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STEP > Hematology, oncology > Flashcards

Hematology, oncology Flashcards

1
Q

Treatment for refractory ITP?

A

splenectomy

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2
Q

What binds GPIIB/IIIA?

A

*Fibrinogen

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3
Q

Weibel-palade

A

Granules that contain VW factor and P-selectin in endothelial cells.

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4
Q

erythrocyte structure

A

enucleate + lacks organelles

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5
Q

RBC life span

A

120 days

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6
Q

anisocytosis

A

RBCs of varying sizes

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7
Q

poikilocytosis

A

RBCs of varying shapes

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8
Q

What does bluish color on Wright-Giemsa stain of reticulocytes represent?

A

Residual ribosomal RNA

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9
Q

thrombocyte life span

A

8-10 days

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10
Q

What do platelets interact with to form platelet plugs?

A

fibrinogen

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11
Q

What are alpha granules?

A

Granules within platelets that contain vWF + fibrinogen + fibronectin.

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12
Q

What are dense granules?

A

Granules within platelets that contain ADP + calcium.

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13
Q

storage site for platelets?

A

1/3 of platelet pool is stored in the spleen.

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14
Q

Etiology of petechiae

A

thrombocytopenia OR decreased platelet function

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15
Q

What is the receptor for vWF?

A

GpIb

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16
Q

What is the fibrinogen receptor?

A

GpIIb/IIa

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17
Q

granulocytes?

A

1) neutrophils
2) eosinophils
3) basophils

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18
Q

Mononuclear cells?

A

1) monocytes

2) lymphocytes

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19
Q

normal neutrophil range

A

54-62%

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20
Q

normal lymphocyte range

A

25-33%

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21
Q

normal monocyte range

A

3-7%

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22
Q

normal eosinophil range

A

1-3%

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23
Q

normal basophil range

A

0-0.75%

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24
Q

What are contained in neutrophil granules?

A

1) leukocyte alkaline phosphatase (LAP)
2) collagenase
3) lysozyme
4) lactoferrin

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25
Q

What are azurophilic granules?

A

Lysosomes in neutrophils that contain proteinases, acid phosphatase, myeloperoxidase, and beta-glucuronidase.

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26
Q

Why do hypersegmented neutrophils indicate?

A

B12/folate deficiency

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27
Q

What do increased bands indicate?

A

States of myeloid proliferation (bacterial infections, CML)

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28
Q

Neutrophil chemotactic agents

A

1) C5a
2) IL-8
3) LTB4
4) kvllikrein
5) platelet-activating factor

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29
Q

What is a monocyte?

A

precursor to macrophage. Monocytes exist in the blood, and when they reach tissue, differentiate into macrophages.

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30
Q

Monocyte description

A

1) Large, kidney-shaped nucleus.

2) extensive “frosted glass” cytoplasm.

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31
Q

describe macrophage involvement in septic shock pathogenesis

A

Lipid A from bacterial LPS binds CD14 on macrophages to initiate septic shock.

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32
Q

eosinophil functions

A

1) defense against helminths

2) highly phagocytic for antigen-antibody complexes

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33
Q

eosinophil description

A

1) bilobate nucleus

2) large eosinophilic granules of uniform size

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34
Q

What do eosinophils produce?

A

1) MBP

2) *histaminase (inactivates histamine)

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35
Q

Causes of eosinophilia

A 
NAACP
Neoplasia
Asthma
Allergic processes
Chronic adrenal insufficiency
Parasites (invasive)
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36
Q

Basophil functions

A

1) Mediate allergic reactions

2) Synthesize and release leukotrienes on demand.

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37
Q

What do basophilic granules contain?

A

1) heparin

2) histamine

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38
Q

Basophilia think…

A

CML

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39
Q

Mast cell function?

A

Mediate allergic reactions in local tissues (type 1)

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40
Q

mast cell in allergic reactions mechanism

A

Bind the Fc portion of IgE to membrane. IgE cross-links upon antigen binding leading to degranulation and release of histamine, heparin, tryptase, and eosinophil chemotactic factors.

