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RMC Patho Exam 2 > Disorders of Hematopoietic System > Flashcards

Disorders of Hematopoietic System Flashcards

1
Q

Hemostasis is the

A

Stoppage of blood flow

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2
Q

Hemostasis is due to

A

Too much or not enough platelets

A clotting factor issue

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3
Q

Disorders of hemostasis are disorders of

A

Platelet

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4
Q

Hypercoagulability is due to ___ and leads to ___

A

Increased hemostasis

Thrombosis and blood vessel occlusion

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5
Q

Disorders of hypercoagulability are due to

A

Increased platelet function

Increased clotting activity

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6
Q

Increased platelet function results in (3)

A
  1. Platelet adhesion
  2. Platelets clot
  3. Disrupted blood flow
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7
Q

Thrombocytosis is

A

Elevated platelet count (above 1,000,000)

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8
Q

What is the most common cause of secondary thrombocytosis?

A

A diseased state that stimulates thrombopoietin production

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9
Q

Thrombopoietin is the

A

Key hormone in platelet production

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10
Q

What are some situations in which you would see secondary thrombocytosis?

A

Surgery (tissue damage), infection, cancer, chronic inflammation

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11
Q

What is the cause of primary thrombocytosis?

A

A disorder in bone marrow

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12
Q

How does thrombocytosis present (clinical manifestations)?

A

Thrombosis
DVT
Pulmonary embolism
Hemorrhage

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13
Q

Why might you see hemorrhaging in thrombocytosis? (put it all together!)

A

Because platelets are abnormal and not working properly (no clotting = bleeding out)

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14
Q

The coagulation system can be inappropriately activated because of (2)

A

Primary disorders

Secondary disorders

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15
Q

Primary disorders are

Secondary disorders are

A

Genetic

Acquired

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16
Q

What is an example of a primary disorder?

A

Factor V Leiden

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17
Q

Factor V Leiden is a

A

Common hereditary thrombophilia (a blood disorder that make blood clot easier)

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18
Q

Primary disorders have an increased risk of

A

VTE- venus thromboembolism (includes DVT and PE)

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19
Q

VTE stands for

DVT stands for

PE stands for

A

Venous Thromboembolism

Deep Vein Thrombosis

Pulmonary embolism

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20
Q

What are some secondary disorder factors?

A 
Immobility
MI
Cancer
Obesity
Oral contraceptives
Smoking
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21
Q

Someone with hypercoagulability due to increased clotting activity might present with

A

Recurrent venous and arterial thrombi

Cardiac valvular vegetations (abnormal growths in heart valves)

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22
Q

Thrombi can affect what systems/organs?

A

Brain
Heart
Renal and peripheral arteries

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23
Q

Platelet disorders are due to (3)

A

Decreased production
Increased destruction
Impaired function

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24
Q

Thrombocytopenia is the

A

Reduction of platelets

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25
Q

Thrombocytopenia can result from

A

Decrease in platelet production

Increased sequestration (holding) of platelets in the spleen

Decreased platelet survival

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26
Q

What are some specific ways that thrombocytopenia can be caused?

A

Drug-induced
Heparin-induced
Autoimmune disorders
Thrombotic

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27
Q

Which drugs can cause thrombocytopenia?

A

Heparin (anticoagulant)
Aspirin
Atorvastatin
Some antibiotics

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28
Q

Why might autoimmune diseases cause thrombocytopenia?

A

Platelets can be destroyed or their formation is inhibited by the immune system

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29
Q

Thrombotic Thrombocytopenic Purpura is a combination of what?

A 
Thrombocytopenia
Hemolytic anemia
Renal failure
Fever
Neurologic abnormalities
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30
Q

Blood coagulation defects can result from

A

Deficiencies

Impaired function of one or more of the clotting factors

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31
Q

What are two examples of inherited bleeding disorders?

A

Von Willebrand disease

Hemophilia

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32
Q

What is deficient in Von Willebrand disease?

A

Von Willebrand factor

The VWF gene provides instructions for making blood clotting protein Von Willebrand factor

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33
Q

Which inheritance pattern is most common with Von Willebrand disease?

A

Type 1 - Autosomal dominant

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34
Q

What is deficient in hemophilia?

A

Factor VII gene

The F7 gene provides instructions for making a protein called coagulation factor VII

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35
Q

Which inheritance pattern is most common with hemophilia?

A

X-linked recessive

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36
Q

Who is most effected by hemophilia?

A

Males (x-link recessive!!!)

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37
Q

What are two ways hemophilia can be aquired?

