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USMLE: step 1 > Hem Onc > Flashcards

Hem Onc Flashcards

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USMLE® Step 1 flashcards
1
Q

2 derivatives of HSCs?

A
  1. Myeloids

2. Lymphoids

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2
Q

What do RBCs derive from?

A

Myeloid line of HSCs

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3
Q

Do RBCs have nucleus?

A

No

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4
Q

What does Wright giemsa stain for?

A
  • Ribosomal RNA in cytoplasm of reticulocytes

- As have just lost nucleus, have lots of this

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5
Q

Movement of CO2 into RBC?

A
  1. Moves into cell and converted to bicarb

2. Moves out in exchange for Cl

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6
Q

What is anisocytosis? Poikilocytosis?

A

Anisocytosis: Varying sizes of RBCs
- Think, aniso sounds like “anysize”
Poikilocytosis: varying shapes

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7
Q

Size of RBC relative to lymphocytes?

A

About same size

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8
Q

What is a thrombocyte?

A

AKA platelet

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9
Q

What is megakaryocyte?

A

Progenitor cell of platelet that is of myeloid lineage

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10
Q

Lifespan of platelet?

A

8 - 10 days: this is why you have to wait this long for platelet to fully regain function post aspirin use as it is binding COX in platelet

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11
Q

Where are 1/3 of body’s platelets found?

A

Spleen: this is why you can have low platelet count if you have splenomegaly
*Reverse is true if you have no spleen

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12
Q

2 granules in platelets and what is contained in them?

A
  1. Dense: ADP, Ca

2. Alpha: vWF, fibrinogen

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13
Q

What does clopidogrel block?

A

ADP receptor on platelet

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14
Q

What does GP1B receptor on platelet bind?

A

VwF

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15
Q

What does GP2b/3a bind?

A

Fibrinogen

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16
Q

Another name for leukocytes?

A

WBCs

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17
Q

2 classes of leukocytes?

A
  1. Granulocytes: myeloid lineage derived from myeloblasts

2. Monocytes: same lineage as above

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18
Q

Which are the granulocytes?

A
  1. Neuts
  2. Basophils
  3. Eosinophils
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19
Q

What is a band neutrophil?

A

Immature neut with one large nucleus as it has not yet had chance to break up into bilobed nucleus

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20
Q

When are neuts hypersegmented?

A

B12 deficiency anemia

*Neuts with > 6 lobes

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21
Q

What is released to activate macs?

A

INF-gamma from T helper cells

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22
Q

What is TNF alpha?

A
  • Released from macs to encourage granuloma formation
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23
Q

What is cell marker for macrophage?

A

CD14

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24
Q

What is CD 14 the cell marker for?

A

Macrophage

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25
Q

Protein specific to eosinophils?

A

Major basic protein

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26
Q

States in which eosinophilia is seen?

A 
"NAACP"
Neoplasia
Allergy
Asthma
Chronic adrenal insufficiency
Parasites
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27
Q

Difference between histo of basophils and eos?

A

Basophils have granules that obscure the nucleus

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28
Q

What do basophils release?

A
  1. Heparin
  2. Leukotrienes
  3. Histamine
  4. Vasoactive amines
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29
Q

What do mast cells mediate? How?

A

Allergic reactions

  1. Allergen binds IgE
  2. Mast cell binds FC portion IgE
  3. Upon enough IgE binding, IgE cross links and cells degranulate
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30
Q

What do mast cells release?

A
  1. Histamine
  2. Heparin
  3. Eosinophil chemotactic factors
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31
Q

What type of hypersensitivity do mast cells regulate?

A

Allergic:

  1. Asthma
  2. Hay fever
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32
Q

Medicine blocking mast cell degranulation?

A

Cromolyn sodium

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33
Q

What are dendritic cells derived from?

A

The monocyte, just like the macrophage

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34
Q

Which are the APCs?

A
  1. Dendritic - best
  2. Macs
  3. B cells
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35
Q

What is main inducer of primary / humoral immunity?

A

Dendritic cells

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36
Q

What are the derivatives of lymphoid lineage?

A
  1. NK - only innate cell of lymphoid origin
  2. B cells
  3. T cells
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37
Q

What do NK cells do?

A

Recognize infected cells that do not have MHC attached or Ig attached
- Induce apoptosis in these cells

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38
Q

What are the B cell markers?

A
  1. CD19

2. CD20

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39
Q

What are CD 19/20 cell markers for?

A

B cells

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40
Q

What do CD8s mainly regulate?

A

Viral infx

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41
Q

What does chromatin in clockface distribution indicate?

A

Plasma cells

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42
Q

Do eosinophils or mast cells act in asthma?

A

Eosinophils

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43
Q

When are where are RBCs made in fetus?

A 
"Young liver synthesizes blood"
Yolk sac: 3 - 8 
Liver: 6 - birth 
Spleen: 10 - 28
Bone marrow: 18 - life
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44
Q

What is a type II hypersensitivity?

A

Rxn against antigen that is on a cell surface as would be seen in a blood transfusion

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45
Q

Difference between anti AB and RH antibodies?

A

RH: IgG Crosses Placenta
AB: IgM Does NOT cross placenta
- If you are blood type O, you naturally have anti A/B antibodies: same goes for A/B blood type
- With RH, regardless of if you are +/- you only develop the antibody if you have been exposed to the antigen

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46
Q

Are proteins positively or negatively charged?

A

Negative, as such RBCs move towards cathode in electrophoresis

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47
Q

Which unit is mutation in in sickle cell?

A

Beta globin, Alpha globin chains are normal

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48
Q

Mnemonic for where HgB types fall on electrophoresis?

A 
"A Fat Santa Claus"
HgA
Fetal 
Sickle
HgC disease
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49
Q

What is job of intrinsic and extrinsic pathway?

A

Extrinsic: initiation of clot
Intrinsic: Amplification of clot

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50
Q

Starting point of extrinsic pathway?

A

Factor VII: activated by Tissue Factor (thromboplastin)

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51
Q

What is first step in common pathway?

A

Factor X

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52
Q

What are intrinsic and extrinsic paths measured with?

A

Intrinsic: PT/INR
Extrinsic: PTT

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53
Q

What sets off factor XII in intrinsic path?

A
  1. Platelets
  2. HMWK
  3. Collagen
  4. Basement membrane
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54
Q

Steps in intrinsic path?

A

XII
XI
IX
VIII - ***VIII is cofactor for IX

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55
Q

What is required for both intrinsic / extrinsic paths to function that must be added before testing?

A

Ca

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56
Q

What is cofactor for X in common pathway?

A

Factor Va

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57
Q

Another name for factor II?

A

Thrombin - activated by X/V complex

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58
Q

What does thrombin do?

A

Break fibrinogen into fibrin which can now bind GP2a/3b on platelets causing them to stick

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59
Q

What does factor XIII do?

A

Activated by thrombin to stabilize thrombin meshwork

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60
Q

What is kallikrein? What does it do?

A
  • Activated by factor XII
    1. Enhances inflammation by converting HMWK to bradykinin
    2. Converts plasminogen to plasmin: plasmin activates C3 turning on complement
  • **TPA is better at this
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61
Q

Functions of bradykinin?

A

Increased:

  1. Pain
  2. Permeability
  3. Dilation
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62
Q

What does antithrombin act on?

A
  1. X

2. IIa

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63
Q

How does heparin work?

A

Potentiates antithrombin

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64
Q

What are the hemophilias and which is more common?

A

A: VIII deficiency - more common
B: IX deficiency

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65
Q

Which factors produced by liver?