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41
Q

Cromolyn sodium clinical use + mechanism

A

1) asthma prophylaxis

2) prevents mast cell degranulation

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42
Q

What is the link between the innate and adaptive immune systems?

A

Dendritic cell

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43
Q

What do dendritic cells express on their surface?

A

MHC class II + Fc

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44
Q

Lymphocyte?

A

B cell, T cell, or NK cell

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45
Q

Lymphocyte description

A

Round, densely staining nucleus with small amount of pale cytoplasm.

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46
Q

What is the costimulatory signal necessary for T-cell activation?

A

CD28

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47
Q

Plasma cell characteristics

A

1) “clock-face” chromatin distribution and eccentric nucleus
2) abundant RER
3) well-developed Golgi apparatus

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48
Q

Where are plasma cells usually found?

A

Bone marrow. Normally do not circulate in peripheral blood.

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49
Q

Fetal erythropoiesis timeframe

A

Young Liver Synthesizes Blood

1) Yolk sac (3-8 weeks)
2) Liver (6 weeks–birth)
3) Spleen (10-28 weeks)
4) Bone marrow (18 weeks to adult)

So in neonatal period, there’s RBC production in the liver + spleen + bone marrow.

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50
Q

What are the embryonic globins

A

zeta and epsilon

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51
Q

fetal hemoglobin structure

A

2 alphas, 2 gammas

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52
Q

Clinical relevance of AB blood type

A

1) Universal recipient of RBCs

2) universal donor of plasma (no antibodies produced)

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53
Q

What immunoglobulin type is anti-B or anti-A?

A

IgM

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54
Q

Clinical relevance of O blood type

A

1) If they receive any non-O blood they’ll get a hemolytic reaction.
2) Universal donor of RBCs (no antigens expressed on surface)
3) **universal recipient of plasma

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55
Q

Clinical relevance of Rh positive blood?

A

Universal recipient of RBCs

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56
Q

What immunoglobulin isotope is against Rh proteins?

A

IgG

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57
Q

Rhogam mechanism

A

Anti-D Ig immunoglobulins, which attack and hemolyze fetal RBCs in the maternal blood stream in order to prevent her from developing an immune response to Rh proteins and attacking the fetus.

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58
Q

How do you prevent anti-D IgG production?

A

Give RhoGAM in third trimester.

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59
Q

more common hemolytic disease of the newborn?

A

ABO hemolytic disease

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60
Q

Usual scenario of ABO hemolytic disease?

A

Type O mother with a type A,B, or AB fetus.

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61
Q

Difference between Rh hemolytic disease and ABO hemolytic disease?

A

1) ABO can occur in a first pregnancy because maternal anti-A or anti-B are formed early in life.
2) ABO doesn’t worsen with future pregnancies.

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62
Q

ABO hemolytic disease presentation

A

Mild jaundice in the neonate within 24 hours of birth.

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63
Q

ABO hemolytic disease treatment

A

Phototherapy or exchange transfusion.

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64
Q

missense mutation in HbS?

A

glutamic acid –> valine

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65
Q

missense mutation in HbC?

A

glutamic acid –> lysine

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66
Q

hemoglobin electrophoresis mnemonic?

A

A Fat Santa Claus

A migrates the farthest, followed by fetal hemoglobin, HbS, and HbC. See FA 383

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67
Q

bradykinin affects

A

1) vasodilation
2) increased permeability
3) increased pain

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68
Q

direct thrombin inhibitors

A

argatrobran
bivalirudin
dabigatran

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69
Q

thrombolytics

A

alteplase
reteplase
*streptokinase
tenecteplase

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70
Q

kallikrein mechanism

A

HMWK –> bradykinin

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71
Q

Tissue factor function

A

VII –> VIIa

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72
Q

What steps in the coagulation cascade require Ca, phospholipid?

A

1) VII –> VIIa
2) VIIa –> X
3) VIIa
4) Va

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73
Q

Epoxide reductase?

A

Enzyme that reduces vitamin K, allowing it to act as a cofactor

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74
Q

Gamma-glutamyl transferase function?