A

Liver disease

Vitamin K deficiency

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38
Q

Liver disease can cause hemophilia because the

A

Synthesis of clotting factors is reduced

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39
Q

Vitamin K deficiency can cause hemophilia because

A

It is needed for normal coagulation activity

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40
Q

DIC stands for

A

Disseminated Intravascular Clotting

Disseminated = spread through organ/body
Intravascular = in the vascular system
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41
Q

What are the characteristics of DIC?

A

Widespread coagulation and bleeding in the vascular compartment

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42
Q

DIC is a primary or secondary disease?

A

Secondary - it occurs as a complication of other conditions

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43
Q

DIC begins with ____

Resulting in ___

At the same time, what is reduced?

___ result and cause ___

Finally, what is the last result?

A

Massive activation of the coagulation sequence

Systemic formation of fibrin (essential for blood clotting)

Anticoagulants

Microthrombi&raquo_space;> vessel occlusion and tissue ischemia (clots form not allowing blood to flow)

Hemorrhage (b/c the clot formation uses all coagulation agents leaving blood to flow)

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44
Q

If DIC is activated through the extrinsic pathway it involves

Some examples would be

A

Tissue injury

OB complications
Trauma
Bacterial sepsis
Cancer

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45
Q

If DIC is activated through the intrinsic pathway it involves

This is due to

A

Extensive endothelial damage

Viruses
Infections
Immune mechanisms
Blood stasis
Temp extremes
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46
Q

What is the most common cause of DIC?

A

OB disorders (50%)

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47
Q

Why are OB complications so high in causing DIC?

A

Tissue factors released may be the trigger of DIC

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48
Q

If a patient had a platelet disorder, what would you expect to see?

A 
Bleeding
- from gums
Bruising
Epistaxis (nosebleed)
Blood stools
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49
Q

If a patient had purpura you would see what?

A

A rash of purple “spots”

Small blood vessels burst causing blood to pool under the skin

Purpura is spanish for purple :)

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50
Q

If a patient had petechiae you would see what?

A

Tiny brown/purple spots on the skin

Caused by capillaries breaking open

Difference from purpura is these are less than 2 mm, purpura are larger

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51
Q

What would be the clue that a patient has Von Willebrand disease versus Hemophilia?

A

Bleeding from externally

Hemophilia presents with internal bleeding, VW disease presents with bleeding outside of the body

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52
Q

With DIC, you’d expect to see

A

Petechiae
Purpura
Oozing from puncture sites
Severe hemorrhage

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53
Q

Acute manifestations of DIC are mostly due to

A

Bleeding problems

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54
Q

What are the jobs of RBCs?

A

Carry O2 to the tissues
» via hemoglobin
Carry CO2
Participate in acid-base balance (we just covered this! woohoo)

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55
Q

What hormone is critical for RBC production?

Where is it produced?

A

Erythropoietin

In the kidney
made in response to decreased O2 levels

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56
Q

Anemia is abnormally low

Results in diminished

A

RBCs, hemoglobin, or both

O2-carrying capacity
(RBCs carry O2, less RBCs=less O2)

If you think you breath heavy walking up stairs, wait until you hear someone with anemia

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57
Q

Every type of anemia presents with (3)

A

Tachycardia (HR 100+ bpm)
Fatigue
Pallor

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58
Q

Effects of acute blood loss are mainly due to

This can lead to

A

Loss of intravascular volume

Cardiovascular collapse and shock

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59
Q

Acute blood loss leads to ___ which stimulates proliferation (rapid increase) of ___

A

Hypoxia
Committed erythroid stem cells in bone marrow (essentially cells that create RBCs)

> > > this just means that hypoxia causes more RBCs to be produced to carry O2 and stop the hypoxia

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60
Q

Chronic blood loss leads to __

A

Iron deficiency anemia (70% of iron is found in hemoglobin, just a fun fact)

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61
Q

What are some causes of chronic blood loss?

A

GI bleeding

Menstrual disorders

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62
Q

Hemolytic anemia is

A

Disorder where blood cells are destroyed faster than they are created

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63
Q

Sickle cell anemia is what kind of disorder?

What does it lead to?

A

Inherited and recessive

Chronic hemolytic anemia
Pain
Organ failure

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64
Q

Sickle cells vs normal cells

A

If an RBC is the moon, a normal cell is a full moon and a sickle cell is a crescent moon

Now, think of a normal RBC like an innertube floaty that has the middle part intact (no hole). Lots of O2 can sit comfy and go for a ride. With sickle cell, O2 has to go white water rafting with a stupid pool noodle only (not much can hang on).

