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From Flashcardlet > Hematology > Flashcards

Hematology Flashcards

1
Q

Hemostasis

A

The arrest of bleeding

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2
Q

Wright’s stain

A

Used in a peripheral smear of blood to differentiate the types of blood cells

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3
Q

Giemsa stain

A

Used in a bone marrow smear

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4
Q

Virchow’s triangle

A

Vascular components (prostacyclins)
Platelets
Plasma proteins

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5
Q

Primary hemostasis

A

Vasoconstriction and platelet plug formation

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6
Q

Secondary hemostasis

A

Fibrin clot formation on platelet template

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7
Q

What mechanisms cause the local vasoconstriction in primary hemostasis?

A

Myogenic response to injury
Endothelin and thromboxane release
SNS reflex response to pain

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8
Q

Thrombomodulin

A

Binds thrombin, this complex activates protein C to lyse active factors V and VIII

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9
Q

Heparin

A

Binds to antithrombin III to inhibit thrombin, thus inhibiting Factors Xa, IXa, and XIa

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10
Q

Tissue factor inhibitor

A

Inactivates Factors VIIa and Xa

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11
Q

Petechiae

A

Pinpoint hemorrhages <0.5cm in diameter, usually due to thrombocytopenia

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12
Q

Purpura

A

Superficial hemorrhages >0.5cm in diameter, usually due to functional platelet problems

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13
Q

Ecchymosis

A

Subcutaneous purpura due to the escape of blood into the tissues from ruptured blood vessels

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14
Q

Hemorrhage

A

Loss of blood from the vascular system in or out of the body

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15
Q

Normal platelet count

A

150-350,000

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16
Q

Thrombopoietin

A

Made in the liver and released when platelet count gets low, causes maturation of megakaryocytes

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17
Q

Romiplostim

A

Thrombopoietin receptor binding domains attached to the Fc region of IgG Ab to increase the half life of the bindings domains to increase platelet count

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18
Q

Platelet lifespan

A

7-10 days

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19
Q

Why would a person who had a splenectomy have a higher platelet count than normal?

A

The platelets that would have been sequestered in the spleen (1/3 of all platelets) would instead be in the bloodstream

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20
Q

GpIb

A

Binds with vWF to subendothelial collagen for adhesion

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21
Q

GpIIbIIIa

A

Binds to fibrinogen to aggregate with other platelets

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22
Q

Weibel-Palade body

A

Granules inside endothelial cells that store vWF

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23
Q

With what platelet count would you expect “elevated bleeding time”?

A

<70,000

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24
Q

With what platelet count would you expect “post-traumatic bleeding”?

A

20-50,000

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25
Q

With what platelet count would you expect “spontaneous bleeding”?

A

<20,000

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26
Q

Thrombocytopenia

A

<100,000 platelets

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27
Q

What level do you have to keep platelets at to prevent spontaneous bleeding?

A

> 10-20,000

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28
Q

What level do you have to keep platelets to insert a central venous catheter?

A

> 20-50,000

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29
Q

What level do you have to keep platelets before starting anticoagulation?

A

> 30-50,000

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30
Q

What level do you have to keep platelets before minor surgery?

A

> 50-80,000

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31
Q

What level do you have to keep platelets before major surgery?

A

> 80-100,000

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32
Q

Pseudothrombocytopenia

A

False low platelet count due to clumping platelets in the blood draw or in processing

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33
Q

EDTA

A

Calcium chelator, makes it easier for Abs to clump to platelets

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34
Q

Ivy protocol

A

BP cuff inflated at 40 mmHg, make 3 punctures on the forearm… should bleed between 2.5-7.5 min

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35
Q

What would you expect the bleeding time to be with ASA therapy?

A

4-21 min

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36
Q

What other disorders could cause an elevated bleeding time?

A

Thrombocytopenia
Qualitative platelet problems
Vascular disorders

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37
Q

What substances are used in aggregation studies?

A

ADP
Collagen
Epi

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38
Q

What substances are used in adhesion studies?

A

Ristocetin

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39
Q

PFA 100

A

Platelet functioning assay used in aggregation studies

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40
Q

What is the mechanism of action for COX inhibitors?