A

II, VII, IX, X

  • Activated in vitamin K dependant process
  • VIII: made in endothelial cells
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66
Q

What is vitamin K necessary for?

A

Synthesis of: II, VII, IX, X, C, S

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67
Q

Enzyme activating Vitamin K and drug that blocks this action?

A

Vitamin K epoxide reductase: warfarin blocks this action

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68
Q

Warfarin Heparin effect monitored by what?

A

Warfarin: extrinsic
Heparin: intrinsic

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69
Q

Why do babies get vitamin K shot?

A
  • Important source of vitamin K is enteric flora and babies lack this so they need shot
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70
Q

What do proteins C/S do?

A

Cleave and inactivate factor V/VIII

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71
Q

What is factor V leiden?

A

Having factor V molecules that are resistant to cleavage of factor C and thus you are hypercoagulable

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72
Q

What to think if young person with DVT and no risk factors?

A

Factor V leiden

***Especially if person is white

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73
Q

What is secondary hemostasis?

A

The process of the coagulation paths with the goals of activating thrombin so that it can convert fibrinogen to fibrin

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74
Q

2 important factors of VwF?

A
  1. Bind platelet to collagen

2. Protect factor VIII

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75
Q

What does TXA2 do?

A
  1. Increases aggregation

2. Constricts to slow down blood

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76
Q

What leads to expression of GP2b/3a receptor?

A

Binding of ADP to platelets

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77
Q

Role of COX in platelets?

A

Generaties TXA2 from arachidonic acid in cell walls

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78
Q

What is bernard Soulier?

A

Deficiency in GP1b receptor

- Platelet can no longer ADHERE to collagen in damaged endothelium

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79
Q

When disease has mechanism similar do bernard Soulier?

A

VwF disease

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80
Q

What is ristocetin?

A
  • Induces binding of VwF to GP1b

- If ristocetin test is negative, you are either lacking VwF or its receptor

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81
Q

What does negative agglutination test indicate?

A
  • VwF disease

- Ristocetin should induce VwF binding so if test is negative you are missing VwF

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82
Q

What is glanzmann’s thrombasthenia?

A

Deficiency of GP2b/3a

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83
Q

First thing that occurs when endothelial layer of vessel is damaged?

A

Vessel constricts mediated by:

  1. Endothelin
  2. Neural stimulation
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84
Q

Where is VwF coming form?

A
  1. Platelets

2. Endothelial cells - majority

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85
Q

What is weibel palade body?

A

Found in vessel endothelial cell holds:

  1. VwF
  2. P selectin
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86
Q

What is P selectin?

A

Found in weibel palade body serving as speed bump in atherosclerosis

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87
Q

Signs of issues in primary hemostasis?

A

Mucosal and skin bleeding

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88
Q

What are mucosal and skin bleeding indicative of?

A

Disorder in primary hemostasis

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89
Q

Main risk in sever thrombocytopenia?

A

Intracranial bleeding

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90
Q

Petechiae sign of qualitative or quantitative issue with platelets?

A

Quantity

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91
Q

What is increased bleeding time indicative of?

A

Platelet issues

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92
Q

What is ITP?

A
  • Autoimmune disease w/ IgG against platelet antigens: usually IIa/IIIb
  • IgG is produced by spleen and macs in spleen will eat the bound platelets
  • ***Since IgG can cross placenta
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93
Q

Difference in ITP in kids and adults?

A

Kids: acute, self resolving after viral
Adults:

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94
Q

PT/PTT in ITP?

A

Normal, this is a platelet issue, coagulation cascade has not been touched

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95
Q

Last ditch therapy for ITP?

A

Remove spleen as this is both source of IgG and platelet destruction

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96
Q

What is microangiopathic hemolytic anemia?

A
  1. Pathologic formation of microthrombi in small vessels
  2. RBCs lysed as they traverse = schistocytes
  3. Thrombi formation drops platelet levels
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97
Q

2 disorders in which microangiopathic hemolytic anemia is seen?

A
  1. TTP

2. HUS

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98
Q

What is TTP?

A

“Thrombotic thrombocytopenic purpura”

  • Caused by decreased in ADAMTS13 increasing VwF multimers
  • Leads to abnormal platelet adhesion and microthrombi
  • Can be genetic or autoimmune
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99
Q

What does decreased ADAMTS13 cause and why?

A

TTP

  • Normally degrades VwF multimers
  • Since low in disease, multimers aggregate and activate paltelets
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100
Q

What is HUS?

A

“Hemolytic uremic syndrome”
Platelet microthrombi caused by endothelial damage from:
1. Drug
2. E-coli 0157

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101
Q

Main location of disease process in TTP and HUS?

A
  1. HUS: kidney

2. TTP: brain

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102
Q

What is blood smear with large platelets and and mild thrombocytopenia indicative of?

A

Bernard Soulier: BS is for “big suckers”
Enlarged: immature
Low levels: Don’t live as long without GP

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103
Q

Uremia impact on platelets?

A

Messes up both adhesion and aggregation

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104
Q

What is required for activation of clotting factors?

A
  1. Ca
  2. Phospholipid surface: surface of platelets
  3. activating substance
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105
Q

Signs of problems with secondary hemostasis?

A

Deep bleeds:

  1. Rebleeds after surgery
  2. Muscle bleeds
  3. Joint bleeds
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106
Q

What activates factor XII, VII?

A

XII: Subendothelial collagen
VII: Tissue thromboplastin

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107
Q

Disease similar to hemophilia A?

A

“Coagulation factor inhibitor”

  • Usually vs factor VIII
  • Will appear same with increase PTT however factor VIII levels are normal
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108
Q

What is the mixing study?

A

Distinguishes hemophilia A from coag inhibitor:

  • Mix patient with normal plasma
    1. If Hemophilia A: normal VIII in sample corrects and coagulation occurs
    2. If inhibitor: donor serum will not correct
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109
Q

What is failed mixing study indicative of?

A

Coagulation factor inhibitor

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110
Q

Lab results in VwF diseased?

A
  1. Increased bleeding time: primary disease
  2. Increased PTT: remember that VwF also protects factor VIII from degradation and VIII
  3. Abnormal ristocetin: ristocetin usually induces VwF function
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111
Q

Treatment for VwF disease?

A

Desmopressin: increase VwF release from weibel palade bodies

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112
Q

What is desmopressin used to treat?

A

VwF disease

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113
Q

Another name for warfarin?

A

Coumadin

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114
Q

What is HIT?

A

“Heparin induced thrombocytopenia”

  • Hep binds to platelet factor IV on surface
  • Ptn develops antibodies to this = platelet destruction
  • Platelet fragments can lead to thrombosis
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115
Q

What is DIC?

A
  • Pathologic activation of coag cascade
  • Forms microthrombi
  • Consumes platelets and clotting factors
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116
Q

When is bleeding from IV sites seen?

A

DIC

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117
Q

How do obstetric complications cause DIC?

A
  • Amniotic fluid contains Tissue thromboplastin with activates factor VII
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118
Q

When are Auer rods seen?

A

Acute promyelocytic anemia

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119
Q

Lab findings in DIC?

A
  1. Increased bleeding time
  2. Decreased PT/PTT
  3. Decreased fibrinogen
  4. Increased D dimer
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120
Q

3 actions of plasmin?

A
  1. Cleaves fibrin
  2. Cleaves fibrinogen
  3. Blocks aggregation
  4. Destroys clotting factors
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121
Q

What inactivates plasmin?

A

A-2-antiplasmin

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122
Q

Risk of radical prostectomy?

A
  • Release of urokinase which activates plasmin
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123
Q

Effect on cirrhosis on plasmin?

