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NBME Micro > Heme and Onc. > Flashcards

Heme and Onc. Flashcards

1
Q

What cell type contains dense granules? What are the two products of dense granules?

A

erythrocytes

ADP and calcium

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2
Q

What type of cell contains αgranules? What are the two products of αgranules?

A

erythrocytes

vWF and fibrinogen

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3
Q

In what organ is approximately 1/3 of platelet pool stored?

A

spleen

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4
Q

What is the vWF receptor?

A

GpIb

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5
Q

What is the fibrinogen receptor?

A

GpIIb/GpIIIa

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6
Q

What four enzymes are contained by neutrophils?

A

ALP
collagenase
lysozyme
lactoferrin

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7
Q

Do macrophages function via MHC II or MHC I?

A

MHC II

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8
Q

What type of nucleus do eosinophils possess?

A

bilobed

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9
Q

What two enzymes are produced by eosinophils granules?

A

histaminase and arylsulfatase

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10
Q

What are the three components of basophil granules?

A

histamine, heparin and leukotrienes

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11
Q

What form of leukemia can isolated basophilia be indicative of?

A

CML

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12
Q

What granulocyte binds Fc of IgE?

A

mast cell

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13
Q

What drug prevents mast cell degranulation?

A

cromolyn sodium

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14
Q

What two receptors do dendritic cells express?

A

Fc and MHC II

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15
Q

What are dendritic cells in the skin called?

A

Langerhans cells

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16
Q

What two CD molecules are expressed by B-lymphocytes?

A

19 and 20

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17
Q

What is the CD molecule required for T-cell activation?

A

CD28

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18
Q

Multiple myeloma is a cancer of what cell type?

A

plasma cells

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19
Q

If a person has type A blood, What antigen do they express on RBCs? What antibody is circulating?

A

A = RBC

Anti-B = circulating

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20
Q

If a person has type B blood, What antigen do they express on RBCs? What antibody is circulating?

A

B = RBC

anti-A = circulating

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21
Q

What type of immunoglobulin targets RBC antigens?

A

IgM

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22
Q

anti-Rh is what type of antibody?

A

IgG

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23
Q

If a person has type AB blood, What antigen do they express on RBCs? What antibody is circulating?

A

A and B = RBC

no circulating antibodies

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24
Q

What can people with AB blood universally donate?

A

plasma

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25
Q

What can people with AB blood universally receive?

A

blood

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26
Q

If a person has type O blood, What antigen do they express on RBCs? What antibody is circulating?

A

no antigen on RBCs

antigens to A and B in plasma

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27
Q

What can people with O blood universally donate?

A

blood

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28
Q

What can people with type O blood universally accept?

A

plasma

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29
Q

What three coagulation factors can thrombin activate?

A

V and VIII and thirteen

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30
Q

What coagulation factors activate factor X?

A

VIIa and VIIIa

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31
Q

Which three coagulation factors does Xa require? What is activated?

A

IIa and Va and VIIIa

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32
Q

What coagulation factor catalyzes the conversion of fibrinogen into fibrin?

A

IIa

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33
Q

Other than fibrinogen, what coagulation factor can thrombin activate?

A

XIII

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34
Q

What is the function of factor XIII?

A

cross-link fibrin

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35
Q

What cofactor requires only calcium?

A

XIII

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36
Q

What coagulation factors require calcium and phospholipid?

A

II, VII, IX, X

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37
Q

What two coagulation factors require thrombin for their activation?

A

five and eight

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38
Q

What is the function of plasmin?

A

to cleave fibrin

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39
Q

What complement can plasmin activate?

A

C3 to C3a

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40
Q

What clotting factor activates kallikrein?

A

XIIa

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41
Q

What two reactions does kallikrein catalyze?

A

HMWK to bradykinin

plasminogen to plasmin

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42
Q

What are the three functions of bradykinin?

A

increase permeability

pain

vasodilation

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43
Q

What three things can convert XII into XIIa?

A

collagen, basement membrane and activated platelets

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44
Q

What is the function of factor nine? What coagulation factors does factor nine need for its activation?

A

activate ten

seven and eight

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45
Q

Hemophilia A is a deficiency in what coagulation factor?

A

eight

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46
Q

Hemophilia B is a deficiency in what coagulation factor?

A

nine

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47
Q

What enzyme does warfarin inhibit?

A

Vitamin K epoxide reductase

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48
Q

What coagulation factor is carried by vWF?

A

VIII

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49
Q

Antithrombin inactivates which coagulation factors? What are the two primary targets of antithrombin?

A

two, seven, nine, ten, eleven, twelve

thrombin and Xa

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50
Q

Factor V Leidin disease produces a mutation in what clotting factor? What can’t this factor now be deactivated by?

A

Va

Activated protein C

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51
Q

Activated Protein C (APC) destroys which two coagulation factors?

A

Va and VIIIa

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52
Q

What are the two locations where vWF can be found?

A

endothelial cells and platelets

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53
Q

What is the receptor for vWF?

A

GpIb

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54
Q

What two products do platelets release in order to initiate the coagulation cascade?

A

ADP and Ca2+

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55
Q

What is the fibrinogen receptor?

A

Gp IIb/IIIa

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56
Q

What causes expression of GpIIb/IIIa? What is GpIIb/IIIa a receptor for?

A

ADP

fibrinogen

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57
Q

What thromboxane increases platelet aggregation? Where is this thromboxane released from?

A

TXA2

platelets

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58
Q

Decreasing what molecule does aspirin produce anticoagulation effects?

A

TXA2

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59
Q

What is the MOA of clopidogrel? What is another drug that produces this effect?

A

clopidogrel inhibits ADP induced expression of GpIIb/IIIa

Ticlopidine

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60
Q

What drugs inhibits GpIIb/IIIa directly?

A

Abciximab

eptifabitide

tirofiban

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61
Q

What is the MOA of Ristocetin?

A

induces vWF to bind GpIb

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62
Q

What receptor do clopidogrel and ticlopidine block?

A

ADP receptor

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63
Q

What protein is defective in Glanzmann Thrombasthenia?