A

Block the production of thomboxane

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41
Q

ADP receptor blockers

A

Clopidogrel (Plavix)
Prasugrel
Ticlopidine (Ticlid)

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42
Q

GpIIb/IIIa blockers

A

Abxizimab (ReoPro)
eptifibatid (Integrilin)
tirofiban

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43
Q

Adenosine reuptake inhibitor and MOA

A

Dipyridamole (Persantine)

Increase cAMP, which potentiates prostacyclins

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44
Q

Cox-2 inhibitors

A

Block inflammation and the production of prostacyclins by endothelial cells

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45
Q

How do you administer platelet replacement therapy with RDP?

A

1 unit Random Donor Platelets for every 10 kg to increase by 50,000 platelets

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46
Q

How would you administer platelet replacement therapy with SDP?

A

1 unit Single Donor Platelets = 6-7 units RDP

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47
Q

May-Hegglin anomaly

A

Autosomal dominant inherited thrombocytopenia… a mutated nonmuscle myosin heavy chain IIa causes megakaryocytes to have a hard time pinching off platelets

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48
Q

What is a histological finding associated with May-Hegglin?

A

Large platelets circled around a neutrophil, which decreases WBCs phagocytic ability

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49
Q

Wiscott-Aldrich syndrome

A

X-linked inherited thrombocytopenia associated with eczema, immunodeficiency (lack of IgM), and bloody diarrhea (d/t low platelet count)

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50
Q

Congenital Amegakaryocytic Thrombocytopenia

A

Autosomal recessive mutation in the platelet growth factor receptor leads to lack of megakaryocytes, a/w thrombocytopenia absent radius (TAR) syndrome

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51
Q

How would you treat congenital amegakaryocytic thrombocytopenia?

A

Bone marrow transplant

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52
Q

Pancytopenia

A

Reduction in the numbers of RBCs, WBCs, and platelets

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53
Q

Neumega

A

IL-11 stimulates megakaryocyte production and maturation, can treat bone marrow aplasia/hypoplasia

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54
Q

What are some drugs that decrease platelet production?

A

Ethanol
Thiazide diuretics
DES
Tolbutamide

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55
Q

What are some causes of ineffective megakaryopoiesis?

A

Vitamin B12 deficiency

Folic acid

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56
Q

When would you expect to see symptoms of drug-induced thrombocytopenia after starting a new drug?

A

2-8 weeks after starting a new drug

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57
Q

When would you expect drug-induced thrombocytopenia to subside?

A

7-10 days after stopping the drug

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58
Q

Idiopathic Immune Thrombocytopenic Purpura

A

Abs (usually IgG) against GpIb or GpIIb/IIIa, typically chronic in adults, rare spontaneous resolution, 3:1 occurrence in females

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59
Q

What are some common manifestations of ITP a/w platelet counts of <20-30,000?

A

Petechiae, easy bruising, epistaxis, gum bleeding, GI bleed and hematuria less common

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60
Q

Whatis the most common cause of death a/w ITP?

A

Intracranial hemorrhage

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61
Q

Evans syndrome

A

Autoimmune hemolytic anemia with reticulocytosis and spherocytes

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62
Q

What are some other histologic findings a/w chronic ITP?

A

Hemolytic anemia
Elevated LDH (d/t cell destruction)
Elevated bilirubin
Decreased haptoglobin

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63
Q

How would you treat someone with chronic ITP with a platelet count of 30-50,000?

A

No treatment, it’s usually a stable and benign course

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64
Q

How would you treat someone with chronic ITP with a platelet count <20-30,000?

A

Prednisone or dexamethazone (slow Ab production)
Rituximab (slow Ab production)
Consider splenectomy (66% complete remission)

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65
Q

Rituximab

A

Ab to CD20, attaches to B cells so they can’t produce Abs

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66
Q

What are the last line treatments for chronic immune-mediated ITP?

A

Thrombopoietin analogs

Chemo

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67
Q

How does ITP generally present in children?

A

Post-viral infection and is self-limited to t require treatment, but keep out of contact sports d/t increased bleeding

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68
Q

What are the platelet count goals for pregnancy associated ITP?