A
  • Decreased A-2-antiplasmin leading to over activation of plasmin
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124
Q

Lab findings in disease of fibrinolysis?

A
  1. Elevated PT/PTT: plasmin cleaves coags
  2. Increased bleeding time: plasmin inhibits aggregation
  3. Increased fibrinogen split products WITHOUT D dimer: D dimer is from fibrin and not clots are actually present
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125
Q

What does aminocaproic acid do?

A

Treats diseases of fibrinolysis as blocks activation of plasminogen

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126
Q

What does PGI2 do?

A
  • Block platelet aggregation

- Released by endothelium

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127
Q

What is heparin like molecule?

A
  • Released from endothelial activating antithrombin III

- Inactivates thrombin

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128
Q

What is thrombomodulin?

A
  • Anticoag released by endothelium
  • Takes thrombin and causes it to activate protein C instead of thrombin
  • C then inactivates V and VIII
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129
Q

How do we get elevated homocysteine and what is the risk?

A

Causes endothelial damage = thrombosis

  1. B12/folate deficiency
  2. CBS deficiency “cystathionine beta synthase”
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130
Q

How does B12/folate deficiency lead to elevated homocysteine?

A
  • Can no longer convert homocysteine to methionine
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131
Q

Interaction of B12/folate?

A
  1. Folate enters body as THF and is methylated
  2. To work in DNA gives methyl to B12
  3. B12 then gives methyl to homocysteine making it methionine = elevated homocysteine
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132
Q

Presentation and cause of homocystinuria?

A

Deficiency in CBS

  1. Thrombosis
  2. Lens dislocation
  3. Long, slender fingers
  4. Mental retardation
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133
Q

What is following indicative of:

  1. Thrombosis
  2. Lens dislocation
  3. Long, slender fingers
  4. Mental retardation
A

Homocystinuria

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134
Q

Function of factor C and S?

A

Inactive Factor V and VIII

- THink that if you double the S = 8, and if you put the Cs on top of each other you kind of have a 5

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135
Q

Associated risk with C/S deficiency?

A
  1. Warfarin skin necrosis

* Also remember that this is just a hypercoagulable state in general as C/S normally inactivate 5/8

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136
Q

What happens in Factor V leiden?

A

Factor V can no longer be inactivated by C/S

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137
Q

What is PTT not rising with standard Hep dose seen in?

A

Antithrombin III deficiency

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138
Q

How does heparin work?

A

Activates antithrombin III

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139
Q

Presentation of fat embolism?

A
  1. Petechiael rash on chest

2. Dyspnea

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140
Q

What causes gas embolism and presentation?

A

Gas embolism:

  1. Dyspnea
  2. Bone pain - infarct of bone if chronic
  3. Muscle pain
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141
Q

What is squamous cells and keratin debris in lung indicative of?

A

Amniotic embolism

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142
Q

Does inner or outer rectum drain to inguinals?

A

Outer

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143
Q

What do superficial inguinals drain? Exceptions?

A
  1. Cutaneous from umbilicus down
  2. External genitals
  3. Anus below dentate
    Exceptions (deep inguinal):
  4. Glans penis
  5. Posterior calf
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144
Q

Where does upper rectum drain lymph?

A

Inferior mesenteric

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145
Q

Does sickle cell trait present?

A

No

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146
Q

Benefit of sickle cell trait?

A

Protection from plasmodium falciparum

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147
Q

Function of BRCA1/2?

A

DSDNA breaks

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148
Q

Why does blood transfusion cause hypocalcemia?

A
  • Contains citrate which is an anticoag and also chelates Ca

* Ca is required for coagulation pathway so this makes sense

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149
Q

Where does VDJ rearrangement occur?

A

Bone marrow

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150
Q

Where does isotype switching occur?

A

Germinal centers

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151
Q

What does isotype switching allow for?

A

Isotype: changing of IgM to other Igs

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152
Q

What does G6PD do in RBCs?

A
  • Maintains NADPH levels
  • Low NADPH = cannot keep glutathione reduced
  • Cells at higher risk for damage
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153
Q

When is 14:18 translocation seen?

A

Follicular lymphoma = BCL 2 overexpression, a protooncogene

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154
Q

Brain tumor that likes cerebellum?

A
  1. Pilocytic astrocytoma: cystic with nodule on cyst
  2. Medulloblastoma “meduloBLUEstoma”
    - Small and thick
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155
Q

Cancer Downs is at risk for?

A

ALL

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156
Q

When is reddish urine that darkens in light seen?

A

Acute intermittent porphyria

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157
Q

What does topoisomerase one / two do?

A

1: single stranded nicks to relieve supercoil
2: double stranded nicks

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158
Q

What is enoxaparin?

A

LMW heparin

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159
Q

What do the oxabans do?

A

Inhibit factor X: look anti X is in the name

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160
Q

Mechanism of aspirin?

A

Irreversibly inhibits COXI and II

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161
Q

What does red pulp of spleen do?

A

Filters:

  1. Old and abnormal RBCs
  2. Circulating bacteria
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162
Q

What is elevated HBA2 indicated of?

A

Betathalesemia

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163
Q

Mutation in CML?

A

BCR/ABL 9/22

***Causes constitutive kinase activity

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164
Q

How do some cancer cell become chemoresistant?

A

ATP dependent efflux pumps

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165
Q

What does P53 do?

A

Cuase mutated cells to arrest in G1/S

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166
Q

Presentation of vincristine toxicty?

A

Numbness and parasthesias

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167
Q

When is 15:17 mutation seen?

A

AML: mutation in retinoic acid receptor

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168
Q

What is wiskott aldrich?

A
  1. Eczema
  2. Thrombocytopenia
  3. Recurrent infx
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169
Q

What is the following indicative of:

  1. Thrombocytopenia
  2. Eczema
  3. Recurrent infx
A

Wiskott aldrich

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170
Q

Problem in spherocytosis?

A

Problem in RBC cytoskeletal abnormalities

- Usually defects in spectrin or ankyrin

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171
Q

Treatment of spherocytosis?

A

Splenectomy

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172
Q

What happens in positive and negative selection?

A

T cells
Positive: bind self antigen = survive
Negative: bind self antigen = death

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173
Q

What happens to hemoglobin and hematocrit during pregnancy?

A
  • Total body volume greatly increases in pregnancy
  • Since HgB and hematocrit are [] dependant, these measures will drop
  • However, total RBC mass is the same and hasn’t changed
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174
Q

Diagnosis and microcytic / macro anemia?

A

Micro: MCV 100

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175
Q

What is broad problem in microcytic anemia?

A
  • For some reason there is decreased HbB causing erythroblasts to undergo extra division when making RBCs
  • **They are trying to keep [HgB] per RBC same so divide to make cell smaller to cope with lower HgB in body
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176
Q

What is responsible for giving the RBC its color?

A

HgB

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177
Q

What is heme composed of? Globin?

A 
Heme:
1. Fe
2. Protoporphyrin 
Globin:
1. AAs
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178
Q

Causes of microcytic anemia

A
  1. Low Fe
  2. Anemia chronic disease: Fe stuck in macs
  3. Sideroblastic anemia: low protoporphyrin
  4. Thalassemia: decreased globin
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179
Q

Disease with decreased globin?

A

Globin: Thalassemia
Protoporphyrin: Sideroblastic

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180
Q

What are heme and nonheme Fe?

A

Heme: from meat, > absorption

Non heme: plant

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181
Q

Where is Fe absorbed?

A 
Duodenum
"Iron First Bro"
Iron
Folate
B12
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182
Q

How is Fe absorbed from Gut?

A
  • Enterocyte takes up Fe from gut and uses ferroportin to send it into blood if needed
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183
Q

What is ferroportin?