A

Uses vitamin K to activate vitamin-k dependent components of coagulation cascade.

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75
Q

Vitamin K dependent components?

A

II,VII,IX,X,C and S

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76
Q

Warfarin mechanism

A

Inhibits epoxide reductase

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77
Q

vWF function?

A

Carries and protects factor VIII

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78
Q

Protein C and S mechanism

A

Thrombin-thrombomodulin complex on endothelial cells activates protein C. Activated protein C and protein S cleave and inactivate Va, VIIIa.

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79
Q

tPA mechanism

A

Activates plasminogen to plasmin

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80
Q

Plasmin function?

A

fibrinolysis:

  1. cleavage of fibrin mesh
  2. destruction of coagulation factors
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81
Q

Antithrombin mechanism

A

Inhibits activated forms of factors II, VII, IX, X, XI, and XII.

82
Q

heparin mechanism

A

Enhances activity of antithrombin.

83
Q

Principal targets of antithrombin

A

Thrombin + factor Xa

84
Q

Factor V leiden mechanism

A

Produces a factor V resistant to inhibition by activated protein C

85
Q

Weibel-palade bodies?

A

Granules in endothelial cells that contain vWF

86
Q

Primary hemostasis

A

platelet plug formation

87
Q

platelet plug formation mechanism

A

1) injury
2) exposure
3) adhesion
4) activation
5) aggregation

88
Q

describe injury step of primary hemostasis

A

Endothelial damage –> transient vasoconstriction via neural stimulation reflex and endothelin (released from damaged cell)

89
Q

describe exposure step of primary hemostasis

A

vWF binds to exposed collagen

90
Q

describe adhesion step of primary hemostasis

A

Platelets bind vWF via GpIb receptor at the site of injury –> platelets undergo conformation change –> platelets release ADP and Ca2+ (necessary for coagulation cascade) and thromboxane –> ADP helps platelets adhere to endothelium.

91
Q

ADP function in primary hemostasis?

A

1) Helps platelets adhere to endothelium.

2) binding to receptor induces GpIIb/IIIa expression at platelet surface

92
Q

describe activation step of primary hemostasis

A

ADP binding to receptor induces GpIIb/IIIa expression at platelet surface.

93
Q

describe aggregation step of primary hemostasis

A

Fibrinogen binds GpIIb/IIIa receptors and links platelets. Balance between pro-aggregation and anti-aggregation factors. Temporary plug stops bleeding but it’s unstable and easily dislodged. Secondary hemostasis occurs afterward with the coagulation cascade.

94
Q

Anti platelet aggregation factors?

A

1) PGI2 and NO (released by endothelial cells)

2) Increased blood flow

95
Q

pro aggregation factors

A

1) TXA2
2) *decreased blood flow
3) increased platelet aggregation

96
Q

source of thromboxane?

A

Platelets

97
Q

what is thrombogenesis?

A

Formation of insoluble fibrin mesh.

98
Q

GpIIB/IIIa inhibitors?

A

Abciximab
Eptifibatide
Tirofiban

99
Q

Ristocetin mechanism?

A

Activates vWF to bind GpIb.

100
Q

Failure of agglutination assay with ristocetin indicates..

A

1) vWF disease
OR
2) Bernard-Soulier syndrome

101
Q

Thromboplastin

A

Plasma protein that aids in blood coagulation through catalyzing conversion of prothrombin to thrombin.

102
Q

What are acanthocytes associated with?

A

1) liver disease

2) abetaliproteinemia (states of cholesterol dysregulation)

103
Q

Abetalipoproteinemia

A

♣ Code: baby looking like kevo on the ground with a distended abdomen + shitting really nasty-smelling poop/manifests during first year of life with symptoms of malabsorption (abdominal distention + foul-smelling stool). /inherited inability to synthesize apolipoprotein B, an important component of chylomicrons and VLDLs. Fern like structures sticking out of the walls everywhere clear fatty deposits in them/lipids absorbed by the small intestine cannot be transported into the blood and accumulate in the intestinal epithelium, resulting in enterocytes with clear or foamy cytoplasm. Tyrion in the bunk bed + /autosomal recessive loss-of-function mutation in the MTP gene. Baby is swing a medieval ball and chain/acanthocytes. Bunk to right is a yellow taxi + eye on the window looking like below/other presentation = progressive ataxia + retinitis pigmentosa (progressive degeneration of rod photoreceptors.
♣ Location: Bunkroom

104
Q

What is associated with basophilic stippling?