A

1st trimester: 10-30,000

2nd/3rd trimesters: >30,000 (>50,000 if C-section)

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69
Q

How would you treat pregnancy associated ITP?

A

Moderate dose oral prednisone
Intermittent IVIG
Splenectomy in 1st or 2nd trimesters only

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70
Q

Direct thrombin inhibitors

A

Argatroban

Lepirudin

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71
Q

DDAVP

A

arginine vasopressin, initiates the release of vWF from endothelial cells

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72
Q

Warfarin MOA

A

Inhibits epoxide reductase to prevent the reduction of Vit K, stops production of factors II, VII, IX, X

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73
Q

Alpha 2 antiplasmin

A

Made in the liver, binds circulating free plasmin to prevent fibrinolysis

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74
Q

EACA

A

Epsilon aminocaporic acid, binds to plasmin to pull it out of activity so clots can form

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75
Q

How would you administer Factor VIII concentrates?

A

1 unit/kg raises Factor VIII level by 2% (remember, they don’t need to be at 100%)

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76
Q

How would you treat afibrinogenemia?

A

Cryoprecipitate

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77
Q

Monckeberg’s atherosclerosis

A

“Lead pipe” calcification presents as pipestem vessels prone to cracking… activates platelets and coag factors

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78
Q

Lines of Zahn

A

Soft and dark red with alternating bands of yellow platelets and fibrin visible on autopsy, indicative of arterial thrombosis

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79
Q

How would you treat arterial thrombosis?

A

Embolectomy to remove the clot

Anticoagulation if patient is prone to clotting

80
Q

Mural thrombosis

A

Thrombosis in the heart attached to the endocardium of a heart chamber

81
Q

What are some predisposing factors for developing mural thrombosis?

A

MI (tissue death, exposed collagen)
Atrial fibrillation (stasis)
Endocarditis (vegetations on valves cause tissue damage)
Cardiomyopathy (altered hemodynamics)

82
Q

Where are 80-85% of arterial embolisms from?

A

The heart (mural)

83
Q

What is the normal length of a PTT?

A

28-30s

84
Q

What is the normal length of a PT?

A

9-12s

85
Q

What factors are most sensitive to heparin therapy?

A

Thrombin and Factor X

86
Q

What factors are the most sensitive to warfarin therapy?

A

Factor VII

87
Q

What is a normal RBC count?

A

4.2-6.2 million

88
Q

What is a normal WBC count?

A

5,000-10,000

89
Q

Describe the maturation of an erythrocyte.

A 
Uncommitted pluripotent stem cell meets erythropoietin
Committed proerythroblast
Normoblast
Reticulocyte
Erythrocyte
90
Q

Where are erythrocytes produced before birth?

A

Liver and spleen

91
Q

Where are erythrocytes produced in early childhood?

A

Long bone marrow cavities, liver, and spleen

92
Q

Where are erythrocytes produced in adulthood?

A

Vertebrae, iliac crest, sternum, ribs

93
Q

HbA

A

alpha2, beta2

96% of all Hgb

94
Q

HbA2

A

alpha2, delta2

3% of all Hb

95
Q

HbF

A

alpha2, gamma2

96
Q

HbS

A

alpha2, beta2-mutation

97
Q

Hgb C disease

A

Autosomal recessive
alpha2, beta2-mutation
1 sickle gene, 1 Hg C gene
Hgb precipitates to form crystals, destroyed by spleen (hemolytic anemia)

98
Q

Normal Hemoglobin

A

Female: 12-16 gm/dL
Male: 14-17 gm/dL

99
Q

Normal Hematocrit

A

**should be 3x the hemoglobin
Female: 37-47%
Male: 40-54%

100
Q

Normal MCV

A

82-96 fL

101
Q

Normal MCHC

A

33-37 g/dL

102
Q

Normal Reticulocyte

A

Around 1

103
Q

How do you calculate Reticulocyte Index?

A

% reticulocytes x Hct/45 x 0.5

104
Q

What RI would you expect from someone with a hemolytic anemia?

A

RI >2… the marrow should be hyperproliferative trying to produce more RBCs

105
Q

What do “burr cells” indicate?

A

Increased uric acid

106
Q

What do “spur cells” indicate?

A

Liver disease

107
Q

What hematocrit levels indicate anemia?