A

“When you look at the ‘portin’ use that to remember its a transporter”

  • Transporter in enterocyte that moves Fe from gut into blood
184
Q

What is transferrin?

A
  • Binds Fe in blood taking in from ferroportin and either takes it to liver or macs for storage
185
Q

How is Fe stored?

A
  • In cells bound to ferritin
186
Q

That does TBC tell us?

A
  • How much transferrin is in blood
187
Q

Cause of Fe Deficiency anemia in adults in developing world?

A
  • Hookworms
188
Q

Fe2+ or 3+ more readily absorbable? How is 2 maintained

A
  • 2+, think “Fe2 goes IN TWO the body”

- Acidity keeps Fe in 2+ state

189
Q

How does gastrectomy lead to Fe anemia?

A
  1. Stomach is a very acidic place
  2. Acid keeps Fe in 2+ from
  3. 2+ is more absorbable
    * **Thus, if we remove stomach and its acidic environment, we have more Fe3+ which is more absorbable
190
Q

Relation to Ferritin and TIBC?

A

Inverse:

  1. When ferritin goes up, TIBC goes down
  2. When ferritin down, TIBC up
    * **Makes sense because if ferritin is low, Liver will realize body needs more Fe so will make more transferrin thus increasing TIBC
191
Q

What is the first stage of Fe deficient anemia?

A

Normocytic: marrow wants to make normally pretty RBCs so will make them with normal Fe in then, but will just make fewer for them
- Eventually this becomes microcytic

192
Q

What is koilonychia?

A

“Spoon shaped nails”

- Seen in Fe deficient anemia

193
Q

What is FEP?

A

“Free erythrocyte protoporphyrin”

  • Increased in Fe deficient anemia
  • Makes sense as normal proto but low Fe, so some proto in HgB will be unbound
194
Q

Explain anemia of chronic disease?

A
  • Hepcidin is an acute phase reactant which sequesters Fe into storage to bacteria can’t use
  • Thus, macs can’t give to erythroblasts
  • Hepcidin also suppresses EPO
195
Q

What is hepcidin?

A
  • Acute phase reactant responsible for anemia chronic disease as it functions to:
    1. Sequester Fe to save from bacteria
    2. Decrease EPO production
196
Q

What is sideroblastic anemia?

A
  • Defect in protoporphyrin synthesis = microcytic anemia
197
Q

Steps in protoporphyrin synthesis?

A
  1. Succinyl CoA converted to aminolevulinic acid (ALA): ALA synthase catalyzes
    - Rate limiting step with B6 as cofactor
  2. ALA -> porphobilinogen: ALA-dehydrogenase
    ……
  3. Fe joined to protoporphyrin by ferrochelatase: occurs in mitochondria
198
Q

Importance of B6 in sideroblastic anemia?

A
  • B6 is cofactor for ALAS which converts SCoA into ALA: rate limiting step
199
Q

What does ferrochelatase do?

A

Joins Fe to protoporphyrin

200
Q

Mitochondrial significance on sideroblastic anemia?

A
  1. Sidero is problem with protoporphyrin synthesis
  2. Ferrochelatase combines Fe and proto in mitochondria of erythroblast
  3. If mitochondria not making enough proto, Fe will still be entering cell and accumulate in mitochondria ringing nucleus “Ringed sideroblasts”
201
Q

Normal cause of sideroblastic anemia?

A

Defect in ALAS: converts SuccCoA -> ALA which is rate limiting step in protoporphyrin synthesis

202
Q

Acquired causes of sideroblastic?

A
  1. Lead: denatures ALAD and ferrochelatase
  2. Alcohol: mitochondrial poison
  3. Deficient B6: ALAS cofactor
203
Q

When is B6 deficiency seen?

A

Isoniazid treatment

204
Q

What does Isoniazid treatment predispose for?

A
  • B6 deficiency = sideroblastic anemia as B6 is cofactor for ALAS which is rate limiting enzyme in protoporphyrin synthesis
205
Q

What findings in sideroblastic?

A 
This is an Fe overloaded state: "imagine that as the Fe is accumulating and ringing the nucleus, it is leaking out into blood"
Ferritin: increased
TIBC: decreased
Serum Fe: Increased 
% Sat: increased
206
Q

What is thalassemia?

A

Decreased production of globin chains = decreased HbB = microcytic anemia

207
Q

What are carriers of thalassemia protected against?

A
  • Plasmodium falciparum
208
Q

2 types of thalassemia?

A

Alpha: decreased A chains
Beta: decreased B chains

209
Q

Difference in HbA and HbA2?

A

HbA: 2 alpha and 2 beta chains
HbA2: 2 alpha and 2 delta chains

210
Q

Is the alpha or beta globin chain more important?

A

Alpha: is present in fetal, HbA, and A2

- Beta chain is only present in HbA

211
Q

Genetics of A-thalassemia?

A
  • There are 4 alpha alleles on chromosome 16

- DELETION leads to varying degrees of A thalassemia

212
Q

Degrees of A-thalassemia?

A
  1. 1 allele: asymptomatic
  2. 2 alleles: cys or trans
  3. 3 alleles: severe anemia HbH damages RBC
213
Q

Explain cis and trans A thalassemia?

A

4 alleles for A chain on chromosome 16
Cys: knock out 2 alleles on some chromosome
1. Worse: can pass both to offspring
2. Seen in asians
Trans: knock out 2 alleles on diff chromosome
1. Seen in africa
2. Less severe: lower risk of passing to offspring

214
Q

What occurs in A-thalassemia with 3 alleles?

A
  • B chains form tetramers (HbH) that damage RBCs

- Fetal HgB does not show issues

215
Q

What is HbH?

A
  • Tetramer of B globin seen in A thalassemia with 3 allele deletion
  • Damages RBCs
  • Not present in fetus
216
Q

Genetics of beta thalassemia?

A
  • 2 alleles on chromosome 11
  • Due to MUTATIONS (A was due to deletion)
    1. Beta null: one allele cant produce any
    2. B+: decreased from one allele
217
Q

What are target cells?

A

Blebs of hemoglobin in normally concave /pale central RBC

  1. Decreased cytoplasm
  2. Increased membrane
    * **Think like deflating basketball making its surface more malleable
218
Q

What is B thalassemia minor?

A
  • One normal B allele and one B+
  • Slightly decreased HbA
  • Slight increase HbA2 (delta for beta)
219
Q

What si B thalassemia major?

A
  • Double B null
  • Fetal HbG is protective at birth
  • Rapid regression after
  • Alpha tetramers damage RBCs
  • Massive erythroid hyperplasia: in other organs
220
Q

Which anemia is massive erythroid hyperplasia seen in? Presentation?

A

B Major

  1. Expansion of skull and facial bones: “crew cut appearance on Xray”
  2. HSM: spleen starts making again
221
Q

When are circulating RBCs seen?

A

B major: being made in places other than marrow allowing nucleated cells to sneak out

222
Q

2 most common causes macrocytic anemia?

A
  1. B12

2. Folate

223
Q

Broad problem in macrocytic anemia?

A
  • Cells undergo one LESS division due to lack of DNA precursors
224
Q

What takes methyl group from THF so it can participate in DNA synthesis?

A

B12 - B12 then gives methyl to homocysteine

225
Q

What takes methyl group from B12?

A

Homocysteine - becomes methionine once it receives the methyl group

226
Q

Presentation of B12/folate deficiency?

A
  1. Megaloblastic anemia
  2. HYPERsegmented neuts
  3. MEGALOblastic change in all dividing cells: ie, epithelial cells of gut would be larger
227
Q

Other cause of macrocytic anemia?

