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Pathoma > Heme > Flashcards

Heme Flashcards

1
Q

steps of hemostatsis

A
  1. rapid constriction
  2. platelet adhesion to surface of vessel (vWF binds collagen, platelets bind vWF vis Gp1b)
  3. platelet degranulation (ADP promotes exposure of GP2b/3a and TXA2 promotes aggregation)
  4. platelet aggregation (Gp2b/3a uses fibrinogen)
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2
Q

vasoconstriction mediated by…..

A

neural stimulation and endothelin released by endothelial cells

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3
Q

Gp1b

A

binds platelets to vWF

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4
Q

where does vWF come from

A

endothelial cells and platelets

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5
Q

WP bodies

A

contain vWF and p-selectin

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6
Q

mediators in platelets

A

ADP and TXA2

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7
Q

ADP leads to expression of….

A

Gp2b3a

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8
Q

Gp2b3a

A

for platelet aggregation, bind fibrinogen

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9
Q

TXA2

A

signal for platelet aggregation

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10
Q

primary hemostasis disorders

A

involves platelets

  • mucosal and skin bleeding
  • intracranial bleeding with severe thrombocytopenia
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11
Q

petechiae are signs of…

A

thrombocytopenia, not qualitative disorders

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12
Q

idiopathic thrombocytopenic purpura

A
  • auto IgG against platelet antigens (GPIIb/IIIa)
  • most common
  • antibodies produced in spleen and consumes platelets
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13
Q

ITP in children

A
  • after viral infection, self limited
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14
Q

ITP in adults

A
  • can be secondary (SLE)

- can cause thrombocytopenia in offspring (IgG across placenta)

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15
Q

labs in ITP

A
  • low platelets
  • normal PT/PTT
  • increase in megakaryocytes
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16
Q

treatment of ITP

A
  • steroids
  • IVIG (eats other sticks)
  • splenectomy
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17
Q

microangiopathic hemolytic anemia

A
  • platelet microthrombi in small vessels
  • consumption of platelets and destruction of RBC
  • schistocytes
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18
Q

microangiopathic hemolytic anemia seen in…

A

TTP and HUS

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19
Q

thrombotic thrombocytopenic purpura

A
  • due to decrease in ADAMSTS13 (usually autoantibody)

- increases platelet adhesion leading to microthrombi

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20
Q

ADAMSTS13

A

breaks up vWF multimers

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21
Q

hemolytic uremic syndrome

A
  • caused by ecoli O157
  • verotoxin damages endothelial cells
  • causes hemolytic anemia
  • in kids with undercooked beef
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22
Q

clinical findings in TTP and HUS

A
  • skin and mucosal bleeding (use up platelets)
  • hemolytic anemia
  • fever
  • renal insufficiency and CNS abnormalities
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23
Q

lab findings in TTP/HUS

A
  • thrombocytopenia
  • normal PT/PTT
  • anemia with schistocytes
  • increased megakaryocytes on bone marrow
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24
Q

bernard-soulier syndrome

A
  • genetic GP1b deficiency
  • platelet adhesion is impaired
  • mild thrombocytopenia with enlarged platelets
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25
Q

Glanzmann thrombasthenia

A
  • GIIb/GIIIa deficiency

- aggregation is impaired

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26
Q

aspirin….

A

irreversibly inactivates cyclooxygenase

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27
Q

uremia and platelets

A

blocks adhesion and aggregation

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28
Q

secondary hemostasis

A

coagulation cascade generates thrombin which converts fibrinogen to fibrin

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29
Q

activation of coag cascade requires

A
  1. exposure
  2. phospholipid surface
  3. calcium
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30
Q

clinical features of secondary hemostasis

A

deep tissue and rebleeding

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31
Q

what activates factor 12

A

subendothelial collagen

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32
Q

what activates factor 7

A

tissue thromboplastin

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33
Q

measure heparin with

A

PTT

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34
Q

measure coumadin with…

A

PT

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35
Q

hemophilia A

A
  • factor 8 deficiency
  • X-linked
  • deep tissue joint bleeding
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36
Q

labs in hemophilia A

A
  • high PTT, normal PT
  • low factor 8
  • normal platlets
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37
Q

hemophilia B

A
  • deficiency in factor 9

- resembles A

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38
Q

coagulation factor inhibitor

A
  • most common is factor 8
  • distinguished by adding patient serum (mixing study)
  • mixing study does not correct
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39
Q

Von Willebrand disease

A
  • most common
  • most common type is decreased factor
  • problem with platelet adhesion
  • mucosal and skin findings
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40
Q

labs in vWF disease

A
  • high bleeding time

- PTT is up (vWF stabilizes factor 8)

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41
Q

vFW stabilizes factor…..