A

1) lead poisoning
2) sideroblastic anemias
3) myelodysplastic syndromes

105
Q

dacrocyte?

A

teardrop cell

106
Q

degmacye?

A

bite cell

107
Q

Burr cell?

A

echinocyte

108
Q

Where are echinocytes found?

A

1) ESRD
2) liver disease
3) pyruvate kinase deficiency

109
Q

Differentiating echinocytes from acanthocytes?

A

Echinocyte projections are more uniform and smaller.

110
Q

Hereditary elliptocytosis

1) blood smear
2) presentation
3) genetics

A

1) elliptocytes
2) usually asymptomatic
3) mutation in genes encoding RBC membrane proteins (eg spectrin)

111
Q

Macro-ovalocytes seen in..

A

1) megaloblastic anemia

2) marrow failure

112
Q

Schistocytes seen in…

A

1) DIC
2) TTP/HUS
3) HELLP syndrome
4) mechanical hemolysis (heart valve prosthesis)

113
Q

helmet cell

A

type of schistocyte

114
Q

When does sickling occur?

A

1) dehydration
2) deoxygenation
3) high altitude

115
Q

When are spherocytes seen?

A

1) hereditary spherocytosis

2) drug and infection-induced hemolytic anemia.

116
Q

When are target cells seen?

A 
HALT said the hunter to his target.
HbC disease
Asplenia
Liver disease
Thalassemia
117
Q

Etiology of Heinz bodies

A

Oxidation of Hb -SH groups to -S–S- –> Hb precipitation

118
Q

When are Howell-Jolly bodies seen?

A

1) functional hyposplenia

2) asplenia

119
Q

What are Howell-Jolly bodies?

A

Basophilic nuclear remnants. Remnants of the nucleus. They are normally removed from RBCs by splenic macrophages.

120
Q

Other anemia that can by microcytic…

A

Late stage of ACD

121
Q

Distinguishing an H pylori duodenal ulcer from ZES ulcer?

A

H pylori is in first part of duodenum. ZES ulcers generally more distal.

122
Q

approach to anemia problems?

A

1) look at MCV

2) look at reticulocyte count

123
Q

What does reticulocyte count tell you?

A

If it’s normal or decreased it’s a non hemolytic anemia. If increased, it’s a hemolytic anemia.

124
Q

Normocytic anemias with decreased reticulocyte count…

A

1) *early IDA
2) early ACD
3) aplastic anemia
4) CKD

125
Q

anemia related to copper deficiency…

A

Microcytic sideroblastic anemia

126
Q

Megaloblastic macrocytic anemias

A

1) folate deficiency
2) B12 deficiency
3) *orotic aciduria

127
Q

non-megaloblastic macrocytic anemia

A

1) liver disease
2) alcoholism
3) Diamond-Blackfan anemia

128
Q

Approach to macrocytic anemias…

A

Determine if megaloblastic or non-megaloblastic

129
Q

How to determine MCV on blood smear

A

Lymphocyte nucleus is roughly the same size as a normocytic RBC. If RBC is larger than lymphocyte nucleus, consider macrocytosis. If smaller –> microcytosis.

130
Q

When do you get iron deficient

A

1) chronic bleeding
2) malnutrition
3) absorption disorders
4) pregnancy (increases demand in final step in heme synthesis)

131
Q

labs in IDA

A

Decreased iron
Increased TIBC
Decreased ferritin

132
Q

When do RBCs look like in IDA?

A

small and area of central pallor (hypochromasia)

133
Q

What does pica indicate?

