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Step 1 2019 First Aid Flashcards > Heme - FA Anat/Phys/Patho p.396-421 > Flashcards

Heme - FA Anat/Phys/Patho p.396-421 Flashcards

1
Q

Life span of RBC?

A

120 days

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2
Q

Life span of platelet?

A

8-10 days

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3
Q

Normally leukocyte count range?

A

4000–10,000 cells/mm3.

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4
Q

Membrane of RBC contains — antiporter,

A

Cl-/HCO3

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5
Q

Name the vWF receptor?

A

GpIb

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6
Q

Erythrocytosis = — = —- hematocrit

A

polycythemia incr

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7
Q

Dense granules in Platelets contain —- and α granules contain —.

A

ADP, Ca2+

vWF, fibrinogen, fibronectin, platelet factor 4

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8
Q

Normal Basophils %?

A

(0–0.75%)

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9
Q

Poikilocytosis =

A

varying shapes

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10
Q

Name Fibrinogen receptor?

A

GpIIb/IIIa.

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11
Q

Neutrophyls specific granules contain 4 components: “CALL”

A

ALP collagenase lysozyme lactoferrin (traps iron)

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12
Q

What are Azurophilic granules?

A

lysosomes

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13
Q

Anisocytosis =

A

varying sizes (seen in thalasemia, spherocytosis)

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14
Q

Azurophilic granules contain what 4 components?

A
  • proteinases
  • acid phosphatase
  • myeloperoxidase
  • β-glucuronidase (in liver)
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15
Q

Important neutrophil chemotactic agents:

A

C5a, IL-8, LTB4, kallikrein, platelet-activating factor

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16
Q

How does gram neg bacteria initiate septic shock?

A

Lipid A from bacterial LPS binds CD14 on macrophages

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17
Q

Incr in myeloid proliferation is observed by the incr of what cells during infection or CML?

A

band cells (immature neutrophils)

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18
Q

Macrophages are activated by

A

γ-interferon.

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19
Q

Which 2 cells are packed with basophylic granules?

A

Basophyls Mast cell

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20
Q

Monocytes differentiates into —- in tissues.

A

macrophage

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21
Q

Macrophages are antigen-presenting cell via —.

A

MHC II.

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22
Q

Extensive “frosted glass” cytoplasm referrers to what type of cells?

A

monocyte

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23
Q

Basophilia is uncommon, but can be a sign of what path?

A

myeloproliferative disease, particularly CML.

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24
Q

Which cells expresses MHC class II and Fc receptors on surface?

A

Dendritic cell, macrophage

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25
Q

Which kind of Leukocyte produces histaminase and major basic protein?

A

eosinophil

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26
Q

Round, densely staining nucleus with small amount of pale cytoplasm describes what type of cells?

A

lymphocyte

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27
Q

Name the 5 causes of eosinophilia

A
  • Acute interstitial nephritis (eosinphilia and eosinophiluria) - Diuretics, NSAIDs, Penicillins/cephalosporins, PPI, Rifampin
  • Neoplasia - ex/ hodgkins
  • Asthma
  • Aspergillus
  • Allergic processes
  • Chronic adrenal insufficiency,
  • Churg Strauss
  • Parasites (invasive)
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28
Q

— is a costimulatory signal necessary for T-cell activation.

A

CD28

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29
Q

Which type of cells have abundant RER, and well developed Golgi apparatus?

A

plasma cells

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30
Q

Which Leukocyte contains Heparin?

A

basophil, mast cells

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31
Q

“Clock-face” chromatin distribution refers to what type of cells?

A

plasma cells

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32
Q

mothers exposed to fetal Rh+ blood (often during delivery) may make what type of Ab? How are they diff from anti-A/B Ab?

A

anti-D IgG. - can cross placenta

Anti-A & Anti-B Ab are IgM and can’t cross placenta

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33
Q

—- administration to Rh− pregnant women during —–, which prevents maternal anti-Rh IgG production.

A

RhoGAM third trimester

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34
Q

Hemophilia C has a deficiency of what factor? And what is the genetic inheritance?

A

XI (AR)

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35
Q

Name 3 LMWHs?