A
  1. Alcohol
  2. Liver disease
  3. Drugs - 5 fu
228
Q

Where is folate found?

A
  • Green vegetabls and fruits

- Absorbed in jejunum “Iron First Bro”

229
Q

How does methotrexate cause megaloblastic anemia?

A

Inhibits dihydrofolate reductase = folate deficiency

230
Q

Presentation of folate deficiency?

A
  1. Glossitis
  2. Increased homocysteine
  3. Normal methylmalonic acid: B12 converts this to succinyl CoA
  4. Hypersegmented neuts
231
Q

In megaloblastic anemia, what does normal methylmalonic acid tell us?

A
  • B12 levels are normal: B12 converts this to succinyl CoA
232
Q

What is R binder?

A
  • Protein made by salivary gland to bind B12 in mouth
  • Cleaved in SI by proteases allowing to bind IF` from parietal cells in stomach
  • Absorbed in ileum when bound to IF
233
Q

Folate of B12 deficiency take longer to develop?

A

B12: Body has very low folate stores

*There are large stores of B12 in liver

234
Q

Cause of low B12 other than pernicious?

A
  1. Pancreatic insufficiency: no R binder cleavage
  2. Ileal damage in crohn’s
  3. Diphyllobothrium latum
235
Q

Dietary cause of low B12?

A

Vegans

236
Q

When is glossitis seen?

A
  1. Plummer vinson

2. Megaloblastic anemia

237
Q

When is methylmalonic acid elevated?

A

B12 deficiency as B12 converts to succinyl CoA

238
Q

What causes subacute combined degeneration? How?

A

B12: methylmalonic acid builds up in myelin of spinal cord

239
Q

2 main classes of normocytic anemia?

A
  1. Underproduction: low retic

2. Peripheral destruction: normal retic

240
Q

What do reticulocytes look like?

A
  • Bigger than normal

- Slightly blue cytoplasm: RNA still in cytoplasm

241
Q

What is normal retic count?

A

1-2%

- If there is anemia and marrow works, should be >3%

242
Q

Why is retic falsely elevated in anemia?

A
  • Retics are measured as % of total RBC
  • Anemia = low RBCs = inaccurate calc
  • *Need to adjust
243
Q

How is the retic adjusted?

A

Multiply by hematocrit / 45

244
Q

Where are RBCs destroyed in extravascular hemolysis?

A

RES system:

  1. Liver
  2. Lymph nodes
  3. Macs in spleen
245
Q

What does protoporphyrin become?

A

Unconjugated Bilirubin

246
Q

Presentation of extravascular anemia?

A
  1. Splenomegally
  2. Unconjugated jaundice: liver can’t keep up w/ spleen
  3. Bili stones
  4. Hyperplastic marrow
247
Q

What is haptoglobin?

A
  • When RBCs destroyed in vessels, haptoglobin binds HgB and takes it to spleen
248
Q

Lab findings in intravascular hemolysis?

A
  1. Decreased haptoglobin
  2. Hemoglobinuria
  3. Hemosiderinuria: Fe in tubular cells eventually is sloughed off into urine
  4. Hemoglobinemia
249
Q

What is going on in spherocytosis?

A
  • Normally, cytoskeleton is anchored to membrane via ankyrin and spectrin giving RBC its its biconcave shape
  • Tethering is lost in sphero, leading to blebs coming off and transition to sphere shape
250
Q

Why does spherocytosis cause anemia?

A
  • Loss of biconcavity prevents spherocytes from moving through spleen
  • Spleen eats them = anemia
251
Q

What is MCHC and when is it seen?

A

“Mean corpuscular hemoglobin []”

- Increased in spherocytosis as cell is shrinking in size as it blebs leading to greater []

252
Q

Diagnosis in spherocytosis?

A
  • Increased fragility in hypotonic solution in osmotic fragility test
253
Q

Treatment of spherocytosis?

A

Splenectomy: RBCs still work fine spleen is just eating them
1. Howell Jolly bodies will still be seen

254
Q

What are howell jolly bodies?

A

RBCs that still have a bit of nucleus left in them

  • Normally is spleens job to remove these but if spleen is gone you will see these
  • Appear as RBC with little blue spots in them
255
Q

What causes sickle cell?

A
  • Mutation in B chain of HgB replacing glutamic acid (hydrophilic) with valine (hydrophobic)
256
Q

How does HbS causes disease?

A
  • Polymerizes into needle like sickle cells when deoxygenated / stressed
257
Q

How does hydroxyurea help in sickle cell?

A

HbF is protective in sickle cell

- Hydroxyurea causes this to form

258
Q

Is sickle intra or extravascular?

A

Both: spleen eats them and some also rupture iin the vessels

259
Q

What does sickle form target?

A
  • Membrane damage dehydrates cell cytoplasm
  • This makes the basketball effect we talked about before where the membrane is now malleable
  • HgB can now move to center of cell making int darker instead of the area of pallor
260
Q

What is dactylitis?

A
  1. Swelling of bones in hands / feet due to vaso occlusive infarcts of bones
    * Common presentation of sickle cell in kids
261
Q

What type of infx at risk for if now spleen?

A
  1. Encapsulated organisms

2. Salmonella paratyphi osteomyelitis

262
Q

Most common cause of death in sickle?

A
  1. Kids: encapsulated orgs

2. Adults: acute chest syndrome

263
Q

What does sickle do to kidney?

A

Renal papillary necrosis

- Hematuria and proteinuria

264
Q

Where does sickle cell trait usually present?

A

Renal medulla: very hypoxic and hypertonic which promotes sickling
- Leads to microinfarcts, microalbumin, and inability to [] urine

265
Q

What does metabisulfite do?

A

Used to diagnose sickle cell trait

- Forces cells to sickle when added

266
Q

How much HbA is there in sickle cell?

A

Zero: HbA is 2 alpha and 2 beta

- In sickle the issue is with both alleles of the beta chain so we have no beta

267
Q

What is hemoglobin C disease?

A
  • B globin mutation of glutamic acid for lyCCCCine

- Less severe with HbC crystals seen in smear

268
Q

What are MIRL and DAF?

A

Both found on surface of RBC and serve to inactivate complement when it comes close to RBC:
DAF: “Decay accelerating factor”
MIRL: “Membrane inhibitor of reactive lysis”
***Both connected to RBC surface by GPI

269
Q

What is GPI?

A

Connects both DAF and MIRL to RBC membrane protecting them from complement
- Is deficient in PNH

270
Q

What happens to acid base status at night? How does it cause PNH?

A
  • Breathe shallow increasing CO2 creating respiratory acidosis in body
  • Acidosis can activate complement and this is what happens in PNH
  • Wake up with dark urine as activation of complement leads to RBC lysis
271
Q

What is PNH?

A

“Paroxysmal nocturnal hemoglobinuria”

  • Defect in GPI = no DAF / MIRL to protect RBCs from complement activation caused by respiratory alkalosis while we sleep
272
Q

How do we screen for PNH?

A
  1. Place sucrose in serum to activate complement then check for lysis
  2. Do same with acid
  3. Screen for DAF / CD55
273
Q

What other disease is at risk in PNH?

A

AML: makes sense as PNH is problem with myeloid precursors just like AML

274
Q

How do RBCs protect selves from oxidative stress?

A
  • Glutathione reduces free radicals and is oxidized
  • To used oxidized glutathione again, must be reduced by NADPH
  • G6PD is required to created NADPH
275
Q

What does G6PD do?

A

Produces NADPH so glutathione can be reduced so cell can continue to protect self from oxidative stress

276
Q

What is issue with G6PD in disease?