A

Iron deficiency anemia

134
Q

krukenberg related to..

A

diffuse GI cancer

135
Q

Sister mary joseph related to..

A

intestinal GI cancer

136
Q

spoon nails medical term..

A

koilonychia

137
Q

other RF for intestinal GI cancer

A

type A blood

138
Q

What is Hb Barts?

A

Excess gamma-globing formation (all subunits gamma) from 4 allele deletion.

139
Q

What is HbH disease?

A

3 alelle deletion: inheritance of chromosome with cis deletion + a chromosome with 1 allele deletion.

140
Q

Components of HbH

A

Very little alpha-globin. Excess Beta-lgobin forms (4 betas)

141
Q

What happens with 2 alpha alleles deleted?

A

Less clinically severe anemia.

142
Q

What happens with 1 alpha allele deleted?

A

No anemia (clinically silent)

143
Q

Genetics of beta-thalassemia?

A

Point mutations in splice sites and promoter sequences.

144
Q

beta-thalassemia minor presentation?

A

Usually asymptomatic.

145
Q

B-thalassemia diagnosis?

A

HbA2 greater than 3.5 on electrophoresis.

146
Q

B-thalassemia major presentation

A

severe anemia. These people need blood transfusion.

147
Q

Major cause of secondary hemochromatosis?

A

Beta-thalassemia major

148
Q

Beta-thalassemia features

A

1) “crew cut” on skull x-ray
2) skeletal deformities
3) “chipmunk” facies
4) hepatosplenomegaly (due to extramedullary hematopoiesis

149
Q

What are beta thalassemia major patients at increased risk for?

A

parvovirus B19-induced aplastic crisis.

150
Q

Hemoglobin in beta-thalassemia major components?

A

Increased fetal hemoglobin. (a2gamma2)

151
Q

Presentation of HbS/beta-thalassemia heterozygote?

A

Mild to moderate sickle cell disease depending on amount of beta-globin production.

152
Q

Lead poisoning etiology and effect

A

lead inhibits ferrochelatase and ALA dehydratase, leading to decreased heme synthesis and increased RBC protoporphyrin.

153
Q

What else does lead inhibit?

A

rRNA degradation. This is why you get rRNA aggregates (basophilic stippling)

154
Q

Lead poisoning Presentation

A

LEAD
Lead lines on gingival and on metaphases of logan bones.
Encephalopathy and Erythrocyte basophilic stippling.
Abdominal colic and sideroblastic Anemia
Drops– wrist and foot drop

155
Q

sideroblastic anemia inheritance

A

X-linked defect

156
Q

sideroblastic anemia etiology

A

defect in delta-ALA synthase gene

157
Q

Causes of sideroblastic anemia…

A

1) genetic
2) myelodysplastic syndromes
3) alcohol
4) lead
5) B6 deficiency
6) copper deficiency
7) isoniazid

158
Q

treatment for sideroblastic anemia

A

pyridoxine (B6, which is a cofactor for delta-ALA synthase)

159
Q

Labs in sideroblastic anemia

A

1) Increased iron
2) normal to decreased TIBC
3) increased ferritin

160
Q

blood smear finding in sideroblastic anemia

A

basophilic stippling of RBCs

161
Q

what causes megaloblastic anemia?

A

Impaired DNA synthesis, so nucleus maturation is delayed relative to cytoplasm.

162
Q

physical exam finding in megaloblastic anemia…

A

Glossitis (inflammation, soreness of the tongue)

163
Q

What else can cause folate deficiency?

A

1) hemolytic anemia

2) pregnancy

164
Q

How do you differentiate folate from b12 deficiency.

A

*no neurologic symptoms with folate deficiency.

165
Q

labs in B12 deficiency

A

Increased homocysteine + increased methylmalonic acid

166
Q

Why do you subacute combined degeneration in b12 deficiency?

A

B12 is involved in fatty acid pathways and myelin synthesis.

167
Q

What is subacute combined degeneration?

A

spinocerebellar tract + lateral corticospinal tract + dorsal column dysfunction.