A

dalteparin, enoxaparin, Fondaparinux

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36
Q

—- activates bradykinin; — inactivates bradykinin

A

Kallikrein (HMWK) activates bradykinin;

ACE inactivates bradykinin

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37
Q

The LMWH have the greatest e€efficacy on which factor?

A

Factor Xa

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38
Q

Which 3 tissue factors require Ca2+ and phospholipid?

A

Thromboplastin (III) (produced from fetus during abortion) Va VIIIa

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39
Q

Which 4 tissue factors are inhibited by vitamin K antagonist warfarin?

A

II VII IX X

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40
Q

Heparin has the greatest e€efficacy on which factor?

A

IIa (thrombin)

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41
Q

Which 2 coagulation factors are cofactors?

A

Va VIIIa

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42
Q

T or F? Plasmin activates but is not part of coagulation cascade.

A

T

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43
Q

CD marker of macrophages

A

CD14

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44
Q

Eosinophils are activated by ___ to defend vs helminthic infection?

A

(+) by IL5 made by TH2 and Mast cells

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45
Q

Marker for mast cells?

A

Tryptase

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46
Q

What Rx is used to (-) mast cell degranulation?

A

Cromolyn sodium - used for asthma prophylaxis

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47
Q

hypersegmented neutrophils are seen in what path?

A

hypersegmented (% or more lobes) - seen in B12/folate def

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48
Q

costimulatory molecule for dendritic cell?

A

B7

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49
Q

Name 3 direct thrombin inhibitors? (DOC for HIT)

A

argatroban bivalirudin dabigatran

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50
Q

Name 2 direct Xa inhibitors?

A

apixaban, rivaroxaban

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51
Q

Fondaparinux inhibits which factor? (it causes HIT)

A

factor Xa

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52
Q

Thrombin converts what to what?

A

Fibrinogen to Fibrin monomers

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53
Q

phytomenadione or phylloquinone or phytonadione help mature which factors?

A

Vit K helps the maturation of clotting factors II, VII, IX, X, and proteins C and S.

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54
Q

Prolonged use of broad-spectrum antibiotics can cause what?

A

hemorrhagic disease due to vit K def ( enteric bacteria produce vit K)

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55
Q

Which complex activates protein C?

A

Thrombin-thrombomodulin complex

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56
Q

—- stabilizes platelet plug.

A

Fibrin mesh

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57
Q

Name the enzyme that produces reduced vit K?

A

epoxide reductase - inhibited by Warfarin

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58
Q

—– cleaves and inactivates Va, VIIIa

A

Protein C, with the aid of Protein S

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59
Q

Describe the Factor V Leiden mutation?

A

it produces a factor V resistant to inhibition by activated protein C.

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60
Q

Plasminogen —> plasmin

A

tPA

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61
Q

Basophils is incr in what path?

A

Chronic myelogenous leukemia (CML) polycythemia

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62
Q

Eosonophils are INC in what infections?

A

Aspergillus infection parasites

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63
Q

Which T cells recruit eosonophils?

A

Th2 cells

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64
Q

Eosinophylic infiltrate is see in what cardiac path?

A

Löffler syndrome (endomyocardial fibrosis with a prominent eosinophilic infiltrate), Eosinophilic granulomatosis with polyangiitis (Churg- Strauss)

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65
Q

Eosinophila in what 2 drugs?

A

Macrolides, NSAIDs

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66
Q

What vitamin in excess can alter the metabolism of vitamin K?

A

vit E

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67
Q

what blocks tPA?

A

Tranexamic acid (analog of lysine), Aminocaproic acid

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68
Q

VWF is stored in which cells?

A

1) Weibel Palade bodies in Endothelial cells - also P-selectin 2) Alpha granules in Platelets

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69
Q

What type of mutation in HbS and HbC?

A

Missense mutations in HbS - glutamic acid (neg aa) –> valine (neutral) HbC - glutamic acid –> lysine (postive)

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70
Q

Stimulate tPA?

A

Alteplase, Reteplase, streptokinase, tenecteplase

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71
Q

Inhibitor of plasmin?

A

alpha 2 anti-plasmin

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72
Q

How would you differentiate b/w Von Willebrand disease and Bernard Soulier syndrome after a positive ristocetin test?