A

Has a reduced half life, 2 types:
African: mildly reduced
Mediterranean: greatly reduced = cells die much younger

277
Q

What causes GRPD to manifest?

A
  1. Sulfa drugs
  2. Primaquine
  3. Dapsone
  4. Infections
  5. Fava beans
278
Q

Disease process in G6PD?

A
  • Oxidative stress causes HgB to precipitate as heinz bodies
  • Spleen eats heinz bodies = bite cells
279
Q

Presentation of G6PD?

A
  1. Hemoglobinuria

2. Back pain

280
Q

What happens in IgG Hemolysis?

A

Extravascular hemolysis

  • IgG coats RBC, splenic macs eat it off
  • Spherocytes now form
  • IgG is bound in warm central body
281
Q

IgG or IgM warm / cold?

A

IgM: cold, distal body, intravascular
IgG: warm, central body, extravascular

282
Q

When is cold agglutination seen?

A
  1. Mycoplasma pneumonia

2. Mono

283
Q

What does coombs test diagnose?

A

Hemolytic anemia:

1. Direct: presence of Ig on RBC

284
Q

How does direct coombs work?

A
  1. Anti IgG added to serum

2. Will agglutinate if RBC is coated

285
Q

What does indirect coombs do?

A

Does patient have IgG in serum

  1. Anti IgG and test RBCs mixed with serum
  2. Will agglutinate if patient had IgG in serum
286
Q

Parvo B19 effect on RBCs?

A
  • Hurts RBC progenitors halting erythropoiesis

- Anemia if already marrow stress

287
Q

What is aplastic anemia?

A
  • Damage to HSC = pancytopenia with low retic
288
Q

2 useful uses of desmopressin?

A
  1. Increase factor VIII and VwF in hemophilia A and VwF disease
  2. Water resorption in diabetes insipidus
289
Q

What does VwF increase stability of?

A

Factor VIII

290
Q

Presentation of platelet issues?

A
  1. Prolonged bleeding time
  2. Easy bruising
  3. Mucosal bleeding
291
Q

What are metalloproteinases?

A

Zinc enzymes that degrade ECM allowing for tumor invasion

292
Q

What is PD1?

A

“Programmed death receptor”

  • Found on surface of T cells
  • When bound by its ligand leads to cell death
  • If blocked can help cell fight cancer
293
Q

Type of drug to treat chemo vomit?

A

5HT blocker

294
Q

HSC marker?

A

CD34+

295
Q

What do myeloblasts become?

A
  1. Neuts
  2. Basos
  3. Eos
296
Q

How do treat chemo induced neutropenia?

A
  1. GCSF

2. GMCSF

297
Q

How would infection cause neutropenia?

A

Most neuts will be out of blood and in tissue in sever infx so they would not be measured on a blood test

298
Q

What is not developed in digeorge?

A

3/4 pharyngeal pouch: lymphopenia as no thymus

299
Q

How does cortisol cause lymphopenia?

A

Induces apoptosis in lymphocytes

300
Q

Cell in body most sensitive to radiation?

A

Lymphocytes

301
Q

2 things causing neutrophilic leukocytosis?

A
  1. Tissue necrosis

2. Bacterial infx

302
Q

What is a left shift?

A

Immature neuts in blood: characterized by decreased FC receptors that would recognize Ig so they dont function as well

303
Q

What does decreased CD16 mean?

A

Immature neuts in blood as this is for FC receptor

304
Q

Marker for immature neuts?

A

Decreased CD 16

305
Q

Cortisol impact on neuts?

A

Disrupts adhesion of marginated cells attached to vessels causing them to enter serum = neutrophilia

306
Q

3 scenarios for eosinophilia?

A
  1. Parasitic infx
  2. Allergy
  3. Hodgkin’s lymphoma: caused by increased IL5
307
Q

When is basophilia seen?

A

CML

308
Q

Change in cell count in CML?

A

Basophilia

309
Q

Change in cell count in CML?

A

Basophilia

310
Q

Cells responsible for viral infx?

A

CD8

311
Q

Bordetella pertussis impact on blood count?

A

Lymphocytosis: clocks T cells from leaving blood to enter nodes so count goes up

312
Q

Mono impact on cell count?

A

Lymphocytosis of CD8

313
Q

What is found in cortex and paracortex of nodes?

A

Cortex (outer most) - B cells

Paracortex - T cells

314
Q

What is periarterial lymphatic sheath?

A

Area surrounding artery in spleen where T cells are found

- Area responsible for splenomegaly in mono

315
Q

What does the monospot test do?

A
  • Detects heterophile IgM antibodies seen in mono
  • **Does not detect CMV
  • Need to follow up test with test for EBV viral capsid antigen
316
Q

What happens if you give penicillin to mono patient?

A
  • Get a rash
317
Q

What is hallmark of acute leukemia?

A
  • MyeloBLASTS and lymphoBLASTS cannot mature to begin to pile up
  • **> 20% blasts in marrow is diagnostic
318
Q

Acute presentation of leukemia?

A
  1. Blasts are pushing out other cells in marrow:
    a. Anemia
    b. Thrombocytopenia
    c. Neutropenia
  2. High blast count in serm
319
Q

Blasts on histo?

A

Large basophilic cell with punched out nucleus (nucleolus)

320
Q

Difference between AML and ALL?

A

ALL: Accumulation of LYMPHOblasts
AML: accumulation of MYELOblasts

321
Q

What is TDT positivity indicative of?

A

LYMPHOblasts: DNA polymerase only found in there nucleus

322
Q

How to tell LYMPHOblast from MYELOblast?

A

AML: MPO+ = AUER rods
ALL: TDT+

323
Q

What is MPO positivity indicative of?

A

AML - in crystallized form becomes auer rod

324
Q

When are auer rods seen?

A

AML - this is crystallized MPO on staining

325
Q

Common person ALL found in?

A

> 5 yo kid with Down syndrome

326
Q

Cancer seen in > 5 yo kid with Down syndrome?

A

ALL

327
Q

How to tell B-ALL from T-ALL?

A

BOTH are TDT positive:
B-ALL: CD 10, 19 20
T-ALL: CD2 - 8 (think T cells are usually 4 and 8)

328
Q

What are the following markers for CD 10, 19 20?

A

B-ALL

329
Q

B-ALL treatment?

A

Great response to chemo with prophylaxis needing for scrotum and CSF - think, BALL affects your BALLS
- Chemo cannot cross barriers here so needs to be injected

330
Q

Translocations in BALL?

A

19 - 21: kids, good prognosis

9 - 22: Adults, poor prognosis

331
Q

Cancer with CD 2 - 8 markers?

A

TALL

332
Q

Presentation of TALL?

A

TTTHymic mass in TTTTeenager

***Actually called a lymphoma since there is a mass whereas a leukemia means cancer is in serum

333
Q

Common mutation in AML?

A

15 - 17 translocation

- RAR disruption = promyelocytic accumulation

334
Q

What is 15 - 17 translocation seen in?

A

AML

335
Q

Treatment for AML?

A

All trans retinoic acid causes cells to mature “ATRA”

336
Q

What is ATRA treatment for?

A

AML

337
Q

What should you think if patient has cancerous swelling of gums?

A

Acute MONOcytic leukemia

338
Q

Cancer in down > 5 yo?

A

5: ALL

339
Q

What are myelodysplastic syndromes?

A

CYTOpenias with Hypercellular bone marrows that can cause leukemias arising in background of radiation

340
Q

What is chronic leukemia?

A

Malignant proliferation of MATURE circulating LYMPHOcytes

- Characterized by high WBC count

341
Q

What is CLL?