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65
Q

A mother and father are both carrier (heterzyg) - what are the chances the child will be a carrier or have the disease?

A

Carrier - 50%

Affected - 25%

66
Q

Abnormal hemoglobin leads to a big risk of

A

Sickling (sickled cells)

67
Q

If a patient has sickle cell anemia, what conditions do you want to watch for and why?

What about with a patient who is just a carrier?

A

Low oxygen (working out, smoking, high altitude)

It triggers HbS to sickle

There is little tendency for them to have symptoms, let them live their life

68
Q

Two consequences of sickling are

A
  1. Chronic hemolytic anemia

2. Blood vessel occlusion

69
Q

Factors associate with sickling

A 
Cold
Acidosis (too much acid)
Dehydration
Physical exertion
Stress
Infection
Hypoxia

> > > all have decreased O2

70
Q

Sickle cell anemia can manifest in many ways. What are the big ones?

A

Severe hemolytic anemia
Chronic hyperbilirubinemia (jaundice)
Vaso-occlusive crisis (traffic jam of sickled cells blocking blood flow and causing pain)

71
Q

Iron deficiency can result from

A

It is very common

Dietary deficiency
Increased demands
Bleeding

72
Q

As RBCs get old, they ____ and ___ is released; it is then reused to produce ___

A

Break down and iron is released
» the iron is used to produce more RBCs

It’s the circle of liiiiiife

73
Q

If you wanted to increase your iron with food, your best bet would be to eat

A

Meat

74
Q

What is a common cause of iron deficiency in the Western world?

What are some examples?

A

Chronic blood loss - there isn’t enough iron for recycling

GI bleeding
Vascular lesions
Intestinal polyps (lead to GI bleeding)
Hemorrhoids
Cancer
75
Q

With a patient diagnosed with iron deficiency anemia, what would their symptoms be?

A 
Fatigue
Palpitations
Dyspnea
Angina (severe chest pain)
Tachycardia
76
Q

Vitamin B12 is responsible for

A

Red cell maturation and division

Prevention of abnormal fatty acids being incorporated into neuronal lipids

77
Q

Where is B12 found?

A

In all food of animal origin

78
Q

The rate of development for B12 deficiency anemia is

A

Slllloooooooowwwww

79
Q

To be absorbed, B12 must bind to ___ which is secreted by ____

A

Intrinsic factor (protein that helps intestines absorb B12)

Gastric parietal cells

80
Q

If a patient has a gastrectomy or gastric bypass, are they more or less likely to develop B12 deficiency anemia?

A

More likely

|&raquo_space;> If there is less gut, there is less intrinsic factor for B12 to bind to = less B12 absorption

81
Q

What symptoms would a patient with B12 deficiency anemia have?

A

Anemia
Mild jaundice
Neuro changes
> Symmetric paresthesia (tingling/pinpricks) of feet and fingers
> Loss of vibratory and position sense
> Spastic ataxia (ataxia - loss of coordination)

82
Q

Why would there be neuro changes in someone with B12 deficiency anemia?

A

Because B12 is involved with the development of brain nerve cells, and if it’s not there, it cannot create the myelin sheath that protects the nerves

> > this causes neuro disorders

83
Q

Folic acid is required for

And is found where?

A

DNA synthesis
Red cell maturation

Green, leafy veggies
Fruits
Meat

84
Q

Symptoms of folic acid deficiency anemia is similar to which other anemia?

A

Vitamin B12 deficiency anemia

> > B12 has neuro changes while folic acid doesnt

85
Q

Common causes of folic acid deficiency

A

Malnutrition or dietary lack of folic acid

Increased alcohol consumption

86
Q

Should a pregnant woman increase or decrease her intake of folic acid?

A

Increase

|&raquo_space; It helps prevent some birth defects

87
Q

Aplastic anemia is a form of

A

Bone marrow failure

88
Q

Aplastic anemia results in a reduction of

A

All three hematopoietic cell lines

RBCs
WBCs
Platelets

89
Q

A big cause of aplastic anemia is exposure to

A

High doses of
> Radiation
> Chemicals
> Toxins that suppress hematopoiesis (formation of blood components)

90
Q

Aplastic anemia can occur with infections such as

A

Viral hepatitis
Infection mononucleosis
Other viral illnesses

91
Q

A patient with aplastic anemia might present with what symptoms?

