small vessel thrombosis.

170
Q

Primary TTP

A

Primary = idiopathic (autoimmune) = antibodies against ADAMTS13

171
Q

Secondary TTP

A

malignancy, bone marrow transplantation, SLE, pregnancy

172
Q

Hemolytic uremic syndrome (HUS)

A

Thrombotic microangiopathy due to platelet activation by exotoxins.

173
Q

Hemolytic uremic syndrome (HUS) risk factors

A

Predominately seen in children with a recent history of gastroenteritis
In adults it is associated with HIV, SLE, antiphospholipid syndrome or chemotherapy.

174
Q

Hemolytic uremic syndrome (HUS) Triad

A

Thrombocytopenia
Hemolytic anemia
Renal dysfunction (uremia)
Fever and neurologic symptoms (seen in TTP) often absent in HUS

175
Q

Hemolytic uremic syndrome (HUS) D+ HUS (classic)

A

diarrhea prodome. Exotoxins from Shigella or Enterohemorrhagic E. coli enters the blood where it damages vascular endothelium, activating platelets and eventually depleting platelets. The toxins preferentially damage the kidney, leading to uremia.

176
Q

Hemolytic uremic syndrome (HUS) D- HUS (atypical)

A

not common; not associated with diarrhea

177
Q

Hemolytic uremic syndrome (HUS) P- HUS

A

Streptococcus pneumonaia releases neuraminidase, which initiates an inflammatory reaction

178
Q

Paroxysmal Nocturnal Hemoglobinuria

A

Rare, acquired (the PIGA gene) stem cell mutation where RBCs become deficient in GPI anchor surface proteins (CD55 & CD59)
CD55 & CD59 normally protect RBCs from compliment destruction. Deficiency in these proteins leads to increased complement activation and intravascular RBC destruction.

179
Q

MC anemia worldwide

A

iron deficiency anemia

180
Q

MCC of iron deficiency anemia in US

A

chronic blood loss

181
Q

MCC of iron deficiency anemia in the world

A

decreased iron absorption

182
Q

risk factors for iron deficiency anemia

A

Increased metabolic requirements = children, pregnant, lactating women
Cow milk ingestion in young children = infants or toddlers who are fed cow’s milk.
Cow’s milk has no iron
It makes it harder for the body to absorb iron
It can cause small bleeds in intestines
Children are full so they don’t eat other proteins with iron .

183
Q

iron deficiency anemia

A

decreased rbc production bc there is a lack of iron and decreased iron stores (decreased ferritin).

184
Q

lead poisoning

A

Lead poisons enzymes, including enzymes needed for heme synthesis.
Lead poisoning is most common in children (especially < 6) due to increased permeability of the blood-brain barrier as well as iron deficiency (may lead to increased iron absorption).

185
Q

sources of lead poisoning

A

ingestion or inhalation of environmental lead (paint chips, lead dust -> lead was used in household paints prior to the 1970s).

186
Q

anemia of chronic disease

A

Anemia due to decreased RBC production in the setting of chronic disease
It is seen in patients with chronic inflammatory conditions (autoimmune disorders, malignancy, etc.) It is caused by both hemolysis and failure of the erythropoietin response and a limitation in iron supply, which inhibit the marrow response. In addition, hepcidin (an acute phase reactant) blocks the release of iron from macrophages and reduces the GI absorption of iron.

187
Q

anemia of chronic disease MC in who

A

It tends to be most common in patient with cancer, HIV infection, autoimmune diseases and patients with chronic kidney disease.
It is also more common in the elderly because of age-associated hematopoietic changes.

188
Q

aplastic anemia

A

Pancytopenia with bone marrow hypocellularity

189
Q

patho of aplastic anemia

A

T cells attack hematopoietic stem cells (HSC) or direct stem cell damage leads to bone marrow failure, including the replacement of marrow with fat.

