Hematology Flashcards

1
Q

RBC membrane transporter

A

HCO3-/Cl- to sequester bicarb and transport CO2

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2
Q

Platelet lifespan

A

10 days

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3
Q

platelet dense granules contents

A

ADP (A dense P), calcium (a dense mineral)

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4
Q

alpha granules contents

A

vWF fibrinogen

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5
Q

vWF receptor

A

GpIb (von wille BRAND)

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6
Q

Fibrinogen receptor

A

GpIIb/IIIa

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7
Q

Macrophages MHC class

A

II

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8
Q

Dendritic cells MHC

A

II

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9
Q

T cell costim signal

A

CD28

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10
Q

MHC II associated with CD

A

4

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11
Q

MHC I associated with CD

A

8

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12
Q

Activates fibrinogen

A

thrombin

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13
Q

activates thrombin

A

Va using prothrombin

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14
Q

First step in clotting cascade

A

XII activation by collagen, BM, or platelets

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15
Q

bradykinin actions

A

vasodilation
permeability
pain

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16
Q

Major extrinsic pathway factor

A

VII activated by TF

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17
Q

Prothrombin times measures extrinsic/intrinsic

A

extrinsic

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18
Q

PTT time measures extrinsic/intrinsic

A

intrinsic

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19
Q

Vit K and coagulation

A

procoagulation via factor formation

warfarin inhibits this production

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20
Q

Protein C and coagulation

A

Anticoagulation; requires protein S to cleave and inactivate Va and VIIIa

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21
Q

tPA and coagulation

A

Anticoagulation; activates plasminogen to plasmin which starts fibrinolysis

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22
Q

Heparin and anticoagulation

A

Anticoagulation; activates antithrombin

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23
Q

Warfarin MOA

A

inhibits epoxide reductase (reduces vitamin K in process of making factors)

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24
Q

Clot formation step 1

A

Injury: vWF binds exposed collagen

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25
Q

Clot formation step 2

A

Adhesion: platelets bind vWF via GpIb(rand) at injury site
platelets release dense granules (ADP and Ca)
ADP increases platelet adherence via shape changes

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26
Q

Clot formation step 3

A

Activation: ADP binding causes GpIIb/IIIa (fibrinogen r) receptor expression on platelet surface

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27
Q

Clot formation step 4

A

Aggregation: fibrinogen binds and links platelets

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28
Q

Pro-aggregation platelet factors

A

TXA2
Low blood flow
Inc. aggregation

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29
Q

Anti-aggregation factors

A

PGI2 and NO (endothelium)
Inc. blood flow
Dec. platelet aggregation

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30
Q

Clopidogrel MOA

A

Inhibits GpIIb/IIIa expression by blocking ADP receptor

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31
Q

Abciximab MOA

A

GpIIb/IIIa mAb

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32
Q

Glanzmann’s thrombasthenia main issue

A

GpIIb/IIIa defect

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33
Q

Basophilic stippling

A

Little specks in round RBC
BASte the ox TAiL:

Thalassemias
Anemia chronic
lead poisoning

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34
Q

Bite cells

A

G6PD deficiency

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35
Q

Ringed sideroblasts

A

Excess iron in mitochondria (sideroblastic anemia)

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36
Q

Schistocyte

A

DIC, TTP/HUS, mechanical hemolysis

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37
Q

Spherocyte

A

hereditary spherocytosis or autoimmune hemolysis

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38
Q

Teardrop cell

A

Bone marrow infiltration forces extramedullary hematopoiesis (sheds a tear because forced out of home)

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39
Q

Target cell

A
HALT he said to the target:
HbC
Asplenia
Liver 
Thalassemia

Excessive RBC membrane

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40
Q

Heinz bodies

A

Oxidation of iron causes denatured Hgb precipitation and membrane damage -> bites taken out

G6PD

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41
Q

Howell-Jolly bodies

A

Nuclear remnants in RBC
Normally removed by spleen
Hypo-/asplenia or mothball ingestion (naphthalene)

looks like olive (Olive-Olly bodies)

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42
Q

Microcytic anemias

A
Iron deficiency
ACD
Thalassemia
Sideroblastic
Lead poisoning
43
Q

Normocytic nonhemolytic anemias

A

ACD at first
CKD
Aplastic anemia

44
Q

Normocytic hemolytic intrinsic anemias

A
Hereditaryspherocytosis
G6PD
HbC
SCD
PNH
45
Q

Normocytic hemolytic extrinsic anemias

A

Autoimmune
Microangiopathic
Macroangiopathic
Infection

46
Q

What is megaloblastic anemia

A

RBCs with high nuclear:cytoplasm ratio due to impaired DNA synthesis (think about requirements for DNA synthesis being lost as a cause)

47
Q

Megaloblastic anemias

A

Folate deficiency

B12 deficiency

48
Q

Non-megaloblastic macrocytic anemias

A

Liver disease
Alcoholism
Reticulocytosis

49
Q

What finding is always present in megaloblastic anemias?

A

Hypersegmented neutrophils

50
Q

Iron deficiency lab/slide profile

A

Low free iron
Inc. TIBC (free transferrin)
Low ferritin

Microcytosis

51
Q

What is Plummer-Vinson syndrome?