A

BS syndrome: inc BT, PT and PTT (N) VW Disease: Inc BT, PT (N), Inc PTT, (vWF stabilizes Factor VIII therefore deficiency increase PTT)

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73
Q

Deficiency of Gp IIb/IIIa?

A

Glanzmann Thrombasthenia

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74
Q

Name the Pathology associated with peripheral blood smear presentation: Degmacyte

A

G6PD deficiency (Degmacyte = Bite cell)

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75
Q

Name the Pathology associated with peripheral blood smear presentation: Basophilic stippling

A

Lead poisoning

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76
Q

MOA of Aspirin?

A

irrev inhibits COX, stopping TXA2 synthesis

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77
Q

Rx that (-) ADP induced expression of GpIIb/IIIa via P2Y12-R

A

Clopidogrel, Prasugrel, Ticlopidine, Ticagrelor

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78
Q

Rx that inhibit GpIIb/IIIa directly (fibrinogen receptor)

A

Abciximab, Eptifibatide, Tirofiban, Lomifiban, Orbofiban

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79
Q

Risocetin - MOA?

A

activates vWF to bind GpIb

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80
Q

Name the Pathology associated with peripheral blood smear presentation: Dacrocyte

A

Myelofibrosis (Dacrocyte = Teardrop cell)

81
Q

Name the Pathology associated with peripheral blood smear presentation: Schistocyte

A

DIC, TTP, HUS, Mechanical hemolysis (Schistocyte =Helmet cell)

82
Q

Name the Pathology associated with peripheral blood smear presentation: Target cell

A

HALT HbC disease, Asplenia, Liver Disease, Thalassemia

83
Q

Name two heme pathologies that show an X linked inheritance pattern?

A

G6PD Deficiency, Sideroblastic Anemia, Hemophilia A

84
Q

Copper deficiency can lead to this heme pathology?

A

Microcytic Sideroblastic Anemia

85
Q

Clinical presentation of a pt with Iron Deficiency Anemia

A

Conjunctival Pallor, Spooned Nails (koilonychia)

86
Q

Describe the genetic defect in Alpha thalassemia? Which mutation is affiliated with which population?

A

Deletion in alpha globin gene (Ch 16). Cis deletion prevalent in Asians, Trans deletion prevalent in Africans

87
Q

Describe the genetic defect in Beta thalassemia? Which mutation is affiliated with which population?

A

Point mutation in the beta globin gene promoter and splice sites (Ch 11). Prevalent in Mediterranean populations

88
Q

What causes formation of Heinz bodies?

A

Oxidation of Hgb -SH groups to -S-S leads to Hgb precipitation = Heinz bodies

89
Q

Crew cut on skull XR, Chipmunk Facies, Extramedullary hematopoiesis

A

B thalassemia major

90
Q

Which virus can induce aplastic crisis (Clinical Presentation)? Name the heme pathologies associated with it.

A

Parvovirus B19 infection leads to slap cheek appearance in pts Patients with aplastic crisis: SCA, B Thalassemia

91
Q

What is basophilic stippling?

A

Remnants of rRNA in RBCs due decreased degradation

92
Q

Q. Clinical Presentation of Lead Poisoning?

A

Lead lines on gums (Burton lines) and long bone metaphyses Encephalopathy Abdominal colic Dropping of wrist and foot

93
Q

Name the enzymes inhibited by Lead

A

Ferrochelatase, ALA Dehydratase

94
Q

DOC for Lead poisoning in Adults?

A

Dimercaprol, EDTA

95
Q

DOC for Lead poisoning in Children?

A

Succimer

96
Q

What is the most common cause of Sideroblastic Anemia ? Is it Irreversible?

A

Alcoholism which is reversible if given B6 as Tx

97
Q

Which three drugs are associated with folate deficiency?

A

Methotrexate, Trimethoprim, Phenytoin

98
Q

Where do you see subacute combined degeneration of the spino cerebellar tract?

A

B12 def - along with lat corticospinal tract, dorsal column dysfunction

99
Q

How do you differentiate Orotic aciduria from Ornithine transcarbamylase def?