190
Q

MCC of aplastic anemia

A

radiation exposure

191
Q

other cause of aplastic anemia

A 
Infection = viral hepatitis, Parvovirus B19 in patients with baseline hemolytic anemias
Medications = antibiotics (Chloramphenicol, sulfa drugs), chemotherapy, anti-epileptics (Carbamazine, Phenytoin)
192
Q

cause of severe aplastic anemia

A

Immune-mediated aplasia (failure of an organ or tissue to develop or function normally)

193
Q

sources of b12

A

mainly animal in origin (meats, eggs, dairy)

194
Q

patho fo b12 deficiency

A

B12 deficiency causes abnormal synthesis of DNA, nucleic acids and metabolism of erythroid precursors.

195
Q

absorption of b12

A

B12 is released by the acidity of the stomach where it combines with intrinsic factor, where it is absorbed mainly in the distal ileum.

196
Q

MMC of b12 deficiency

A

pernicious anemia, which is a lack of intrinsic factor because of parietal cell antibodies in the stomach, which causes decreased absorption of B12.

197
Q

decreased b12 absorption also seen with

A

Crohn disease, celiac disease, chronic alcohol use.
Some drugs can decrease absorption, such as H2 blockers, PPIs, Metformin.
Can also be caused by decreased intake - mostly vegans who don’t eat meat products.

198
Q

folate deficiency

A

abnormal synthesis of DNA, nucleic acids & metabolism of erythroid precursors.

199
Q

folate stores only last

A

2-4 months

200
Q

mcc of folate deficiency

A

inadequate intake

201
Q

causes of folate deficiency

A

Increased requirements = pregnancy, infancy, hemolytic anemias, malignancy
Impaired absorption = celiac disease, inflammatory bowel disease, chronic diarrhea, anticonvulsants (Phenytoin, Carbamazepine)
Impaired metabolism due to drugs

202
Q

hereditary hemochromatosis

A

Autosomal recessive disorder characterized by EXCESS IRON deposition in the parenchymal cells of the heart, liver, pancreas and endocrine organs.

203
Q

hereditary hemochromatosis associated with what genotype

A

C282Y HFE

204
Q

hereditary hemochromatosis patho

A

Mutation in the HFE protein leads to decreased hepcidin, the iron regulatory hormone.
Decreased hepcidin leads to increased intestinal iron absorption, leading to organ dysfunction from iron deposition in the parenchymal cells.

205
Q

polycythemia vera (primary erythrocytosis)

A

Acquired myeloproliferative disorder with autonomous bone marrow overproduction of all three myeloid stem cell lines (primarily increased RBCs, but also increased granulocytic WBCs and platelets).

206
Q

Primary (Polycythemia Vera)

A

normal O2 saturation, increased hematocrit, decreased erythropoietin, increased WBCs & platelets. This is due to a JAK gene mutation.

207
Q

secondary polycythemia vera (primary erythrocytosis)

A

decreased O2 saturation, increased erythropoietin, normal WBC & platelets (this is usually seen with people who live at high altitudes, patients with COPD, etc.)

208
Q

polycythemia vera (primary erythrocytosis) risk factors

A

Peaks 50-60 years of age; most common in men (60%)

209
Q

what are blasts

A

early premature blood cells

210
Q

presence of immature granulocytes in serum is sign of what

A

serious infection and the bone marrow is working hard to keep up with the high need for WBCs.

211
Q

how many days do mature granulocytes spend in bone marrow before entering circulation and it can be shorted to what with severe infection

A

5-6 days to a day or less with release of immature granulocytes

212
Q

The earliest response to an infection

A

emigration of granulocytes out of circulation and into the site of bacterial invasion. They trigger the full inflammatory response, which releases more cytokines capable of stimulating marrow-progenitor proliferation.

213
Q

A mature neutrophil is called

A

“seg” or “segmented” cell.

214
Q

An immature neutrophil is called

A

“band.”

215
Q

Neutrophilia

A

increase in neutrophils beyond what is expected in a healthy individual.

216
Q

Clinically, neutrophilia is defined as an absolute neutrophil count (ANC) in excess of

A

7,000 -7,500 /L. (Normal is 3,000 - 7,000).