A

8

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42
Q

ristocetin test

A

ristocetin causes platelet aggregation

- abnormal in von willebrand disease

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43
Q

treatment of von willbrand

A

desmopressin

- increase vWF from WP bodies

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44
Q

vitamin K deficiency

A
  • gamma carboxylation of 2,7,9,10 and C,S

- activated by epoxide reducates in liver

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45
Q

vitamin K generated by…

A

bacteria in gut

- problems in newborns, long term antibiotics, or malabsorption

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46
Q

liver failure hemostasis

A
  • lack of coag factors
  • no epoxide reductase of vitamin K
  • use PT to measure
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47
Q

heparin-induced thrombocytopenia

A
  • heparin forms complex with PF4, antibody forms
  • platelets are activated
  • increased in thrombosis
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48
Q

DIC

A
  • pathologic activation of coag cascade

- consume platelets and factors leading to bleeding

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49
Q

DIC caused by…

A

something else

  • OB complication (tissue thromboplastins)
  • sepsis from cytokines
  • mucin in adenocarcinoma
  • APML (auer rods)
  • rattlesnake bite
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50
Q

labs in DIC

A
  • low platelets
  • high PT/PTT
  • low fibrinogen
  • hemolytic anemia
  • elevated d-dimer
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51
Q

d-dimer test

A

for DIC - fibrin split products

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52
Q

fibrinolysis

A

removal of thrombus by plasmin

- tPA allows for plasmin activation

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53
Q

effects of plasmin

A
  1. cleaves fibrin
  2. cleaves plasma fibrinogen (stop future production)
  3. destroys coag factors
  4. blocks platelet aggregation
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54
Q

disorder of fibrinolysis

A

overactivity results in excessive cleavage of fibrinogen

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55
Q

examples of fibrinolysis

A
  1. radical prostatectomy - release or urokinase activates plasmin
  2. cirrhosis - antiplasmin not produced
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56
Q

presentation of fibrinolysis disorder

A
  • just like DIC
  • high PT/PTT
  • increased bleeding time
  • increased fibrinogen split products, no d-dimes
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57
Q

difference between DIC and fibrinolysis disorder

A 
DIC = low platelets because they are used up, d-dimer
fibrinolysis = normal platelets, no d-dimer (no clot)
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58
Q

treatment of fibrinolysis disorder

A

aminocaproic acid

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59
Q

characteristics of thrombus

A
  1. lines of Zahn (RBCs and fibrin)
  2. attachment to vessel
  • used to distinguish before and after death
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60
Q

3 risk factors of thrombosis

A
  1. disruption in blood flow
  2. hypercoagulability
  3. endothelial damage
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61
Q

prostaglandin I2

A

blocks platelet aggregation, produced to endothelium

62
Q

thrombomodulin

A
  • takes thrombin and allows it to activate protein C
63
Q

causes of endothelial damages

A
  • atherosclerosis
  • vasculitis
  • high serum homocysteine
64
Q

B12 deficiency and thrombus

A
  • leads to high homocysteine

- damages endothelium leading to thrombosis

65
Q

cystathionine beta synthase deficiency

A
  • results in high homocysteine levels

- characterized by thrombosis, mental retardation, lens dislocation and long fingers

66
Q

protein C and S

A

inactivate 5 and 8 factors

67
Q

protein C and S deficiency

A
  • decrease in negative feedback

- at risk for warfarin skin necrosis (C and S are destroyed first that can lead to clot)

68
Q

action of warfarin

A

blocks epoxide reductase

69
Q

factor V Leiden

A
  • factor 5 cant be cleaved by C and S

- hypercoaguability

70
Q

prothrombin 20210A

A
  • promotes hypercoaguable state
71
Q

ATIII deficiency

A
  • ATIII produced by endothelium
  • like heparin
  • inactivates coagulation factors
  • PTT does not rise with heparin
72
Q

mechanism for heparin

A

ATIII (doesn’t work in ATIII deficiency)

73
Q

cholesterol clefts

A

athersclortic plaque

74
Q

amniotic fluid embolus

A
  • during labor or delivery
  • SOB and neurologic
  • fluid loaded with tissue thromboplastin leading to DIC
  • squamous cells and keratin debris
75
Q

microcytosis occurs because of…

A

extra division to maintain hemoglobin concentration

76
Q

four causes of microcyctic anemia

A
  1. iron deficiency (no iron)
  2. chronic disease (trapped iron)
  3. sideroblastic anemia (no porphyrin)
  4. thalassemia (no globin)
77
Q

iron absorption

A

enterocyte use ferroportin (import) to move iron, transferrin (transports) iron, stored in cell (in) by ferritin