A

There is no hyperammonemia with Orotic aciduria

100
Q

Most common causes of macrocytic anemia that is not megaloblastic?

A

Alcoholism, liver disease,

101
Q

Defective enzyme in Orotic aciduria? Substrate?

A

Orotic Acid –> UMP, done by UMP synthase

102
Q

Short stature, thumb and radial defects?

A

Diamond Blackfan anemia and Fanconi anemia

103
Q

Two heme diseases with inc HbF?

A

B thalassemia major, Diamond blackfan anemia

104
Q

dec haptoglobin, inc LDH, target cells seen in ?

A

intravascular hemolysis

105
Q

Urine test shows Hgb, hemosiderin, and urobilinogen?

A

Intravacular hemolysis

106
Q

iron def anemia, esophageal webs, dysphagia

A

Plummer Vinson syndrome

107
Q

Which 3 GI issue can lead to iron def anemia?

A

GI bleed - colon cancer, etc

Malabsorption - gastritis, celiac disease,etc

108
Q

Genetic mutation in B thalassemia?

A

Point mutation in splice site and promoter sequences - C replaces Guanine before AUG seq – dec mRNA

109
Q

Type of genetic defect in sideroblastic anemia?

A

X linked defect in delta- ALA synthase - req B6 as cofactor

110
Q

Rx for Sideroblastic anemia

A

B6 (pyridoxine)

111
Q

Inc risk of gallstones and BM hyperplasia with what type of hematological disordeR?

A

hemolysis

112
Q

Iron, TIBC, ferritin - inc or dec in ACD (anemia of chronic disease)?

A

Dec FE, TIBC (indicator of transferrin) Inc Ferritin

113
Q

What differentiates Diamond Blackfan from Fanconi Anemia?

A

Fanconi has cafe au lait spots

114
Q

Is retic # inc or dec with aplastic anemia?

A

Dec

115
Q

Most common congenital cause of Aplastic anemia?

A

Fanconi anemia

116
Q

MCV and MCHC in heriditary spherocytosis ?

A

Inc MCHC (bc no central pallor) and normal to low MCV

117
Q

Dec ATP production is a part of the pathogenesis of what heme disorder?

A

Pyruvate kinase deficiency - dec ATP will lead to rigid RBCs –> extravasc hemolysis

118
Q

What amino acid mutation in HbC disease?

A

glutamic acid to lysine in B globin chain

119
Q

CD marker in Paroxysmal nocturnal hemoglobinuria?

A

CD 55/59

120
Q

Tx for Paroxysmal nocturnal hemoglobinuria?

A

Eculizumab - complement inhibitor

121
Q

Pathogenesis of PNH?

A

Impaired synthesis of GPI anchor for decay accelerating factor that protects RBC from complement

122
Q

Why are newborns normally asymptomatic in sickle cell?

A

They have higher hbF and lower HbS

123
Q

Why is there a crew cut skull in thalassemia major and Sickle cell?

A

Marrow expansion due to inc erythropoiesis

124
Q

What renal issue in Sickle cell and why?

A

Renal papillary necrosis - due to dec pO2 in papilla

125
Q

Why give hydroxyurea in Sickle cell?

A

Inc the amount of HbF

126
Q

Sickle cell osteomyelitis is assoc with which bact?

A

Salmonella paratyphi

127
Q

What Ig is seen in Warm AIHA and Cold AIHA?

A

IgG and IgM

128
Q

Which 3 pathologies are associated with cold agglutination AIHA?

A

Mycoplasma pneumoniae, Infectious Mono, CLL

129
Q

Drug assoc with Warm AIHA?

A

methyldopa (alpha)

130
Q

Microangiopathic hemolytic anemia seen in which diseases? (6)

A

DIC, TTP, HUS, SLE, Malig HTN, HELLP

131
Q

Two diseases associated wtih macroangiopathic anemia?

A

Prosthetic heart valves and aortic stenosis

132
Q

What is mixed with patients blood in Direct coombs test?

A

anti Ig antibody

133
Q

What in patients serum will yield a positive result in indirect coombs test?