A

Iron deficiency anemia +
Esophageal webs +
Atrophic glossitis

Blood + Throat + Tongue

52
Q

Alpha thalassemia defect

A

Alpha globin

53
Q

Alpha thalassemia cis deletion common in __

A

Asians - ChIneSe

54
Q

Alpha thalassemia trans deletion common in __

A

TRANSatlantic - Africa

55
Q

Alpha thalassemia 4 gene deletion –>

A

No alpha globin –> gamma 4 globin (Hb Bart’s) –> dead at birth hydrops fetalis

56
Q

Hb Bart’s is _

A

gamma 4 globin from no alpha

57
Q

Alpha thalassemia 3 gene deletion

A

HbH

Excess B so B4 (HbH)

58
Q

Alpha thal 1 or 2 gene deletion

A

clinically insignificant

59
Q

Alpha thalassemia is bad because it disrupts the__

A

Alpha:gamma globin ratio

Gamma 4 is the worst

60
Q

Alpha thalassemia smear

A

Microcytic anemia (not enough alpha globin so similar to iron deficiency)

61
Q

Beta thalassemia major issue

A

Dec. B-globin synthesis from point mutations

62
Q

Beta thalassemia major population to think of

A

Mediterraneans and Italians!

63
Q

B-thal minor

A

Asymptomatic
Heterozygote
Diagnosis by inc. HbA2 >3.5 on gel

64
Q

This hemoglobin is necessary from gestation on

A

alpha

65
Q

B thal major smear

A
Anisocytosis
Poikilocytosis
Microcyotsis
Target cells
Schistocytes
66
Q

B thal major

A

B chain absent so severe anemia req/ blood transfusion
Marrow expands to make up leading to crew cut skull XR and skeletal deformities

Inc. HBF (alpha 2 gamma 2)

67
Q

Folate deficiency findings

A

hyperseg PMNs
glossitis
high homocysteine

68
Q

B12 deficiency risk factors

A

vegans
malabs (Crohn’s)
Pernicious anemia
PPIs

69
Q

B12 deficiency findings

A

Neurologic symptoms!
Peripheral neuropathy
Dementia

70
Q

Findings of intravascular hemolysis

A

High LDH

Hemoglobinuria

71
Q

Findings of extravascular hemolysis

A

Spleen clears RBCs
Inc. LDH
Inc. unconj bilirubin –> jaundice

72
Q

Mechanism of ACD

A

Inflammation causes inc. hepcidin which binds ferroportin and inhibits iron transport

73
Q

ACD lab findings

A

Low iron
Low TIBC
High ferritin

74
Q

Hereditary spherocytosis mechanism

A

defective membrane-associated proteins causes round cells that get caught by spleen causing splenomegaly (ankyrin, band 3, protein 4.2, spectrin)
extravascular

75
Q

Hereditary spherocytosis smear

A

No central pallor
Inc. MCHC

(Premature removal by spleen)

76
Q

G6PD deficiency mechanism

A

low glutathione and inc. RBC susceptibility to oxidant stress
extravascular hemolysis

77
Q

G6PD deficiency smear

A
Heinz bodies  (oxidized iron bodies)
Bite cells
78
Q

Pyruvate kinase deficiency presentation

A

hemolytic anemia in a newborn

Low ATP leads to rigid RBCs

79
Q

PNH mechanism

A

intravascular

Complement-mediated RBC lysis due to low decay-accelerating factor

80
Q

PNH triad

A

Hemolytic anemia
Pancytopenia
Venous thrombosis

81
Q

SCD most common mutation

A

Point mutation G6V of beta chain

82
Q

Virus to watch out for with SCD

A

Parvovirus B19 bc of aplastic crisis

83
Q

Howell jolly bodies would be found after __ in SCD

A

Autosplenectomy

84
Q

AIHA Warm agglutinin antibodies

A

IgG because from chronic anemia from SLE, CLL, drugs, etc.

85
Q

AIHA cold agglutinin antibodies

A

IgM because from acute anemia by CLL, Mycoplasma, or mono

86
Q

Microangiopathic anemia smear findings

A

Schistocytes

87
Q

Microangiopathic anemias

A
DIC
TTP
HUS
SLE
Malignant hypertension
All cause mechnical destruction
88
Q

Macroangiopathic anemia mechanism

A

Prosthetic valves and aortic stenosis, heavy running causes schistocytes and hematuria

89
Q

Hemophilia A defect

A

Deficiency of factor VIII which increases PTT

90
Q

Hemophilia B defect

A

Deficiency of factor IX which increases PTT also

91
Q

PTT measures all factors except:

A

VII and XIII

92
Q

Bernard-Soulier syndrome mechanism

A

Defective GpIb (vWF receptor) causes low platelet adhesion

93
Q

Glanzmann’s thormbasthenia mechanism

A

Defective GpIIb/IIIa (ADP receptor) so defective platelet-platelet aggregation

94
Q

ITP defect

A

GpIIb/IIIa antibodies causes splene consumes complexes and inc. megakaryocytes

95
Q

TTP defect

A

ADAMTS13 (vWF cleaver)
Multimers cause platelet aggregation and thrombosis
Labs shows schistocytes and inc. LDH (hemolysis)

96
Q

TTP presentation

A
Pentad:
Neurologic
Renal
Fever
Thrombocytopenia
Microangiopathic hemolytic anemia
97
Q

von willebrand factor is highly associated with this coagulation factor

A

VIII

protects / carries it

98
Q

Causes of DIC

A
Sepsis
Trauma
OB complications
Pancreatitis
Malignancy
Nephrotic syndrome
Transfusion
99
Q

DIC labs

A

Schistocytes, D-dimer, low fibrinogen, low factor V and VIII

100
Q

Factor V Leiden mutation

A

Mutant V resists degradation by protein C and causes hypercoagulability; MC inherited hypercoagulability in whites

101
Q

Protein C or S deficiency

A

Inability to inactivate V and VIII causes inc. thrombotic skin necrosis with hemorrhage after warfarin administration

102
Q

Main benefit of transfusing fresh frozen plasma

A

Coagulation factors

103
Q

Uses of FFP

A

DIC, cirrhosis, warfarin overdose