A

GpIIb/IIIa

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64
Q

What is deficient in Bernard-Soulier?

A

GpIb

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65
Q

What are the two causes of acanthocytes?

A

abetalipoproteinemia

liver disease

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66
Q

What four conditions can produce basophilic stippling of RBCs?

A

anemia of chronic disease

alcohol abuse

Lead

Thalassemias

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67
Q

What is the acronym of basophilic stipling?

A

BASically, ACiD Alcohol is LeThal

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68
Q

What deficiency can lead to bite cells? What is removed?

A

G6PDase

oxidized Hb

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69
Q

What is the role of ferroportin?

A

to transfer iron out of a cell

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70
Q

What is the function of hepcidin?

A

to block ferroportin

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71
Q

Where are the two locations of ferroportin?

A

enterocytes of GI tract

macrophages

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72
Q

What disease produces a macroovalocyte?

A

megaloblastic anemia

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73
Q

What is a ringed sideroblast?

A

nucleated RBC with iron in mitochondria

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74
Q

Name for diseases where schistocytes are present?

A

DIC, TTP, HUS and traumatic hemolysis

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75
Q

What causes tear drop cells?

A

bone barrow infiltrate

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76
Q

What type of RBC features an increased cell membrane surface area?

A

target cells

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77
Q

What is the acronym do remember the causes of target cells?

A

HALT

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78
Q

What does the H in HALT stand for?

A

HbC

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79
Q

What does the A in HALT stand for?

A

Asplenia

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80
Q

What does the L in HALT stand for?

A

Liver disease

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81
Q

What does the T in HALT stand for?

A

Thalassemia

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82
Q

What type of prosthetic group is oxidized in Heinz bodies?

A

sulfhydryl

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83
Q

What type of stain is used to visualize heinz bodies?

A

crystal violet

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84
Q

What type of thalassemia produces Heinz bodies?

A

alpha-thalassemia

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85
Q

How do Howell-Jolly bodies stain?

A

basophilic

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86
Q

What are Howell-Jolly bodies composed of? What cell type?

A

nuclear remnants

RBCs

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87
Q

In what two circumstances do Howell-Jolly bodies arise?

A

asplenia or hyposplenia

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88
Q

What can cause an iron deficient anemia?

A

chronic bleeding

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89
Q

What is the triad of Plummer-Vinson Syndrome?

A
  1. esophageal webs
  2. Iron deficient anemia
  3. atrophic glossitis
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90
Q

Most microcytic RBCs also feature what?

A

hypochromia

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91
Q

In what population is the cis-deletion prevalent in alpha-Thalassemia?

A

Asians

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92
Q

In what population is the trans-deletion prevalent in alpha-Thalassemia?

A

Africans

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93
Q

What does four allele deletion alpha-thalassemia result in? What chain is present?

A

Hb barts

gamma chain

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94
Q

What does three allele alpha-thalassemia result in? What type of globin composes this?

A

HbH

β-globin

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95
Q

What type of mutation produces alpha-Thalassemia?

A

deletion

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96
Q

What type of population is β-thalassemia most commonly found?

A

Mediterranean

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97
Q

What type of mutation produces β-thalassemia? What two locations do these mutations occur?

A

point

promoter and splice site

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98
Q

What chains is HbH composed of?

A

four beta chains

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99
Q

β-thalassemia minor is characterized by what type of Hb? How much?

A

HbA2

> 3.5%

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100
Q

What happens to the β-chain in β-thalassemia minor?

A

β-chain is underproduced

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101
Q

What happens to the β-chain in β-thalassemia major?

A

β-chain is absent

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102
Q

What form of β-thalassemia produces a crew-cut on X-ray?

A

β-thalassemia major

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103
Q

What happens to bones during β-thalassemia major?

A

extramedullary hematopoiesis

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104
Q

A person with extra-medullary hematopoiesis is at risk of what viral infection?

A

Parvovirus B19

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105
Q

What form of hemoglobin predominates in β-thalassemia major?

A

HbF

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106
Q

What two enzymes are inhibited during lead poisoning?

A

ferrochelatase

ALA Dehydratase

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107
Q

What does the L of LEAD stand for?

A

Lead Lines on gingivae and metaphyses of long bones

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108
Q

What two locations do lead lines appear during lead poisoning?

A

metaphyses of long bones and gingiva

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109
Q

What does the E’s of LEAD stand for?

A

Encephalopathy and erythrocyte stipling

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110
Q

What does the A’s of LEAD stand for?

A

abdominal colic and sideroblastic Anemia

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111
Q

What does the D of LEAD stand for?

A

wrist and foot Drops

Dimercaprol and EDTA

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112
Q

What drug is used to chelate lead in children?

A

Succimer

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113
Q

What is the most commonly defective enzyme in Sideroblastic Anemia?

A

ALA Synthase

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114
Q

Other than iron, deficiency of what metal can cause sideroblastic anemia?

A

copper

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115
Q

What drug can cause sideroblastic anemia?

A

Isoniazid

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116
Q

What is the treatment for sideroblastic anemia? What enzyme will this restore activity to?

A

B6

ALA Synthase

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117
Q

What are the three antimetabolites that can cause megaloblastic anemia?

A

methotrexate

trimethoprim

phenytoin

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118
Q

What type of GI drug can cause a megaloblastic anemia?

A

PPIs

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119
Q

OTCase deficiency will increase what metabolite?

A

Orotic acid

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120
Q

What is the treatment for orotic aciduria?

A

uridine monophosphate

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121
Q

What is the function of haptoglobin?

A

to bind free Hb

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122
Q

Where is Hb-hemoglobin degraded?

A

Reticuloendothelial system

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123
Q

What are the two main laboratory markers of intravascular hemolytic anemia? What happens do their levels during hemolysis?

A

decreased haptoglobin

increased LDH

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124
Q

What is the breakdown of heme that is found in the urine?

A

urobilinogen

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125
Q

What destroys RBCs in paroxysmal nocturnal hemoglobinuria?