A

If serum has anti RBC surface Ig, RBC will agglutinate = pos result

134
Q

What is the difference in Labs between ACD and Hemochromatosis?

A

Both have low TIBC and and high ferritin, but Hemochromatosis has high Fe levels and also high %transferrin sat’n therefore.

135
Q

Neutropenia cut off point?

A

<1500 cell/mm3

136
Q

Lymphopenia cut off point?

A

<1500 cell/mm3 (<3000 cells/mm3 in children)

137
Q

Which drug causes neutrophilia, inspite of eosinopenia and lymphopenia?

A

Corticosteroids (dec activation of neutrophil adhesion molecules, dec migration out, stay in blood - However, tend to keep eosinophils in l.n and cause lymphocyte apoptosis )

138
Q

Enzymes def in lead poisoning?

A

ALA DH, and Ferro chelatase - “Dont eat DAH Fucking Lead!”

139
Q

5 Ps of Acute intermittent porphyrias?

A

Painful stomach, Port wine colored urine, Polyneuropathy, Psych issues, Precipitated by drugs, etc

140
Q

Tx for AIP? (2) Why?

A

Glucose and Heme, they inhibit ALA synthase

141
Q

Enzyme def in AIP?

A

PBG deaminase

142
Q

Which HIV drugs are CI with Acute Intermittent Porphyria?

A

NNRTIs - Nevirapine, Efavirenz (p450 inducer)

143
Q

Def enzyme in Porphyria cutanea tarda?

A

UPG decarboxylase

144
Q

Which is the most common Porphyria?

A

PCTarda

145
Q

Which pathology is assoc with def in ALA synthase?

A

Sideroblastic anemia - S = synthase and sideroblastic

146
Q

Which porphyria is assoc with photosensitivity?

A

PCT

147
Q

What causes cell death due to peroxidation of membrane lipids?

A

Fe poisoning

148
Q

Symptoms of Fe poisoning?

A

n/v, gastric bleeding, lethargy, scarring leading to GI obstruction

149
Q

Drugs used for Fe poisoning?

A

Chelation - IV deferoxamine, oral deferasirox

150
Q

Which factors are tested in PT?

A

Factors I, II, V, VII, and X

151
Q

Which factors are not tested in PTT?

A

All factors except VII and XIII

152
Q

Inheritance patterns of all three hemophilias?

A

Hemophilia A and B - XLR, and C is AR

153
Q

Tx for Hemophilia A?

A

Desmopressin + factor VIII concentrate

154
Q

Vitamin K def will have an increase in what lab tests?

A

PT and PTT, Bleeding time is Normal

155
Q

What parts of the coag pathway are affected by Vit K def?

A

F II, VII, IX, X, and Prot C and S

156
Q

Which 3 hemat diseases have an issue with platelet plug formation?

A

Both Bernard Soulier, Glanzmann thrombasthenia, vW disease

157
Q

Which platelet disorder has normal platelet count?

A

Glanzmann , vW disease

158
Q

With which heme disorder will you potentially see enlarged platelets?

A

Bernard Soulier - release of relatively immature platelets to replace loss.

159
Q

Which heme disorder is associated with inc BUN ?

A

TTP, DIC, Hemolytic Uremic Syndrome, PNH, Sickle Cell, porphyria, Multple Myeloma

160
Q

What enzyme activates vit K?

A

vit K epoxide reductase

161
Q

Normal bleeding time?

A

2-7 min

162
Q

Normal PT time?

A

11-15 sec

163
Q

Normal PTT time?

A

25-40 sec

164
Q

Which two hematological disorders affect GpIIb/IIIa? How do they affect it?

A

Glanzmann - dec levels of GpIIb/IIIa

Immune Thrombocytopenia - anti GpIIb/IIIa antibodies

165
Q

Which hemat disorder show inc megakaryocytes on bone marrow biopsy?

A

ITP

166
Q

Which hemat disorder has a def of metalloprotease?

A

TTP - ADAMTS 13 = vWF metalloprotease

167
Q

Schistocytes are seen in which platelet disorder? why?

A

TTP - due to microthrombi formation (inc vWF multimers –> Inc platelet adhesion –> Inc platelet aggregation and thrombosis) DIC, HUS

168
Q

Which hemat disorders have neurological symptoms?