168
Q

Orotic acuduria etiology

A

Inability to convert orotic acid to UMP (de novo pyrimidine synthesis pathway) because of defect in UMP synthase.

169
Q

orotic acuduria inheritance

A

autosomal recessive.

170
Q

Megaloblastic anemia refractory to folate and B12 think…

A

orotic aciduria

171
Q

treatment for orotic acuduria?

A

uridine monophosphate to bypass mutated enzyme.

172
Q

How do you differentiate orotic aciduria from ornithine transcarbamylase deficiency?

A

No hyperammonemia in orotic aciduria.

173
Q

Physical exam finding in orotic aciduria?

A

Orotic acid in urine

174
Q

Diamond-Blackfan anemia

A

Rapid-onset anemia within 1st year of life due to intrinsic defect in erythroid progenitor cells.

175
Q

Diamond-Blackfan anemia

A

Increased HbF but decreased total Hb.

176
Q

Diamond-Blackfan anemia presentation?

A

Short stature + craniofacial abnormalities + upper extremity malformations (triphalangeal thumbs).

177
Q

Nonmegaloblastic anemia

A

Microcytic anemia in which DNA synthesis is unimpaired.

178
Q

Nonmegaloblastic anemia on smear

A

RBC macrocytosis without hypersegmented neutrophils

179
Q

If there are no hypersegmented neutrophils and macrocytic?

A

1) alcoholism

2) liver disease

180
Q

Where would you block the brachial plexus?

A

Between anterior and middle scalenes.

181
Q

Findings in intravascular hemolysis

A

1) decreased haptoglobin
2) Increased LDH
3) schistocytes and increased reticulocytes on blood smear

182
Q

urine findings with intravascular hemolysis

A

1) hemoglobinuria
2) hemosiderinuria
3) urobilinogen
4) possibly increased unconjugated bilirubin

183
Q

What is extravascular hemolysis?

A

Macrophages in spleen clearing RBCs

184
Q

Findings in extravascular hemolysis

A

1) spherocytes in peripheral smear
2) Increased LDH.
3) ***no hemoglobinuria/hemosiderinuria
4) increased unconjugated bilirubin.

185
Q

urine findings with extravascular hemolysis

A

1) urobilinogen

186
Q

ACD associations

A

1) RA
2) SLE
3) neoplasia
4) CKD

187
Q

ACD findings

A

1) decreased iron
2) decreased TIBC
3) increased ferritin

188
Q

treatment for ACD related to CKD?

A

EPO

189
Q

ACD classification

A

Normocytic, but can become microcytic

190
Q

Underlying etiology of aplastic anemia

A

destruction of myeloid stem cells

191
Q

Drugs that can cause aplastic anemia?

A

1) benzene
2) chloramphenicol
3) alkylating agents
4) antimetabolites

192
Q

viruses that can cause aplastic anemia?

A

1) parvovirus B19
2) EBV
3) HIV
4) hepatitis

193
Q

falcon anemia

A

short stature + increased incidence of tumors/leukemia + cafe-au-lait spots + thumb/radial defects.

194
Q

Other common cause of aplastic anemia

A

Following acute hepatitis

195
Q

Labs in aplastic anemia?

A

1) decreased reticulocyte count

2) increased EPO

196
Q

dry bone marrow tap…

A

aplastic anemia

197
Q

symptoms of aplastic anemia..

A

Fatigue + malaise + pallor + purpura + mucosal bleeding + petechiae + infection.

198
Q

Aplastic anemia treatment

A

1) stop the drug.
2) immunosuppression with antitymocyte globulin Or cyclosporine
3) bone marrow allograft
4) RBC/platelet transfusion
5) bone marrow stimulation (eg, GM-CSF)

199
Q

defective proteins in hereditary spherocytosis

A

1) nnkyrin
2) band 3
3) protein 4.2
4) spectrin

200
Q

hemolysis type in hereditary spherocytosis?

A

extravascular. Spleen is removing RBCs

201
Q

What accumulates with lead poisoning?

A

protoporphyrin

STEP (85 decks)

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