A

complement

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126
Q

Is paroxysmal nocturnal hemoglobinuria considered intravascular or extravascular hemolysis?

A

intravascular

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127
Q

What is the most commonly defective protein during PNH?

A

GPI anchor

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128
Q

What two CD molecules are most often mutated in PNH?

A

CD55/DAF

CD59

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129
Q

What is the function of CD55/DAF?

A

limit the formation of C3 convertase

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130
Q

What are the two functions of CD59?

A

bind MAC and prevent C9 from binding RBC cell membrane

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131
Q

Circulating Hb binds with what molecule? What does this lead to? How can this be treated?

A

NO

ED, esophageal spasm, abdominal pain

Sildenafil

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132
Q

Will unconjugated bilirubin increase during intravascular or extravascular hemolysis?

A

extravascular

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133
Q

What cell type is damaged during microangiopathic hemolytic anemia? What damages the RBCs?

A

endothelial cells

fibrin mesh

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134
Q

HUS is characterized by what triad?

A

hemolytic anemia

acute kidney failure

thrombocytopenia

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135
Q

What three bacteria can cause HUS?

A

E. coli O157:H7

Camplyobacter

Shigella

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136
Q

What toxin is implicated in HUS? What cell surface protein does this toxin bind? What is the net effect?

A

Shiga

Gb3

endothelial cells to become thrombogenic

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137
Q

What metalloprotinease is inactivated during HUS? What is the normal function of this protein?

A

ADAMTS-13

cleave vWF

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138
Q

Most cases of TTP arise due to inactivation of what protein? What is the normal function of this protein?

A

ADAMS-13

to cleave vWF

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139
Q

What is an inherited defect in ADAMTS-13 called?

A

Upshaw-Shulman Syndrome

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140
Q

What does myeloid mean?

A

granulocyte precursor

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141
Q

What are the four major granulocytes?

A

Neutrophil
Eosinophil
Basophil
Monocyte

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142
Q

Aplastic anemia is a destruction of what type of stem cells?

A

Myeloid

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143
Q

What four types of drugs can cause aplastic anemia?

A

benzene, chloramphenicol, antimetabolites, alkylating agents

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144
Q

What four virus can cause aplastic anemia?

A

B19, EBV, HCV and HIV

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145
Q

What type of anemia can cause aplastic anemia? Patients with this type of anemia are predisposed to develop what disease?

A

Fanconi anemia

AML

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146
Q

Idiopathic aplastic anemia may follow what infectious disease?

A

acute hepatitis

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147
Q

What are the three major blood cellular deficiencies of aplastic anemia?

A

pancytopenia (severe anemia)

thrombocytopenia

leukopenia

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148
Q

What are the two treatments for aplastic anemia?

A

immunosuppressive

G-CSF or GM-CSF

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149
Q

What four proteins are defective in hereditary spherocytosis?

A

ankyrin
spectrin

band 3
band 4.2

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150
Q

What is missing in RBCs with hereditary spherocytosis?

A

central pallor

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151
Q

What is the organ manifestation of hereditary spherocytosis?

A

splenectomy

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152
Q

What is used as a screening test in Hereditary Spherocytosis?

A

eosin-5-maleimide

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153
Q

What is the treatment of hereditary spherocytosis?

A

splenectomy

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154
Q

What product is decreased in G6PDase deficiency?

A

glutathione

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155
Q

How will a pyruvate kinase deficiency manifest in a new born?

A

hemolytic anemia

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156
Q

What substitution is present in a patient with HbC? What position?

A

glutamic acid to lysine in beta-globin

position six

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157
Q

What two complement proteins does DAF recognize and sequester?

A

C4b and C3b

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158
Q

What does the direct Coombs test detect?

A

IgG bound to RBCs

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159
Q

What does the indirect Coombs test detect?

A

circulating anti-RBC IgG

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160
Q

Would PNH have a positive Coombs test?

A

no

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161
Q

What is the function of CD59?

A

prevent formation of MAC

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162
Q

What is the treatment of PNH? What is the MOA of this drug?

A

eculizumab

terminal complement inhibitor

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163
Q

What glutamic acid substitution is prevent in HbS?

A

glutamic acid to valine

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164
Q

What two blood diseases will feature a ‘crew-cut’ Xray?

A

Thalassemia

HbS

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165
Q

Patients with an autosplenectomy are at an increased risk of what type of infection?

A

encapsulated bacteria

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166
Q

What disease can produce splenic sequestration crisis?

A

sickle cell anemia

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167
Q

What is the pneumonic for encapsulated bacteria?

A

Some Nasty Killers Have Some Capsule Protection

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168
Q

Salmonella osteomyelitis almost exclusively effects patients with what type of disease?

A

sickle cell anemia

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169
Q

What is the specific type of kidney damage seen in patients with sickle cell disease?

A

renal papillary necrosis

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170
Q

What is the drug Tx for sickle cell? Why?

A

Hydroxyurea

breaks down cells susceptible to sickle cell damage

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171
Q

What type of Hb does hydroxyurea increase?

A

HbF

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172
Q

Warm agglutinin detects what type of Ab? Against what antigen? Acute or chronic?

A

IgG

RBCs

chronic

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173
Q

Would autoimmune hemolytic anemia produce a positive or negative coombs?

A

positive

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174
Q

Other than hemolytic anemia, warm agglutinin test can detect the presence of what other two diseases?

A

SLE and CLL

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175
Q

Warm agglutinin test can be present if a patient is on what drug?

A

α-methyldopa

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176
Q

Cold agglutinin detects what antibody?

A

IgM

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177
Q

Is warm agglutinin chronic anemia or acute anemia? Cold agglutinin?

A

warm = chronic

cold = acute

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178
Q

What type of leukemia will produce a positive Cold Agglutinin test?

A

CLL

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179
Q

What type of bacterial infection will produce a cold agglutinin test?

A

Mycoplasma pneumoniae

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180
Q

What type of viral infection will produce a cold agglutinin test?

A

mononucleosis

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181
Q

Does serum iron increase or decrease in iron deficient anemia?

A

decrease

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182
Q

Does TIBC increase or decrease in iron deficient anemia?