217
Q

The ANC is really an estimate of the body’s ability to

A

fight an infection.

218
Q

Significant risk of bacterial sepsis

A

Grade IV = Severe = ANC < 500/uL

219
Q

Grade I = Mild neutropenia

A

ANC 4,000 - 1,500/uL

220
Q

Grade 2 = Mild neutropenia

A

ANC 1,499 - 1,000/uL

221
Q

Grade 3 = Mod neutropenia

A

ANC 999 - 500/uL

222
Q

Grade 4 = Severe neutropenia

A

ANC < 500/uL

223
Q

cause of neutropenia

A 
Autoimmune disease (SLE & RA are the most common)
Hypersplenism
HIV / AIDS
Hematopoietic malignancy
Leukemias
Hodgkin disease
T-cell malignancies
224
Q

Myeloid

A

tissue of bone marrow

225
Q

Myelogenous tissue

A

tissue arising from bone marrow

226
Q

Myeloid disorders

A

disorders arising from the myeloid stem cells

227
Q

Myeloproliferative disorders

A

group of conditions that cause blood cells (platelets, white blood cells, and red blood cells) to grow abnormally in the bone marrow.

228
Q

myelodysplastic syndrome

A

Preleukemic disorders characterized by abnormal differentiation of cells of the myeloid cell line resulting in ineffective hematopoiesis in the bone marrow.

229
Q

myelodysplastic syndrome risk factors

A

Age > 65 y
Hx of radiation therapy or chemotherapy
Benzene, mercury, or lead exposure; tobacco smoke

230
Q

acute myeloid leukemia (aml)

A

malignancies that are characterized by the appearance of increased numbers of immature myeloid cells in the marrow and blood.

231
Q

The fundamental oncogenic event of acute myeloid leukemia (aml)

A

takes place at the level of a very early progenitor cell, which results in a LEUKEMIC stem cell with self renewal and multipotential properties.

232
Q

MC acute leukemia in adults

A

acute myeloid leukemia (aml)

233
Q

acute myeloid leukemia (aml) patho

A

accumulation of leukemic blasts (immature WBCs) in the bone marrow, blood, or occasionally other tissues. Can cause pancytopenia.

234
Q

acute myeloid leukemia (aml) age onset

A

65 y/o

235
Q

Acute promyelocytic leukemia (APL or M3)

A

AUER rods, myeloperoxidase positive and associated with DIC.

236
Q

Acute megakaryoblastic leukemia

A

most common in children < 5 y.o. w/ Down syndrome.

237
Q

Acute monocytic leukemia

A

associated with gingival hyperplasia

238
Q

three main types of aml

A

Acute promyelocytic leukemia (APL or M3).
Acute megakaryoblastic leukemia.
Acute monocytic leukemia.

239
Q

Leukostasis

A

symptomatic hyperleukostasis = “blast crisis” seen in AML or CML

240
Q

Leukostasis patho

A

super high levels of blast cells which leads to increased blood viscosity that clogs up the microvasculature, impeding tissue perfusion and causing local hypoxemia.

241
Q

chronic myelogenous leukemia

A

myeloproliferative disorder of uncontrolled production of mature and maturing granulocytes.
In contrast to AML, they tend to have a more protracted clinical course as the malignant cells maintain the ability to mature during the chronic phase of the disease.

242
Q

chronic myelogenous leukemia patho

A

Translocation between chromosomes 9 and 22 that results in a Philadelphia chromosome, which harbors a BCR-ABL1 fusion gene.

243
Q

acute lymphocytic leukemia (all)

A

Malignancy arising from immature lymphoid stem cells in bone marrow.

244
Q

MC childhood malignancy

A

acute lymphocytic leukemia (all)- peak 2-5 y/o more boys than girls

245
Q

risk factor of acute lymphocytic leukemia (all)

A

down syndrome

246
Q

MC acute lymphocytic leukemia (all)

A

B cell

247
Q

acute lymphocytic leukemia (all) patho

A

overpopulation of immature WBCs (blasts) which overtake normal hematopoiesis, resulting in pancytopenia.