A
  • Proliferation of Naive B cells
  • Express CD 5 and 20
  • Increased lymphocytes and smudge cells seen on smear
342
Q

What is CD 5/20 indicative of?

A

CLL

343
Q

When are smudge cells seen?

A

CLL

344
Q

Complications of CLL?

A
  1. HYPOgammaglobulinemia: B cells are not maturing to secrete Ig as they should
    * Most common cause of death = infx
  2. Autoimmune hemolytic anemia: B cells are making messed up Ig that is attacking self
  3. Transform to diffuse large B cell lymphoma
345
Q

What is hairy cell leukemia?

A
  • Malignant proliferation of mature B cells
  • TRAP positive
  • Hairy cytoplasmic processes
346
Q

What is TRAP + indicative of?

A

Hairy cell leukemia, think “Look out, its a hairy TRAP!!!!!!!!”

347
Q

Hairy cell presentation?

A
  1. Splenomegally in red pulp: atypical
  2. Absent lymphadenopathy
  3. Dry tap on marrow aspiration
    Think, they are TRAPPED in spleen so can make it nodes or marrow
348
Q

Hairy cell treatment?

A

2-CDA: adenosine deaminase inhibitor causing adenosine to accumulate to toxic levels in B cells

349
Q

What is ATLL?

A

“Adult T cell leukemia / lymphoma”

  • Neoplasm of Mature CD4+ T cells
  • HTLV1 association in caribbean and japan
350
Q

Cancer to think if HTLV1?

A

ATLL “Adult T cell leukemia / lymphoma”

351
Q

Presentation of ATLL?

A
  1. Rash: T cells like to go to spleen
  2. Lymphadenopathy
  3. Splenomegally
  4. Lytic bone lesions and HYPER Ca
352
Q

Blood cancer with lytic bone lesions?

A

ATLL

353
Q

What is mycosis fungoides?

A
  • Neoplasm of mature CD4+ T cells presenting with:
    1. Skin rash, plaques, nodules
    2. Pautrier microabscesses in skin
    3. Sezary syndrome: cerebriform nuclei on smear
354
Q

When are Pautrier microabscesses seen?

A

Mycosis fungoides

355
Q

When is cerebriform nuclei on smear seen?

A

Mycosis fungoides

356
Q

What is a myeloproliferative disorder?

A
  • Accumulation of mature myeloid cells

- All myeloid cells are increased but classified on dominant form

357
Q

Associated risks in myeloproliferative disorders?

A
  1. Hyperuricemia / gout: developing lots of cells so turning over rapidly
  2. Marrow fibrosis
  3. Transform to acute leukemia
358
Q

What is end result of purine degradation path?

A

Uric acid

359
Q

What is CML?

A

“Chronic Myeloid leukemia”

  • Predominant proliferation of granulocytes
  • Basophils are characteristically overproduced
360
Q

Mutation in CML?

A
  • 9/22 BCR/ABL fusion = increased TK activity
361
Q

What is imatinib treatment for?

A

CML

362
Q

How to treat CML?

A
  • Imatinib: blocks overexpressed TK activity
363
Q

Worst CML complication?

A

Transformation to acute leukemia: AML or ALL

364
Q

3 ways to distinguish CML from leukemoid rxn:

A

Leukemoid is natural increase granulocyte to infx. In CML we have:

  1. LAP negative granulocytes
  2. 19/22 translocation
  3. Increased basophils
365
Q

What is polycythemia vera?

A

Neoplastic proliferation of RBCs

  • Granulocytes and platelets also increased
  • JAK2 kinase mutation seen
366
Q

What is JAK2 mutation seen?

A
  1. Polycythemia vera
  2. Essential thrombocythemia
  3. Myelofibrosis
367
Q

Symptoms of polycythemia vera?

A

Hyperviscosity of blood:

  1. Blurry vision and headache
  2. Thrombosis: bud chiari
  3. Flushed face
  4. Itching after bathing: mast cells degranulate in hot water
368
Q

When is itching after bathing seen?

A

Polycythemia vera: mast cells degranulate in hot water

369
Q

Most common cause of budd chiari?

A

Polycythemia vera

370
Q

Treatment for polycythemia?

A
  1. Phlebotomy

2. Hydroxy urea

371
Q

What is hydroxyurea used to treat?

A

Polycythemia vera

*Also useful in sickle cell as it causes HbF to form

372
Q

Characteristics of reactive lung disease from polycythemia?

A
  1. Low O2

2. Increased EPO

373
Q

Mutation in essential thrombocythemia?

A

JAK2 kinase

374
Q

What doesn’t thrombocythemia not have risk for gout/hyperuricemia?

A
  • Platelets do not have nucleus so not creating uric acid via purine degradation path when being demolished
375
Q

What is myelofibrosis?

A
  • Neoplastic proliferation of megakaryocytes
  • JAK2 mutation
  • Are secreting excess PDGF = marrow fibrosis
376
Q

What is fibrosis of marrow seen in?

A

Myelofibrosis: driven by JAK2 mutation in megakaryocuytes

377
Q

When is leukoerythroblastic smear seen?

A

Myelofibrosis:

  • The marrow is shot so extramedullary creation of cells occurs
  • Places outside of marrow cannot filter immature cells so we are seeing blasts of all types in the smear
378
Q

When are tear drop cells seen?

A

Myelofibrosis

379
Q

What is multiple myeloma?

A
  • Malignant growth of PLASMA cells in bone marrow

- High IL6 often seen: important plasma cell growth factor

380
Q

What is high IL6 indicative of?

A

Multiple myeloma: important plasma cell growth factor

381
Q

What does multiple myeloma cause back pain with hyper Ca?

A
  • Plasma cell releases osteoclast activating factor which turns on RANK on the osteoclasts
  • Lytic lesions seen in vertebrae and skull
  • Increased risk of fracture
382
Q

Multiple myeloma presentation?

A
  1. Increased fracture risk
  2. Lytic bone lesions
  3. Hyper Ca
  4. Risk for infx: Increased Ig lacks antigen diversity
  5. Primary AL amyloidosis: plasma overproduces light chain
  6. Proteinuria: bence jones proteins
  7. Renal failure: same as above
383
Q

What does electrophoresis look like in multiple myeloma?

A

“M Spike” monocolona Ig

  • Spike in gamma region from increased IgG
  • **Most commonly is IgG or IgA
384
Q

When is rouleaux formation seen?

A

Multiple myeloma from increased Ig causing RBCs to aggregate

385
Q

When are bence jones proteins seen?

A

Multiple myeloma: these are the same excess light chains being excreted that are causing the amyloidosis

386
Q

What to think if M spike seen but not other myeloma features?

A

MGUS “Monoclonal gammopathy of unexplained significance”

387
Q

What is waldenstrom macroglobulinemia?

A
  • B cell lymphoma with monoclonal IgM
388
Q

What are langerhans cells?

A

Dendritic cells of skin

389
Q

What is langerhans cell histiocytosis?

A
  1. Neoplastic langerhan cell proliferation
  2. Birkbeck / tennis racket cells on EM
  3. CD1a/s100 positive
390
Q

When are Birkbeck / tennis racket cells seen?

A

Langerhans cell histiocytosis

391
Q

When are CD1a/s100 positive cells seen?

A

Langerhans cell histiocytosis

392
Q

What is eosinophilic granuloma?

A

Benign proliferation of langerhan cells in bone

- Pathologic fracture in kid with no skin involvedment

393
Q

What to think when pathologic fracture in kid with no skin involvement?

A

Eosinophilic granuloma

394
Q

When are painful and painless lymphadenopathy seen?