A

increase

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183
Q

Does ferritin increase or decrease during iron deficient anemia?

A

decreases

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184
Q

Does % transferrin saturation increase or decrease during iron deficiency?

A

decrease

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185
Q

Does serum iron increase or decrease during ACD?

A

decrease

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186
Q

Does TIBC increase or decrease during ACD?

A

decrease

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187
Q

Does ferritin increase or decrease during ACD?

A

increase

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188
Q

Does TIBC decrease or increase during hematochromatosis?

A

decrease

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189
Q

Does pregnancy increase or decrease TIBC?

A

increase

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190
Q

What blood related protein increases during pregnancy and the use of oral contraceptives?

A

transferrin

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191
Q

What do corticosteroids do to neutrophils?

A

increase their count

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192
Q

What do corticosteroids do to eosinophils?

A

decrease

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193
Q

What do corticosteroids do to lymphocytes?

A

decrease

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194
Q

What B-vitamin is required for heme synthesis?

A

B6

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195
Q

What enzyme of heme synthesis is effected during sideroblastic anemia? How is this treated?

A

ALA synthase

B6

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196
Q

What are the two reactants of ALA Synthase?

A

glycine and succinyl-CoA

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197
Q

What are the two enzymes of heme synthesis that lead can interrupt?

A

ALA dehydratase

ferrochelatase

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198
Q

What enzyme is interrupted during Acute Intermittent Porphyria?

A

Porphobilinogen Deaminase

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199
Q

What enyzme of heme synthesis is interrupted during Porphyria Cutanea Tarda?

A

Uroporphyrinogen Decarboxylase

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200
Q

What two metabolites accumulate during lead poisoning?

A

aminolevulinic acid

Protoporphyrin

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201
Q

Does lead poisoning cause microcytic or macrocytic anemia?

A

microcytic

202
Q

What are the two inhibitors of ALA synthase?

A

glucose and heme

203
Q

What are the five P’s of AIP?

A 
painful abdomen
port wine urine
Polyneuropathy
Psychological disturbances 
Precipitated by alcohol/drugs/starvation
204
Q

What metabolite accumulates during Porphyria Cutanea Tarda?

A

Uroporphyrinogen

205
Q

What factors are measured by the prothrombin time?

A

one, two, five, seven and ten

206
Q

What factors are measured by PTT?

A

all except seven and thirteen

207
Q

What does GpIb bind? Name a disease defective in GpIb?

A

GpIb binds vWF

Bernard-Soulier disease

208
Q

Immune thrombocytopenia features antibodies against what?

A

GpIIb/GpIIIa

209
Q

What type of illness may trigger immune thrombocytopenia?

A

viral

210
Q

What enzyme will be present in the serum during TTP?

A

LDH

211
Q

vWF carries/protects what factor?

A

eight

212
Q

What is the treatment of von Willebrand Disease? Why?

A

DDAVP

desmopressin increases vWF release from endothelial cells

213
Q

What is the mnenomic for remember the causes of DIC?

A

STOP Making New Thrombi

214
Q

What does the S of STOP Making New Thrombi stand for?

A

gram-negative sepsis

215
Q

What does the T of STOP Making New Thrombi stand for?

A

trauma

216
Q

What does the O of STOP Making New Thrombi stand for?

A

obstetric complications

217
Q

What does the P of STOP Making New Thrombi stand for?

A

acute Pancreatitis

218
Q

What does the M of STOP Making New Thrombi stand for?

A

Malignancy

219
Q

What does the N of STOP Making New Thrombi stand for? Why does this happen?

A

nephrotic syndrome

lose antithrombin in urine

220
Q

What does the 2nd T of STOP Making New Thrombi stand for?

A

transfusion

221
Q

There is a widespread release of what clotting factor in DIC?

A

Tissue Factor

222
Q

What two coagulation factors are most consumed in DIC?

A

five and eight

223
Q

What part of coagulation does antithrombin deficiency effect?

A

diminishes the increase in PTT following heparin administration

224
Q

What can cause acquired antithrombin deficiency?

A

nephrotic syndrome

225
Q

Warfarin induced necrosis can be though of as a deficiency in what protein?

A

Protein C

226
Q

What four clotting factors are contained in cryoprecipitate?

A

VIII, XIII, vWF and fibronectin

227
Q

Is there an increase in leukocyte ALP durig active infection or CML?

A

active infection

228
Q

Does Hodgkin lymphoma affect a single node or mutiple nodes?

A

single

229
Q

Is Hodgkin or non-Hodgkin lymphoma characterized by Reed Sternberg cells?

A

Hodgkin

230
Q

What type of cell are Reed-Sternberg cells derived from?

A

B-cells

231
Q

What are Reed-Sternberg cells considered?

A

crippled germinal centers that have not undergone hypermutation to express their Ab

232
Q

What are the two CD markers of Hodgkin Lymphoma?

A

CD15 and CD30

233
Q

Which form of Lymphoma has non-contiguous spread?

A

non-Hodgkin

234
Q

Which form of Lymphoma has a bimodal age distribution?

A

Hodgkin

235
Q

What age does non-Hodgkin lymphoma usually strike?

A

20-40

236
Q

What virus is often hypothesized to cause Hodgkin lymphoma?

A

EBV

237
Q

Which has a better prognosis, Hodgkins Lymphoma with or without lymphocyte rich infiltrate?

A

rich in lymphocyte = better prognosis

238
Q

Regarding cancer, what oncogenic protein is located on chromosome 8?

A

c-Myc

239
Q

What translocation is present in Burkitt’s Lymphoma?

A

t(8;14)

240
Q

Regarding cancer, what oncogenic protein is located on chromosome 14?

A

Ig heavy chain

241
Q

What is the histological key word for Burkitt’s Lymphoma?

A

‘Starry Sky’

242
Q

What virus is associated with Burkitt’s Lymphoma?

A

EBV

243
Q

How does Burkitt’s arise in African? Sporadic form?

A

African = Jaw

Sporadic = Abdomen

244
Q

What translocation takes place in diffuse large B-cell lymphoma?