A

TTP - pentad of neuro/renal symptoms, fever, thrombocytopenia, fever, microangiopathic hemolyic anemia

B12 deficiency -

169
Q

What is the difference between HUS in adults and children?

A

Children tend to have diarrhea, adults do not.

170
Q

Most common cause of dec platelet #?

A

ITP

171
Q

Which blood tests are elevated in vW disease?

A

BT inc, and PTT may be normal or Inc

172
Q

Explain the Ristocetin test result of vW Disease?

A

No platelet aggregation, without vW.

173
Q

Which two hemat diseases are treated with desmopressin?

A

Hemophilia A, vW disease (affiliated with FVIII)

174
Q

Causes of DIC?

A

Sepsis, Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion, Snake Bite

175
Q

Lab values of DIC?

A

Inc fibrin degradation, dec fibrinogen, Dec factors V and VIII

176
Q

How does Antithrombin deficiency affect PT, PTT, and thrombin time? It can affect which of those values following an admin of which drug?

A

No direct effect on PT, PTT, or thrombin time. However, it can decrease the level of PTT increase following Heparin admin. (PTT is normally used to follow Heparin admin)

177
Q

Nephrotic syndrome is associated with which two coagulation disorders?

A

DIC and Antithrombin deficiency

178
Q

What type of mutation in FV Leiden?

A

guanine –> adenine DNA point mutation, Arg506Gln

179
Q

The mutation is FV Leiden def stops its degradation by what protein?

A

Prot C

180
Q

Most common cause of hypercoagulability in Caucasians?

A

F V Leiden

181
Q

What issue specific to women must one be concerned about in terms of FV Leiden def?

A

1) Inc risk of PE with OCP 2) Recurrent pregnancy loss

182
Q

Prot C and S def will lead inability to inhibit which factors?

A

Factor Va and VIIIa

183
Q

Be careful with prescribing which anti-coag when it comes to Prot C or S def?

A

Warfarin - inc risk of skin necrosis

184
Q

location of Gene mutation of Prothrombin?

A

Mutation in 3’ UTR

185
Q

Risk associated with blood transfusion?

A

transfusion reactions, Fe overload (could lead to 2ndary hemochromatosis) hypocalcemia, hyperkalemia

186
Q

Viruses associated with Hodgkins and Non Hodgkins lymphoma?

A

Hodkin - EBV

Non-Hodkin - HIV, EBV, HTLV

187
Q

Which type of lymphoma is assoc with B symptoms? What are those symptoms?

A

low grade fever, night sweats, weight loss - Both Hodgkin’s and non Hodgkin’s

188
Q

CD # of Reed-Sternberg cells?

A

CD15, CD30

189
Q

What are the two genes affected in Burkitts? (not the translocation itself, but the products of them)

A

Chr 8 - c -myc Chr 14 - Ig heavy chain

190
Q

Tingible body macrophages associated with?

A

Burkitts

191
Q

What type of lesion is seen in the sporadic form of Burkitts?

A

Pelvis and abdomen lesions

192
Q

Most common type of Non Hodgkin’s in adults?

A

Diffuse large B cell lymphoma

193
Q

Why can blood transfusions lead to tingling sensations and paresthesias?

A

hypocalcemia

194
Q

Gene products of the genes translocated in Mantle cell lymphoma?

A

Chr 11 - cyclin D1 (G1–> S) Chr 14 - Ig heavy chain

195
Q

What are the gene products of the gene translocation of Follicular lymphoma?

A

Chr 14 - Ig heavy chain Chr 18 - bcl2

196
Q

Which lymphoma presents with intermittent pain of lymph nodes?

A

Follicular lymphoma

197
Q

What are the cell types in grades 1-3 of Follicular lymphoma?

A

Grade 1 - small cleaved cells Grade 2 - Mixed Grade 3 - large cells

198
Q

Inc plasmin seen in which path?

A

Radical prostatectomy - release urokinase – (+) plasmin

Liver cirrhosis - dec prod’n of alpha antiplasmin looks like DIC w/o a postive D-dimer

Step 1 2019 First Aid Flashcards (63 decks)

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