A

Female <40%

108
Q

What hemoglobin levels indicate anemia?

A

Female <13.5

109
Q

Why might you notice leukocytosis after acute blood loss?

A

Blood loss causes increased adrenergic hormones, which mobilize granulocytes, causing the leukocytosis

110
Q

What are some characteristics of extravascular hemolysis?

A

Jaundice (increased unconjugated bilirubin) if liver hypo-functional
Splenomegaly if producing RBCs
Increased EPO to stimulate RBC production

111
Q

What are some characteristics of intravascular hemolysis?

A

Decreased haptoglobin from binding the free Hob
Hemoglobinemia d/t used up haptoglobin
Renal hemosiderosis from iron build up in tubular cells
Red/brown urine d/t metHb
Increased EPO to stimulate RBC production

112
Q

What usually causes microcytic anemias?

A

Disorders of Hgb synthesis

113
Q

What usually causes macrocytic anemias?

A

Disorders of maturation (nucleus and cytoplasm development)

114
Q

If a patient has MCV <70, what types of anemia do you suspect?

A

Iron deficiency

Thalassemia

115
Q

How much iron does 1 mL of packed RBCs contain?

A

1mg of iron

116
Q

How much iron does a female lose during menstruation?

A

2-3 mg/day

117
Q

Normal ferritin

A

20-400 ng/mL

118
Q

What lab results for Total iron, Ferritin, TIBC, and % Saturation would indicate iron deficiency anemia?

A

Dec total iron
Dec ferritin
Inc TIBC
Dec % Saturation

119
Q

Describe the 3 physiological phases of iron deficiency anemia.

A
  1. Deplete iron stores (takes 120 days)
  2. Increased erythropoiesis
  3. Increased reticulocytes, small RBCs with low Hgb content
120
Q

What would cause a patient with iron deficiency anemia to have a normal ferritin level?

A

An inflammatory condition like RA or OA

121
Q

How do you treat iron deficiency anemia?

A

Ferrous sulfate 325mg PO TID, gives 150mg/day iron
Could absorb up to 10mg/day
See again in 4 weeks

122
Q

How long do you continue ferrous sulfate therapy?

A

3-6 months after normal Hgb levels to replenish the stores

123
Q

What are some foods/drinks that inhibit iron absorption?

A

Coffee, tea, milk, carbonated drinks, cereals, dietary fiber

124
Q

What are some things that facilitate iron absorption?

A

Vitamin C

Acidic foods

125
Q

What is a classic histological finding of sideroblastic anemia in a bone marrow smear and in a peripheral blood smear?

A 
Ringed sideroblasts (iron that has precipitated out of an erythroblast)
RBC stippling
126
Q

What lab results (peripheral and bone marrow) would indicate sideroblastic anemia?

A

Peripheral: inc iron, inc ferritin

Bone marrow: inc iron, ringed sideroblasts

127
Q

What is the problem in thalassemia?

A

Reduction in the synthesis of globin chains, and the weird RBCs are destroyed more frequently

128
Q

How many genes control the production of alpha chains?

A

4 genes on Chromosome 16

129
Q

How many genes control the production of beta chains?

A

2 genes on Chromosome 11

130
Q

What mutations generally lead to beta thalassemias?

A

Point mutations

131
Q

What causes decreased RBC production in beta thalassemias?

A

Unpaired alpha chains precipitate out of the precursor cell which damages the membrane, causing apoptosis in 70-85% of the RBC precursors

132
Q

What causes increased RBC destruction in beta thalassemias?

A

The microcytic hypochromic RBCs are recognized and destroyed prematurely in the spleen

133
Q

What is a systemic effect associated with beta thalassemias?

A

Erosion of the bony cortex by the RBC precursors
Extramedullary hematopoiesis and hepatosplenomegaly
RBC precursors steal nutrients from ischemic tissue leading to cachexia
Excessive absorption of iron, leading to hemochromatosis

134
Q

What are some histological characteristics of a peripheral smear with a beta thalassemia?

A

Target cells
Basophilic stippling
Normal or increased reticulocytes

135
Q

What is another name for beta thalassemia major?