A

t(14;18)

245
Q

Where is the mantle zone located?

A

corona of germinal center

246
Q

What translocation is present in a Mantle Cell Lymphoma?

A

t(11;14)

247
Q

Regarding oncogenesis, what protein is located on chromosome 11?

A

Cyclin D1

248
Q

What CD molecule is present on a mantle cell lymphoma?

A

CD5

249
Q

What translocation is present during Follicular Cell lymphoma?

A

t(14;18)

250
Q

Regarding oncogenesis, what protein is present on chromosome 18?

A

bcl-2

251
Q

What type of non-Hodgkin lymphoma presents with waxing and waning lymphadenopathy?

A

follicular lymphoma

252
Q

What is HTLV1 associated with?

A

IV drug use

253
Q

What three populations are predisposed to HTLV1?

A

Japan, West Africa, Caribbean

254
Q

What are three common presentations for HTLV1?

A

cutaneous lesions, bone lesions and hypercalcemia

255
Q

What is mycosis fungoides?

A

cutaneous T-cell lymphoma

256
Q

What type of T-cell is present in Mycosis Fungoides?

A

CD4+

257
Q

What are the two locations where Mycosis Fungoides can spread?

A

lymph nodes and viscera

258
Q

Are mutltiple myeloma monoclonal or polyclonal?

A

monoclonal

259
Q

What two immunoglobulins are most often produced by Multiple Myeloma? Which more?

A

IgG and IgA

IgG

260
Q

What type of spike takes place during Multiple Myeloma?

A

M spike

261
Q

What are Bence Jones proteins composed of? Where are Bence Jones proteins found? What disease do Bence Jones proteins suggest?

A

Ig light chain

urine

multiple Myeloma

262
Q

Clock face chromatin is indicative of what disease?

A

Multiple Myeloma

263
Q

Which immunoglobulin is overproduced during Waldenstrom Macroglobulinemia?

A

IgM

264
Q

How is Waldenstrom Macroglobulinemia differentiated from Multiple Myeloma?

A

WM does NOT have lytic bone lesions

265
Q

What disease has Rouleaux formation? What are Rouleaux formations?

A

Multiple Myeloma

stacked RBCs

266
Q

What disease is an asymptomatic precursor to Multiple Myeloma?

A

Monoclonal Gammopathy of Undetermined Significance

MGUS

267
Q

What is a Pelger Huet anamoly? Caued by?

A

bilobed neutrophil

chemotherapy

268
Q

Under what age is ALL most likely to arise?

A

15 years

269
Q

Where can T-cell ALL create a mass?

A

thymus/mediastinum

270
Q

Down Syndrome patients are at an increased risk of developing what two types of leukemia?

A

ALL and AML

271
Q

What is the immunohistochemical marker for pre-T and pre-B cells? Function of this?

A

TdT+

VDJ rearrangement of TCR

272
Q

What is the immunohistochemical for pre-B cells only?

A

CD10

273
Q

What translocation has the best prognosis in ALL?

A

t(12:21)

274
Q

What two immunohistochemical does CLL possess? Is CLL an expansion of B-cells or T-cells?

A

CD20 and CD5

B-cells

275
Q

Hairy cell leukemia features what type of lymphocytes? Mature or immature?

A

Mature B-cell

276
Q

What is the average age of onset of AML?

A

65 years

277
Q

What type of leukemia stains with a TRAP stain?

A

hairy cell leukemia

278
Q

What leukemia causes marrow fibrosis? What is the stain for this leukemia?

A

hairy cell

TRAP

279
Q

What two drugs are used to treat Hairy Cell Leukemia? What is the MOA of these drugs?

A

Cladribine

adenosine analgoue that inhibits adenosine deaminase

280
Q

What type of cells are Auer rods present? What disease features Auer rods?

A

leukemic blasts

AML

281
Q

Patients with previous exposure to alkylating agents are at an increased risk of developing what type of leukemia?

A

AML

282
Q

What type of leukemia responds to trans-retinoic acid? Why?

A

M3 AML

causes blasts to mature

283
Q

DIC is a common presentation of what specific type of leukemia?

A

M3 AML

284
Q

Which type of AML is most likely to cause DIC? Why?

A

M3

release of Auer rods due to chemo. agents

285
Q

The Philadelphia Chromosome is possessed in what type of Leukemia?

A

CML

286
Q

What type of translocation takes place in M3 AML?

A

t(15:17)

287
Q

What three types of lymphocytes increase in number during CML?

A

neutrophils, metamyelocytes and basophils

288
Q

What two diseases can CML transform into?

A

ALL or AML

289
Q

Will there be a high or low level of leukocyte alkaline phosphatase during CML?

A

low

290
Q

What is the tx of CML?

A

imatinib

291
Q

Birbeck granules are indicative of what disease?

A

Langerhans Cell Histiocytosis

292
Q

What precursor are Langerhans cells derived from?

A

monocytes

293
Q

How does LCH present in child?

A

lytic bone lesions

294
Q

What disease can be recurrent in LCH?

A

otitis media

295
Q

S-100 is a marker for what cancer?

A

melanoma

296
Q

S-100 is an IHC marker for what embryological origin?

A

mesoderm

297
Q

What two IHC markers are expressed by LCH?

A

S-100 and CD1a

298
Q

What cell signaling molecule is involved in myeloproliferative disorders?

A

JAK2

299
Q

What does hematocrit have to be greater than to be considered polycythemia vera?

A

55%

300
Q

What gene is mutated in polycythemia vera?

A

JAK2

301
Q

How does polycythemia vera often present? Why?

A

intense itching after a hot shower

release of histamine

302
Q

What hematological issue can present with gouty arthritis?

A

polycythemia vera

303
Q

Why can essential thrombocytosis produce bleeding?

A

platelets sequester too much vWF

304
Q

How do RBCs look during myelofibrosis?

A

tear drop

305
Q

What drug inhibits heparin?

A

protamine sulfate

306
Q

What is heparin bound to during HIT? What class of Ab?