A

Painful: acute infx being drained
Painless: chronic infx, cancer, lymphoma

395
Q

When are lymph node follicles enlarged?

A
  1. RA

2. Early HIV

396
Q

When are lymph node para cortexes enlarged?

A

Viral infx: this is where T cells live

397
Q

When are sinus histiocytes of nodes enlarged?

A

Draining tissue with cancer

398
Q

What lives in medulla, cortex, and paracortex of LN?

A

Cortex: B cells
Paracortex: T cells
Medulla sinus histiocytes

399
Q

What is lymphoma?

A

Proliferation of lymphoid cells that forms a mass

- Can be in node or outside

400
Q

2 major types of lymphoma?

A
  1. Hodgkins: 40%

2. Non hodgkin’s: 60%

401
Q

Where is mantel found?

A
  • Surrounds follicles which are found in cortex
402
Q

Where is marginal zone found?

A

Surrounding mantle zone with surrounds the follicle which is found in cortex

403
Q

3 lymphomas arising from small B cells?

A
  1. Follicular
  2. Marginal
  3. Mantle
404
Q

What is CD20 marker for?

A

B cells

405
Q

What is follicular lymphoma?

A
  • CD20 B cells making follicle like nodules

- Painless generalized lymphadenopathy throughout body

406
Q

Genetics of follicular lymphoma?

A
  • 14:18 translocation of heavy chain / BLC2

- IgH is highly expressed so BCL2 now is overexpressed

407
Q

What does BCL2 do?

A
  • Stabilizes mitochondrial membrane preventing cytochrome C from leaving and killing
  • Overexpressed in follicular lymphoma = indestructible self reactive B cells which no longer can be killed
408
Q

When is 14:18 translocation of BLC2 seen?

A

Follicular lymphoma: BCL2 stabilizes mitochondrial membrane preventing cytochrome C from leaving
- Indestructible self reactive B cells which no longer can be killed

409
Q

Follicular lymphoma treatement?

A

Rituximab: anti CD20 Ig

410
Q

How to distinguish follicular lymphoma from hyperplasia seen in rxn to normal infx?

A
  1. BCL2 expression in follicles
  2. Monoclonality: polyclonal expansion is seen in infx
  3. Lack of tingible body macs in germinal centers: you expect to see macs if infx
411
Q

What is mantle cell lymphoma?

A
  • Neoplastic CD20 B cells expand in mantle zone “area immediately adjacent to follicle”
412
Q

Mutation driving mantle cell lymphoma

A
  • 11:14 translocation placing Cyclin D next to IgH

- Overexpression of cyclin D allows cells to move from G1 - S phase when they should not

413
Q

Where is the Ig Heavy chain found?

A

Chromosome 14

414
Q

What does cyclin D do?

A

Help cell move from G1 - > S phase

415
Q

Is marginal zone always present in LN?

A
  • No, when there is infx and germinal centers form the marginal zone forms
416
Q

What is marginal zone lymphoma associated with?

A
  1. Hashimotos
  2. Sjogren’s
  3. H pylori
417
Q

2 forms of Burkitt lymphoma?

A

African: jaw mass
Sporadic: abdominal mass

418
Q

Burkitt’s genetics?

A
  • 8:14 translocation of cMYC oncogene which promotes cell growth
  • Starry sky appearance on histo
419
Q

When is starry sky appearance seen?

A

Burkitt’s

420
Q

What is diffuse large B cell lymphoma?

A
  • Large B cells that grow diffusely in sheets
  • Most common NHL
  • Very aggressive
421
Q

What occurs in hodgkin’s lymphoma?

A
  • Reed sternberg cells secrete cytokines that dram in inflammatory cells leading to mass
422
Q

What are reed sternberg cells?

A
  • Found in hodgkin’s lymphoma
  • CD15/30 +
  • Large B cell with prominent nucleoli and multilobed nuclei
423
Q

What cell is CD15/30?

A

Reed sternber

424
Q

Presentation of nodular sclerosis?

A
  • Enlarging cervical/mediastinal node in young female

- Fibrosis dividing nodes with RS cells in lacunar areas

425
Q

Lymphocyte rich or depleted have better prognosis?

A

Rich

426
Q

What does IL5 do?

A

Call in eosinophils

427
Q

When are Bite cells seen?

A

X linked G6PD deficiency

428
Q

Inheritance of enzymes and proteins?

A

Enzymes: recessive
Proteins: autosomal dominant

429
Q

When are acanthocytes seen?

A
  1. Liver disease

2. Abetalipoproteinemia

430
Q

When are target cells seen?

A 
"HALT" when you reach the target
Hemoglobin C disease 
Asplenia
Liver Disease 
Thalassemias
431
Q

When are Heinz bodies seen?

A

Heinz G6PD deficiency, which the spleen taking a bite out of them

432
Q

2 main categories of microcytes?

A
  1. Problem making heme

2. Problem making globin

433
Q

When is basophilic stippling seen?

A

Lead poisoning

434
Q

How to treat lead poisoning?

A
  1. Dimercaperil
  2. EDT
  3. Succinyl CoA
435
Q

What to give in patient low in clotting factors?

A

Fresh frozen plasma`

436
Q

What is cryoprecipitate?

A

Factor VIII, XIII, VwF, fibrinogen

437
Q

What does TNF-alpha do?

A

Responsible for cachexia seen in cancer: causes appetite suppression and increased BMR which makes senses when you think about symptoms

438
Q

What is start codon in translation?

A

AUG - “translation starts in august”

439
Q

What does rituximab do?

A

Target surface CD120

440
Q

Signs of carcinoid?

A
  1. Flushing
  2. Bronchospasm
  3. Watery Diarrhea
  4. Right heart valve plaques
441
Q

What is increased 5 HIAA in urine indicative of?

A

Carcinoid: this is a metabolite of 5HT - think that they both start with 5 H so makes sense that is metabolite

442
Q

Lab studies in hemolytic anemia?

A
  1. Increased LDH

2. Decreased Haptoglobin

443
Q

Importance of folic acid in DNA synthesis?

A

Carbon donor required for purine and pyrimidine synthesis

444
Q

Inheritance of G6PD?

A

X recessive

445
Q

Mechanism of allopurinol?

A

Xanthine oxidase inhibitor

446
Q

What does RB do and how is it regulated?

A

Regulates G-> S transition

  • Dephosphorylated: active, cell cant progress
  • Phosphorylated: inactive, done by cyclins, cell progreses
447
Q

What reduces hemorrhagic cystitis seen in chemo?

A

MENSA

448
Q

KRAS, APC, BRCA1, RB oncogene or tumor suppressor?

A 
KRAS: Proto oncogene 
APC: suppressor 
BRCA1: suppressor 
RB:  suppressor 
TP53: suppressor
449
Q

Function of MYC?

A

Oncogene that is a transcription activator

450
Q

Which opioid side effects do not go away?

A
  1. Constipation

2. Miosis

451
Q

Disease process in inflammatory breast cancer?

A
  • Cancer cells obstructing lymphatic draining to breast leading to peau d’orange presentation
452
Q

What is fibronectin responsible for?

A

Cell adhesion

453
Q

When is FFP administered?

A

Warfarin OD

454
Q

Treatment of warfarin OD?

A

FFP

- K also works but takes days

455
Q

Treatment of heparin OD?

A

Protamine sulfate

*****FFP does not work as it contains ATIII which will potentiate warfarin

456
Q

What do POL gene mutations convey?

A

In HIV, this conveys proteases that are resistant “paul the black smith”
- Also can render reverse transcriptase resistant

USMLE: step 1 (15 decks)

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