A

Transfusion dependent anemia

136
Q

Why are children affected by beta thalassemia major normal at birth?

A

They’ve been producing HbF (alpha and gamma chains) and haven’t switched to producing HbA yet

137
Q

Why can children with beta thalassemia have hemochromatosis?

A

They’re being transfused and can’t get rid of iron as quickly as they’re acquiring it

138
Q

Hydroxyurea

A

Increases production of HbF to increase O2-carrying capacity

139
Q

How would you treat beta thalassemia minor?

A

Screening and counseling

140
Q

How would you treat beta thalassemia major?

A

Transfusions with iron chelation
Possible bone marrow transplant
Hydroxyurea

141
Q

What mutation usually causes an alpha thalassemia?

A

Deletion

142
Q

Hemoglobin Barts

A

Gamma4 tetramers d/t lack of alpha chains in newborns

143
Q

Hemoglobin H disease

A

Beta4 tetramers d/t only 1 normal gene for alpha chains in children/adults, hemolytic anemia, pallor, splenomegaly
Common in Asians

144
Q

Hydrops fetalis

A

0 normal genes for alpha chains leads to gamma tetramers in HbF which have very high affinity for O2, little O2 delivery to tissues
Stillborn delivery

145
Q

What lab findings would indicate alpha thalassemia trait?

A 
Dec Hct
Dec MCV
Dec MCHC
Target cells
Normal electrophoresis
146
Q

What lab findings would indicate Hemoglobin H disease?

A 
Dec Hct
Dec MCV
Dec MCHC
Target cells
Abnormal electrophoresis
147
Q

How would you diagnose and treat an alpha thalassemia?

A

Diagnose with electrophoresis

Treat with transfusions, possible bone marrow transplant

148
Q

What is the relationship between Hgb and albumin in someone with anemia of chronic disease?

A

If albumin is 20% below normal, you also expect Hgb to be 20% below normal

149
Q

Describe changes in RBC production and iron stores a/w anemia of chronic disease.

A

Bone marrow fails to increase RBC production

Iron is sequestered in the macrophages

150
Q

What is the most common type of condition a/w anemia of chronic disease?

A

Infections (esp. parasites and fungal)

151
Q

What lab results would indicate anemia of chronic disease?

A 
Dec Hct, Hgb
Dec MCV, MCHC
Reticulocytes depend on bone marrow function
Dec serum iron, TIBC
Increased ferritin
152
Q

How would you treat anemia of chronic disease?

A

Treat the chronic disease

Could give EPO if RBCs are really low

153
Q

What are megaloblastic causes of macrocytic normochromic anemia?

A

Vitamin B12 deficiency

Folate deficiency

154
Q

What is a non-megaloblastic cause of macrocytic normochromic anemia?

A

Liver disease causes defective cholesterol esterification

155
Q

What are some peripheral blood characteristics of megaloblastic anemias?

A

Ovalocytes
Anisocytosis/Pokilocytosis
Dec reticulocyte count
Neutrophils with hypersegmented nucleus

156
Q

Pernicious anemia

A

Vitamin B12 deficiency due to lack of intrinsic factor

157
Q

Chronic atrophic gastritis

A

Loss of parietal cells (they produce intrinsic factor)

158
Q

What do the 3 types of Abs in pernicious anemia do?

A

Type I blocks B12 binding intrinsic factor
Type II blocks the complex binding to the receptor
Type III destroys gastric parietal cells

159
Q

What symptoms do you see with Vitamin B12 deficiency that you don’t see with folate deficiency?

A

Paresthesia, numbness, tingling

Permanent neurological defects d/t demyelination of lateral and posterior spinal cord columns

160
Q

Schilling test

A

Give patient radioactive B12 orally. If they have IF, it will come out in urine. If not, in the feces.

161
Q

What tests would you run to diagnose pernicious anemia?

A 
Vitamin B12 levels (dec)
methylmalonic acid (inc)
162
Q

How would you treat pernicious anemia?

A

Vitamin B12 injections 1000 micrograms QD x7 days, then weekly, then monthly

163
Q

What is the function of folic acid?

A

Important in DNA synthesis

164
Q

What is the normal storage amount of folic acid? How much is the daily requirement?