A

PF4

IgG

307
Q

What is the one draw back to LMW heparin?

A

not easily reversible

308
Q

What two drugs are used in patients with HIT? What is their MOA?

A

Argatroban and Bivalirudin

inhibit thrombin directly

309
Q

Which anticoagulant can cross the placenta?

A

warfarin

310
Q

What drug is given to reverse warfarin OD?

A

vitamin K

311
Q

What are the two direct inhibitors of Factor X?

A

apixiban and rivaroxaban

312
Q

What are the three thrombolytics? What are their MOAs?

A

Alteplase, Reteplase and Tenecteplase

conversion of plasminogen into plasmin

313
Q

What two proteins does plasmin cleave?

A

thrombin and fibrin

314
Q

Name two common ADP receptor inhibitors

A

clopidogrel ticlopidine

315
Q

Which ADP receptor antagonist can cause neutropenia?

A

Ticlopidine

316
Q

What are the two PDE3 inhibitors?

A

Cilostazol and Dipyridamole

317
Q

What antibody is a direct inihibitor of GpIIb/GpIIIa?

A

abciximab

318
Q

What enzyme is inhibited by methotrexate?

A

DHFR

319
Q

5-FU covalently complexes what molecule? What enzyme is inhibited?

A

folic acid

thymidylate synthase

320
Q

What is given during a 5-FU overdose?

A

uracil

321
Q

What enzyme does cytarabine inhibit?

A

DNA polymerase

322
Q

What is the most notable side effect of cytarabine?

A

pancytopenia

323
Q

Azathioprine/6-MP are analogues of what?

A

purines

324
Q

What enzyme activates 6-MP into azathioprine?

A

HGPRTase

325
Q

What drug can increase the toxicity of 6-MP and azathioprine?

A

allopurinol

326
Q

What is the MOA of actinomycin (dactinomycin)?

A

intercalates DNA

327
Q

What is the MOA of doxorubicin? What does this stop the progression of?

A

intercalates DNA

DNA topoisomerase

328
Q

What type of toxicity does doxorubicin cause?

A

cardiac

329
Q

What is the MOA of bleomycin?

A

free radical generation leadign to dsDNA breaks

330
Q

What is the MOA of cyclophosphamide?

A

alkylating agent at guanine N-7

331
Q

What is the toxicity of bleomycin?

A

pulmonary fibrosis

332
Q

p450 is require to activate what type of anti-neoplastics?

A

Alkylating agents

333
Q

What is the by-product of cyclophosphamide? What does this byproduct cause? What drug can overcome this?

A

acrolein

hemorrhagic cystitis

mesna

334
Q

What is the main toxicity of cyclophosphamide?

A

myelosuppression

335
Q

What is the MOA of nitrosureas?

A

cross-links DNA

336
Q

What type of cancers are nitrosureas used for?

A

brain cancers

337
Q

What are the main toxicities of nitrosureas?

A

CNS toxicity

338
Q

What is the MOA of busulfan? What is it used for?

A

cross-links DNA

ablate bone marrow

339
Q

What is the MOA of vincristine and vinblastine?

A

bind β-tubulin

340
Q

What is the toxicity of vincristine?

A

peripheral neuropathy

341
Q

What does vinblastine blast?

A

bone marrow

342
Q

What is the MOA of paclitaxel?

A

prevent breakdown of mitotic spindle

343
Q

What are the three main toxicitues of taxols?

A

myelosuppression

alopecia

hypersensitivity

344
Q

What is the MOA for cisplatin/carboplatin?

A

cross-link DNA

testicular

345
Q

What are the two toxicities caused by cisplatin?

A

nephrotoxicity and CN VIII damage

346
Q

What drug is used to treat the nephrotoxicity associated with cisplatin?

A

amifostine

347
Q

What is the MOA of etoposide/tenoposide?

A

inhibit topo. II

348
Q

What drug can increase HbF?

A

sickle cell anemia

349
Q

What is the MOA of irinotecan and topotecan?

A

inhibition of topo. I

350
Q

What enzyme does hydroxyurea inhibit?

A

ribonucleotide reductase

351
Q

Which SERM can be a partial agonist in the endometrium?

A

tamoxifen

352
Q

What drug is used to target HER2+ tumors? What is the main toxicity of this drug?

A

trastuzumab

heart

353
Q

What is another name for imatinib? What two neoplasias is imatinib used to treat?

A

Gleevec

Philadelphia chromosome CML and GI stromal

354
Q

Rituximab is used to treat which three conditions?

A

all Bcell lymphomas

rheumatoid

ITP

355
Q

Vemurafenib is used to treat what disease? Which specific marker?

A

malignant melanoma

B-raf kinase with V600E mutation

356
Q

Bevacizumab is used to treat which two cancers?

A

colorectal

renal cell carcinoma

357
Q

What is the toxicity of Methotrexate?

A

Myelosuppression

358
Q

What four proteins are contained within neutrophils?

A

ALP, collagense, lactoferrin and lysozyme

359
Q

What oxidative enzyme is contained within neutrophils?

A

myeloperoxidase

360
Q

What two molecules are expressed by Thelper cells?

A

CD3 and CD4

361
Q

What two molecules are expressed by cytotoxic Tcells cells?

A

CD3 and CD8

362
Q

What are the two primary targets of antiheparin?

A

II and X

363
Q

What molecule accumulates in hereditary sideroblastosis? What organelle?

A

Iron

mitochondria

364
Q

What prosthetic group of Hb is oxidized in bite cells?

A

sulfhydryl

365
Q

Where is a common location on the body for pallor to develop during iron deficient anemia?

A

conjuctiva

366
Q

Lead poisoning inhibits the degradation of what sort of nucleic acid? What does this lead to?

A

rRNA

basophilic stipling

367
Q

B6 is a cofactor for what enzyme of heme synthesis?

A

ALA synthase

368
Q

Fanconi anemia features a decrease in what type of repair mechanism?

A

DNA

369
Q

What are the two findings of G6PD deficiency?

A

back pain

hemoglobinuria

370
Q

Non-Hodgkin lymphoma may be associated with what virus?