78
Q

iron labs

A
  • serum iron
  • transferrin (TIBC)
  • % sat
  • serum ferritin (stored in macrophages)
79
Q

main causes of blood loss

A

menorrhagia in women, peptic ulcer disease in males

80
Q

bleeding in elderly

A

colon/polyps and carcinoma and hook worm in developing world

81
Q

how iron helps get absorbed

A

acid environment, gastrectomy can make it worse

82
Q

stages of iron deficiency

A
  1. ferritin stores decrease
  2. serum iron is decreased
  3. normocytic anemia
  4. microcytic anemia
83
Q

relationship between ferritin and TIBC

A

inverse

84
Q

labs in iron anemia

A
  • microcytic, hypochromic anemia
  • high RDW
  • low ferritin, high TIBC
  • low serum iron, low saturation
85
Q

FEP

A

free erythrocyte protoporphyrin - iron is down so proto is not bound to iron

86
Q

blood smear in iron deficiency

A

microcytic-normal, central pallor

87
Q

Plummer Vinson syndrome

A

iron deficiency anemia, esophageal web, glossitis, red tongue

88
Q

chronic disease

A
  • increase in hepcidin (block it from bacteria)

- suppresses EPO

89
Q

lab findings in chronic disease

A
  • high ferritin, low TIBC
  • low serum iron, low saturation
  • high FEP
90
Q

sideroblastic anemia

A

defect in protoporphyrin synthesis

- leads to macrocytic anemia

91
Q

enzymes involved in proto synthesis

A

ALAS - requires B6, can be congenital, isoniazid
ALAD - lead poisoning
ferrochelatase - in mitochondria, lead poisoning

92
Q

ringed sideroblasts

A

iron trapped in the mitochondria

93
Q

isoniazid vitamin deficiency

A

B6

94
Q

labs in sideroblastic anemia

A

high ferritin, low TIBC

  • high serum iron, high saturation
  • high iron overload
95
Q

thalassemia is explained by…

A

protection from falciparum

96
Q

how many copies of alpha gene

A

4 - most important, in every globin

97
Q

2 alpha genes deleted

A
  • mild anemia, slightly increased RBC

- cis is worse for progeny (more common in Asia)

98
Q

3 alpha genes deleted

A
  • severe anemia

- beta tetramers form HbH

99
Q

HbH

A

beta tetramers

- seen on electrophoresis

100
Q

4 alpha genes deleted

A
  • lethal in utero, hydrops fetalis
101
Q

Hb Barts

A

gamma tetramers

- seen on electrophoresis

102
Q

beta thal is due to…..

A

gene mutations, can vary the amount of production

103
Q

beta minor

A
  • increase in HbA2
  • asymptomatic with increase in RBC
  • target cells
104
Q

target cells

A
  • seen in beta thal (decreased hemoglobin)

- either decreased in cytoplasm or increase in membrane

105
Q

beta major

A
  • severe form of disease
  • no problem in fetus
  • alpha tetramers lead to extravascular hemolysis
106
Q

massive erythroid hyperplasia

A
  • expansion of hematopoiesis in other places

- crew cut and chipmunk cheeks

107
Q

risk of aplastic crisis with…

A

parvovirus B19 in beta thal,

  • infects erythroid precursors
  • usually dependent on all red blood cell production
108
Q

HbA2

A

alpha 2 and delta 2

109
Q

what drives MCV to be big

A

not enough divisions

110
Q

characteristic of B12 or folate deficiency

A
  • megaloblastic anemia
  • hypersegmented neutrophils
  • other large cells (only seen in megaloblastic)
111
Q

other causes of macrocytic anemia

A

alcoholism, liver disease, drugs

112
Q

folate is absorbed in…

A

jejunum, can’t be stored

113
Q

lab findings in folate deficiency

A
  • macrocytic RBCs and hypersegmented neutrophils
  • glossitis
  • low folate
  • high serum homocysteine
  • normal MMA
114
Q

pernicious anemia

A
  • destruction of parietal cells

- loss of IF, can’t take up B12

115
Q

other causes of B12

A
  • pancreatitis (separates R binder from intrinsic factor for B12 binding)
  • damage to terminal ileum (Crohn’s or diphyllobothrium latum)
  • vegans
116
Q

labs of B12 deficiency

A
  • macrocytic anemia with hypersegmented neutrophils
  • glossitis
  • subacute combined degeneration (myelin stops due to high MMA)
  • low B12
  • high homocystein
  • high MMA
117
Q

reticulocyte count

A
  • young red blood cells with bluish cytoplasm

- should be increased in anemia

118
Q

corrected retic count

A

multiply by hematocrit/45

119
Q

extravascular hemolysis

A

involves reticuloendothelial system

120
Q

lab findings in extravascular hemolysis

A
  • anemia with splenomegaly
  • jaundice (too much UCBilli)
  • bilirubin gallstones
  • marrow hyperplasia (bone marrow is fine so it is working hard)
121
Q

intravascular hemolysis

A

hemoglobin directly in blood, binds to haptoglobin (decreases)