248
Q

chronic lymphocytic leukemia (cll)

A

Malignancy of mature B-cells

249
Q

MC form of leukemia in adults

A

chronic lymphocytic leukemia (cll)

250
Q

chronic lymphocytic leukemia (cll) risk factors

A

increasing age (median = 70 y/o); men > women

251
Q

multiple myeloma

A

Cancer associated with proliferation of a single clone of plasma cells, leading to increased production of ineffective monoclonal antibodies (especially IgG & IgA).

252
Q

MC primary bone malignancy in adults

A

multiple myeloma

253
Q

multiple myeloma risk factors

A

elderly > 65y, African American, male, benzene exposure

254
Q

multiple myeloma patho

A

plasma cells accumulate in the bone marrow, interrupting bone marrow’s normal cell production. Protein accumulation causes kidney injury

255
Q

Lymphoma

A

cancer where lymphocytes grow out of control.

256
Q

hodgkin lymphoma

A

B-cell malignancy originating in the lymphatic system

257
Q

peak of hodgkin lymphoma

A

at 20 y/o and again > 50 y/o

258
Q

hodgkin lymphoma risk factors

A

Epstein-Barr virus, immunosuppression, smoking

259
Q

four types of Hodgkin lymphoma

A 
Nodular sclerosing (64%) 
Mixed cellularity (25%) 
Lymphocyte rich/predominant
Lymphocyte depleted (4%)
260
Q

Nodular sclerosing (64%)

A

most common Hodgkins lymphoma type overall; female > male

261
Q

Mixed cellularity (25%)

A

associated with EBV

262
Q

Lymphocyte rich/predominant

A

most common in men < 35 y/o; best prognosis

263
Q

Lymphocyte depleted (4%)

A

most common in males > 60 y/o; worst prognosis

264
Q

non hodgkin lymphoma

A

Group of lymphocyte neoplasms with proliferation in the lymph nodes & spleen

265
Q

non hodgkin lymphoma

A 
Diffuse large B-cell
Follicular
Mantle cell
Marginal zone
Burkitt Lymphoma
Small lymphocytic
266
Q

Diffuse large B-cell

A

Fast growing, aggressive
Middle age and elderly
Rapidly enlarging lymph nodes
MOST COMMOn

267
Q

follicular

A

slow growing but hard to cute.
MC in adults
Painless lymphadenopathy.
2nd mc

268
Q

mantle cell

A

poor prognosis
older adults
painless lymphadenopathy w/ GI liver involvement
small cells surrounding follicular zone “mantle”

269
Q

marginal zone

A

small B cell hyperplasia from chronic immune/inflammatory states.
mucosal associated lymphoid tissue (malt) = gastric lymphomas
3 types:MALT, nodal, plenic

270
Q

burkitt lymphoma

A

Intermediate B-cell proliferation; very aggressive but curable
peds/adolescent/HIV
extra nodal mass.
associated w/ EBV.

271
Q

small lymphocytic

A

similar to cll

272
Q

risk factors of non hodgkin lymphoma

A

Increased age
Immunosuppression: HIV, HCV, viral infection, organ transplant
Infections: EBV, HIV, H.pylori (with MALT), HHV-8 (Karposi Sarcoma)
Autoimmune disorders: SLE, dermatomyositis, RA, Hashimotos thyroiditis

273
Q

tumor lysis syndrome

A

Oncologic emergency that can occur with the treatment of neoplastic disorders due to rapid tumor cell lysis after the induction of chemotherapy.
As the tumor cells lyse, they release massive amounts of potassium, phosphate and nucleic acids into the circulation.

274
Q

risk factors for tumor lysis syndrome

A

High tumor burden (initial WBC count > 20,000 /uL), dehydration
Large proliferation rate (ALL) and high-grade lymphomas (Burkitt)

CM 4 (9 decks)

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