A

HIV

371
Q

Which form of lymphoma presents with low-grade fever and night sweats?

A

Hodgkins

372
Q

What three clotting proteins decrease during DIC?

A

fibrinogen, V and VIII

373
Q

Auer rods contain what enzyme?

A

peroxidase

374
Q

Does PCV have low or high EPO levels?

A

low

375
Q

What clotting factor does LMW heparin bind to more?

A

ten

376
Q

What is the MOA of argatroban and bivalirudin?

A

inhibit thrombin directly

377
Q

How is warfarin metabolized?

A

CYP 2C9

378
Q

Which anticoagulant is lipid soluble, warfarin or heparin?

A

warfarin

379
Q

Which anticoagulant is given orally, warfarin or heparin?

A

warfarin

380
Q

Which anticoagulant acts rapidly, heparin or warfarin?

A

warfarin

381
Q

Which anticoagulant has a duration of hours, , warfarin or heparin?

A

heparin

382
Q

What drug is used to treat an overdose of alteplase/reteplase/tenecteplase?

A

aminocaproic acid

383
Q

What does 5-fluorouracil complex with? The production of what compound is inhibited? What enzyme is inhibited?

A

folic acid

dTMP

thymidylate synthase

384
Q

Cytarabine is an analogue of purines or pyrimidines?

A

pyrimidnes

385
Q

What is the side effect of cytarabine?

A

pancytopenia

386
Q

What enzyme metabolizes azathioprine?

A

xanthine oxidase

387
Q

What drug is used for childhood tumors?

A

dactinomycin

388
Q

Which antineoplastic requires bioactivation by the liver?

A

cyclophosphamide

389
Q

Drugs that inhibit microtubules prevent what phase of the cell cycle?

A

M phase

390
Q

What is the most commonly used glucocorticoid for antineoplastic medicine?

A

Prednisone

391
Q

In what tissue are SERMs an agonist?

A

bone

392
Q

What two diseases can imatinib be used to treat?

A

CML and GI stromal tumors

393
Q

What is the cofactor for myeloperoxidase?

A

heme

394
Q

What is the function of CD40?

A

co-stimulatory molecule of APCs

395
Q

Where are langerhans cells located?

A

areas of body in contact with external environment

396
Q

What is the funtion of CD3?

A

to bind the T-cell recpetor

397
Q

What is the function of Tregs?

A

help immune system differentiate self from non-self

398
Q

What syndrome can develop if Tregs do not function properly?

A

IPEX syndrome

399
Q

What txn factor is defective during IPEX syndrome?

A

FOXP3

400
Q

What two compounds bind to protein C? What binds to activated protein C?

A

thrombin and thrombomodulin

protein S

401
Q

What clotting factor binds exposed collagen?

A

vWF

402
Q

What two hematological complications arise from Copper Deficiency?

A

anemia and neutropenia

403
Q

How would a patient with subacute combined deficiency present with regards to their motor movement?

A

spastic movements

404
Q

Is Hb released into the serum during extravascular hemolysis?

A

no

405
Q

What type of infections are patients with a splenectomy predisposed to?

A

encapsulated organisms

406
Q

What five conditions can produce microangiopathic hemolytic anemia?

A 
DIC
TTP
HUS
SLE
malignant hypertension
407
Q

What two parasite diseases can cause hemolytic anemia?

A

malaria and Babesia

408
Q

Do corticosteroids cause neutropenia or neutrophilia?

A

neutrophilia

impair neutrophil migration out of vasculature

409
Q

Do corticosteroids cause eosinopenia or eosinophilia?

A

eosinopenia

410
Q

Do corticosteroids cause lymphopenia or lymphophilia?

A

lymphopenia

411
Q

What drug is used to diagnose vWF Disease?

A

ristocetin

412
Q

What is the most common cause of inheritable hypercoagulability in humans?

A

Factor V leiden deficiency

413
Q

Warfarin induced necosis is exacerbated by what disease?

A

Protein C or S deficiency

414
Q

What neoplasm has a ‘fried egg’ appearance on histlogy?

A

Multiple Myeloma

415
Q

Myelodysplastic Syndromes have an increased risk of transforming into what disease?

A

AML

416
Q

What are histiocytes?

A

activated macrophages or dendritic cells

417
Q

What is Langerhans Cell Histiocytosis also known as?

A

Hand-Schuller-Christian Disease

418
Q

What is the triad of LCH?

A

insipidus/exopthalmos/lytic bone lesions

419
Q

What two types of cells compose histiocytes?

A

activated dendrites and macrophages

420
Q

What three classes of cells are contained within pancytopenia?

A

erythroblasts

megakaryoblasts

granulocytes

421
Q

What are the three toxicities of aspirin?

A

gastric ulceration

tinnitus (CN VIII)

interstitial nephritis

422
Q

What is pulmonary side effect of aspirin overdose?

A

respiratory alkalosis

423
Q

What is the main side effect of clopidogrel or ticlopidine?

A

acute coronary syndrome

424
Q

What does increased cAMP in platelets do? What two drugs can cause an increase in cAMP in platelets?

A

inhibits aggregation

Cilostazol and Dipyridamole

425
Q

What is the only drug that leucovorin can be used in combination with to prevent myelosuppression?

A

methotrexate

426
Q

What is an overdose of 5-FU treated with?

A

uridine

427
Q

What enzyme does 6-MP/azathioprine inactivate? What rxn is catalyzed by this rxn?

A

amidophosphoribosyltransferase

PRPP to PRA

428
Q

What is the toxicity of rituximab?

A

multifocal leukoencephalopathy

429
Q

Aminocaproic acid is an analogue of what amino acid?

A

lysine

430
Q

What are the three causes of megaloblastic anemia?

A

B12, Folate and orotic aciduria

431
Q

What type of drug can cause a B12 deficiency?

A

PPI

432
Q

A patient with PNH has an increased liklihood of developing what disease?

A

acute leukemias

433
Q

What three situations precipitate Hb Sickling?

A

hypoxemia, acidosis, dehydration

434
Q

What are the two inhibitors of ALA Synthase?