122
Q

labs in intravascular hemolysis

A
  • hemoglobinemia
  • hemoglobinuria
  • hemosiderinuria
  • low haptoglobin
  • basically hemoglobin is going directly into blood
123
Q

hereditary spherocytosis

A
  • due to ankyrin, spectrin defects
  • blebs of membrane are removed by spleen
  • leads to round cells
  • trapped in spleen
124
Q

labs in hereditary spherocytosis

A
  • anemia with loss of central pallor
  • high RDW
  • high MCHC
  • splenomegaly, jaundice (extravascular hemolysis)
  • gallstones
  • aplastic crisis in parvo B19 infection
125
Q

diagnosis of HE

A

osmotic fragility test

- in hypotonic solution, nor extra membrane for cell to expand, leads to bursting of cells

126
Q

HE after splenectomy

A
  • spherocytes persist

- Howell-Jolly bodies (no spleen)

127
Q

extravascular hemolysis

A

hereditary spherocytosis

sickle cell

128
Q

sickle cell disease

A
  • hemoglobin S (mutated B)

- polymerizes when deoxygenated (reversible)

129
Q

situations of sickling

A
  • dehydration
  • acidosis
  • deoxygenation
130
Q

treatment of sickle cell

A

hydroxyurea

131
Q

things seen in sickle cell

A
  • extra and intravascular hemolysis
  • target cells (dehydrated cells)
  • massive erythroid hyperplasia (crew cut, extramedullary hematopoiesis)
132
Q

irreversible sickling

A
  • leads to vaso-occlusion
  • dactylitis in children
  • autosplenectomy (increase in encapsulated organisms)
  • salmonella osteomyelitis
  • howell jolly bodies
133
Q

common causes of death in sickle cell

A

strep pneumo in kids (encapsulated organisms)

acute chest in adults

134
Q

sickle cell trait

A

less than 50% HbS, usually don’t sickle

  • expect in renal medulla
  • leads to decrease ability to concentration urine
135
Q

metabisulfite

A

causes any cell to sickle

- screens for trait

136
Q

hemoglobin C

A

in beta chain

  • C has lysine
  • mild anemia
  • have hemoglobin C crystals
137
Q

paroxysmal nocturnal hemogloinuria

A
  • deactivating CDs on RBC block complement (lacking GPi)
  • acquired
  • due to acidosis activating complement at night
138
Q

findings in PNH

A
  • intravascular hemolysis

- hemoglobinuria, hemoglobinuria, hemosiderinuria days later

139
Q

tests for PNH

A
  • sucrose for screening
  • acidify serum to confirm
  • check for CD55
140
Q

death in PNH

A

thrombosis (platelets lack GPi and get destroyed)

141
Q

other complications in PNH

A
  • iron anemia

- AML (due to other mutations)

142
Q

G6PD deficiency

A
  • X Linked recessive
  • less NADPH produced
  • more susceptible to oxidative stress
143
Q

cause of oxidative stress

A

infection, drugs, fava beans

144
Q

things seen in G6PD

A
  • Heinz bodies (hemoglobin precipitates)
  • intravascular hemolysis
  • bite cells
  • hemoglobinuria
  • back pain (nephrotoxic)
145
Q

Heinz prep

A

screen for G6PD

146
Q

when to due enzyme studies

A

after everything is resolved

147
Q

IgG hemolysis

A
  • extravascular
  • warm
  • remove membrane
  • leads to spherocytes
  • removed by spleen
  • associated with lupus and CLL and drugs
148
Q

IgM hemolysis

A
  • intravascular
  • cold
  • fixes complement killing cells
  • mycoplasma pneumoniae and mono
149
Q

diagnosis in Ab hemolysis

A

Coombs test

- test for Ab on cell (direct) or in serum (indirect)

150
Q

when is MAHA seen

A 
TTP - platelet thrombi
HUS - toxin produced
DIC - platelet and fibrin thrombi
HELLP
aortic stenosis
fake heart valves
151
Q

MAHA findings

A

schistocytes and helmet cells

152
Q

low production anemias

A

micro/macrocytic anemia
renal failure
- parovirus B19
- aplastic anemia

Pathoma (11 decks)

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