A

Stored: 5000 mcg

Daily intake: 50-100 mcg

165
Q

What test would you order if you suspect folic acid deficiency?

A

Red cell folate levels

166
Q

How would you treat folate deficiency?

A

Folic acid

167
Q

Spectrin

A

Deficient in the RBC membrane in people with hereditary spherocytosis

168
Q

What causes an aplastic crisis?

A

Acute parvovirus infection can infect the RBC precursors to cease RBC production

169
Q

What can cause a hemolytic crisis?

A

An infection like mono that causes increased destruction

170
Q

What lab results would indicate hereditary spherocytosis?

A

Dec Hct/Hgb
Dec MCV
Inc MCHC

171
Q

What results from an osmotic fragility test would indicate spherocytosis?

A

Cells burst very easily

172
Q

How would you treat hereditary spherocytosis?

A

Splenectomy

Folic acid

173
Q

How is hereditary spherocytosis passed on?

A

Autosomal dominant in 75% of cases

Autosomal recessive in 25% of cases

174
Q

How is G6PD deficiency passed on?

A

X-linked recessive

175
Q

What is G6PD’s normal function?

A

Protective against oxidants

176
Q

GSH

A

Protective free radical scavenger for RBCs, dependent on presence of G6PD

177
Q

Heinz bodies

A

Precipitates from globin chains denaturing

Cause intravascular and extravascular hemolysis

178
Q

What lab results would indicate G6PD deficiency?

A 
\+ family hx
Dec Hct/Hgb
Normal MCV
Normal MCHC
Inc bilirubin (d/t inc hemolysis)
179
Q

How would you treat G6PD deficiency?

A

Remove oxidant stressor
Splenectomy
Folic acid

180
Q

How is sickle cell anemia passed on, and what is the mutation?

A

Autosomal recessive

Point mutation in the beta chain replaces glutamic acid with valine

181
Q

How would a low pO2 affect someone with sickle cell anemia?

A

O2 dissociates from Hgb easier causing RBCs to change shape, the sickled cells are recognized in the spleen to be destroyed or the cells get caught in circulation

182
Q

How does sickle cell anemia lead to a small spleen?

A

Numerous infarcts (d/t sequestered sickle cells) in the small vasculature leads to fibrosis

183
Q

Describe the change of shape that sickle cells undergo in low pO2 conditions.

A 
The low pO2 causes O2 to dissociate from Hgb easier
HbS molecules polymerize
Polymers herniate thru the membrane
Water escapes, leading to dehydration
Cell changes shape
184
Q

Why is it important for people with SCA to have adequate iron?

A

Decreased MCHC increases polymerization, so they should avoid iron-deficiency anemia

185
Q

How does pH affect sickle cells?

A

Decreased pH = Dec O2 affinity = inc sickling

**want diabetic patients to avoid acidotic state

186
Q

What kind of hemolysis takes place in SCA?

A

Intravascular AND extravascular

187
Q

Sickle cell crisis

A

Vaso-occlusion leads to infarction, causing pain

Abdominal pain very common

188
Q

How would you treat someone in a sickle cell crisis?

A

Control pain and give fluids to push sickled cells through the vascular system

189
Q

What is a sequestration crisis?

A

The spleen takes up a ton of sickled cells and enlarges rapidly, blood pools in spleen leading to hypovolemia

190
Q

What is an aplastic crisis?

A

Parvovirus B19 infection can cause cessation of erythropoiesis in the bone marrow

191
Q

What types of infection are people with SCA most susceptible to?

A

Encapsulated organisms (pneumococcus, h. influenzae)

192
Q

How would you treat SCA?

A

Avoid triggers!

Hydroxyurea

193
Q

Fanconi anemia

A

Genetic aplastic anemia with early onset, pancytopenia

194
Q

In what population is idiopathic aplastic anemia most common?

A

80% of those with idiopathic aplastic anemia are >50 y.o.

195
Q

What would you see in the bone marrow in someone with aplastic anemia?

A

“Dry tap”… hypocellular, mostly fat

196
Q

How would you treat someone with aplastic anemia?

A

Transfusions for RBCs, WBCs, platelets

Bone marrow transplant for long-term survival

From Flashcardlet (22 decks)

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