A

glucose and heme

435
Q

What cell would increase in number during Immune Thrombocytopenia in the bone marrow?

A

Megakaryocytes

436
Q

What receptor is defective in Pelger-Huet Anamoly?

A

Lamin B receptor

437
Q

What type of cell is present in CLL?

A

Smudge cell

438
Q

Which anticoagulant is known to cause osteoporosis over long time use?

A

Heparin

439
Q

What are the four ADP receptor inhibitors?

A

Clopidogrel, ticlopidine, prasugrel, ticagrelor

440
Q

What drug is used in combination with doxorubicin to prevent cardiomyopathy?

A

Dexrazoxane

441
Q

What is the antigen of Type A blood?

A

N-acetylgalactosamine

442
Q

What is the antigen of Type B blood?

A

Galactose

443
Q

Would an infection increase or decrease ESR?

A

increase

444
Q

Would an auto-immune disease increase or decrease ESR?

A

increase

445
Q

Would a neoplasm increase or decrease ESR?

A

increase

446
Q

Would plycythemia vera increase or decrease ESR?

A

decrease

447
Q

Would SSA increase or decrease ESR?

A

decrease

448
Q

Would CHF increase or decrease ESR?

A

decrease

449
Q

Would pregnancy have an increase or decreased ESR?

A

increased

450
Q

Which three drugs can cause a non-megaloblastic macrocytic anemia?

A

hydroxyurea

5FU

zidovudine

451
Q

What is the mode of inheritance for orotic aciduria?

A

autosomal recessive

452
Q

What is the mode of inheritance for G6PDase Deficiency?

A

X-linked recessive

453
Q

What is the mode of inheritance of Pyruvate Kinase Deficiency?

A

autosomal recessive

454
Q

What is the most common cause of death in adults with SSA?

A

acute chest syndrome

455
Q

What hematological abnormality can produce hemarthroses?

A

Hemophilia A and B

456
Q

What type of RBC is created during TTP?

A

Schistocyte

457
Q

Does TTP produce more renal or neurological symptoms?

A

renal

458
Q

Does TTP produce a fever?

A

yes

459
Q

What is the mode of inheritance for vWF disease?

A

autosomdal dominant

460
Q

What two forms of Hodgkin Lymphoma does EBV produce?

A

mixed-cellularity

lymphocyte depleted

461
Q

What gene is activated during EBV induced Hodgkins Lymphoma?

A

NF-KB

462
Q

What cell is neoplastic during HTLV1?

A

CD4

463
Q

What is the most common type of Hodgkins Lymphoma?

A

nodular sclerosing

464
Q

Which form of myelogenous leukemia can produce splenomegaly?

A

CML

465
Q

Other than crewcut/spleen/liver, where can extramedullary hematopoiesis take place?

A

serosal surfaces

466
Q

What cell is over-produced during myelofibrosis? Which two cytokines released drive this process?

A

Megakaryocytes

PDGF and TGF

467
Q

What non-kidney malignancy can produce an increase in EPO?

A

hepatocellular carcioma

468
Q

Does warfarin monitor the extrinsic or intrinsic clotting cascade?

A

extrinsic

469
Q

Which vitamin can potentiate the effects of warfarin?

A

vitamin E

470
Q

Which endocrine disorder can potentiate the effects of warfarin?

A

Hyperthyroidism

471
Q

Which fibrinolytic has the longest half-life?

A

Tenecteplase

472
Q

What three types of tumors is doxorubicin used to treat?

A

solid tumors

leukemia

lymphomas

473
Q

What two types of cancer is bleomycin used to treat?

A

Hodgkins

testicular cancer

474
Q

What residue and position do nitrosureas alkylate?

A

O6 guanine

475
Q

What cancer is busulfan used to treat?

A

CML

476
Q

What four cancers are cisplatin/carboplatin used to treat?

A

Testicular

Ovarian

Breast

Lung

477
Q

Which two cancers is Hydroxyurea used to treat?

A

melanoma and CML

478
Q

In which two locations is tamoxifen an agonist?

A

bone and endometrium

479
Q

What cancer is the ABVD regimen used for?

A

Hodgkins Lymphoma

480
Q

What are the drugs of the ABVD regimen?

A

adriamycin/bleomycin/vinblastine/dacarbazine

481
Q

What two cell lines express MPO?

A

neutrophils and monocytes

482
Q

What three proteins can imatinib inhibit?

A

BCR/ABL

PDGF

c-Kit

483
Q

Which leukemia can express CD52?

A

CLL

484
Q

What drug targets CD52?

A

alemtuzumab

485
Q

Which two neoplastic agents inhibit G1 and S?

A

Etoposide

Tenoposide

486
Q

Which class of drugs are used during HIT?

A

Direct thrombin inhibitor

487
Q

Do factor X Inhibitors require monitoring?

A

no

488
Q

Are Langerhans cells during LCH mature or immature? What cant they do?

A

immature

activate Tcells

489
Q

Which bone can be involved during LCH?

A

mastoid

490
Q

What is seen in the bone marrow during Essential Thrombocytosis?

A

enlarged megakaryocytes

491
Q

Does AML cause splenomegly?

A

no

492
Q

Does Hairy Cell Leukemia have splenomegaly? Which pulp?

A

yes

white

493
Q

What is a Stage One Lymphoma?

A

single node

494
Q

What is a Stage Two Lymphoma?

A

two nodes on same side of diaphragm

495
Q

What is a Stage Three Lymphoma?

A

both sides of diaphragm

496
Q

What is a Stage Four Lymphoma?

A

invaded non-lymphoid tissue

497
Q

Which two drugs can be used to turn down the immune response during Aplastic Anemia?

A

cyclosporine

antithymocyte globulin

498
Q

Can be Lymphocytes function as an APC?

A

yes

499
Q

What are eosinophils highly phagocytic for?

A

antigen/antibody complexes

500
Q

What does anistocytosis mean?

A

different sizes

501
Q

What does poikilocytosis mean?

A

different shapes

NBME Micro (36 decks)

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