A 
Weakness
Fatigue
Pallor
Petechiae
Ecchymosis (discoloration of the skin caused by bleeding underneath)
Bleeding from nose, gums, vagina, or GI
Prone to infection
92
Q

WBCs and lymphoid tissue function to

A

Protect the body against invasion of foreign agents

93
Q

WBC and lymphoid tissue disorders can be due to

A

Leukopenia - decreased WBC

Leukocytosis - increased WBC

Other proliferative disorders (infection, neoplasia (uncontrolled, abnormal tissue/cell growth), dyscrasia (abnormal state of body or body part))

94
Q

Leukopenia is the

A

Decrease in number of leukocytes (most often neutrophils) in the blood

95
Q

Neutrophils play a critical role in what?

A

The host-defense mechanism against infection

96
Q

What are the causes of Neutropenia?

A

Autoimmune
Infection-related
Drug-related

97
Q

Autoimmune neutropenia results from

A

Antibodies directed against neutrophil cell membrane antigens or bone marrow progenitors

Either way, neutrophil count is decreasing

98
Q

What is the most common infection-related cause of neutropenia?

A

Viral diseases

99
Q

Infections can produce neutropenia in what ways?

In what way can it be aqcuired?

A

> Decreased neutrophil production

> Loss of neutrophils by toxins

> Problems resulting in neutrophil sequestration in the spleen (what a hog)

Immunodeficiency syndrome

100
Q

Drug-related neutropenia is

A

Idiosyncratic - reaction is different from the effect obtained in most people and that cannot be explained in terms of allergy

101
Q

Neutropenia manifestations are

A

Mild skin lesions
Stomatitis (inflamed/sore mouth)
Pharyngitis (inflamed pharynx)
Diarrhea

-Severe- 
Malaise
Chills
Fever
Weakness
Fatigue
102
Q

Why would you not see symptoms in someone with severe neutropenia?

A

There aren’t enough neutrophils to cause an inflammatory response

103
Q

Which type of lymphoma is in the lymph nodes and spreads to the whole body?

A

Non-Hodgkin Lymphoma (NHL)

104
Q

Which type of lymphoma is in a single node or group of nodes?

A

Hodgkin Lymphoma

105
Q

NHLs represent a clinically diverse group of

A

B-cell, T-cell, or NK-cell origin

106
Q

What are the causes of NHL?

A

Unknown, but linked to
> EBV
> Human T-lymphotropic virus
> Helicobacter pylori

107
Q

What are the most common NHL subtype?

A

B-cell lymphomas

108
Q

B-cell lymphomas predominantly affect

A

Lymph nodes

Also
> Spleen
> Bone marrow
> Peripheral blood
> Head and neck region
> GI tract
> Skin
109
Q

What would a patient with Non-Hodgkin Lymphoma present with?

A

If it is indolent (slow-growing):
> Painless lymphadenopathy (swelling of lymph nodes) typically below the diaphragm

More aggressive:
> Fever
> Drenching night sweats
> Weight loss

110
Q

Which lymph nodes are often involved with NHL?

A

Retroperitoneum
Mesentery
Pelvis

111
Q

Many low-grade lymphomas eventually transform into

A

More aggressive forms of lymphoma/leukemia

112
Q

Hodgkin lymphoma has the presence of what abnormal cell?

A

Reed-Sternberg cell

113
Q

Who is more likely to get HL?

A

People from 15-40, and then 55+

114
Q

What are the possible causes of HL?

A

Exposure to carcinogens and viruses

Genetic and immune mechanisms

115
Q

A patient with HL might present with what?

A

Painless enlargement of one or a group of lymph nodes typically above the diaphragm
> Mediastinal masses are frequent!!

Cough
Dyspnea
Fever
Chills
Night sweats
Weight loss

basic symptoms are the same, look at location of initial lymph node

116
Q

Leukemias are malignant ___ of cells originally derived from ____

A

Neoplasms (abnormal growths)

Hematopoietic precursor cells (cells that create new blood cells)

117
Q

Leukemias are characterized by

A

Diffuse replacement of the bone marrow with unregulated, proliferating, immature neoplastic cells

118
Q

Your patient is a child with cancer, based off of statistics, what kind of cancer is she likely to have?

A

Leukemia (makes up 29% of cancer cases in children)

119
Q

Leukemias are commonly classified according to

A

Their predominant cell type
> Lymphocytic or Myelocytic

Whether it is acute or chronic

120
Q

Lymphocytic leukemias involve ____ lymphocytes and progenitors that originate in ___

A

Immature

Bone marrow

121
Q

Myelogenous leukemias involve the ___ stem cells in bone marrow and interfere with ___

A

Pluripotent myeloid stem cells (pluripotent cells can give rise to all of the cell types that make up the body

The maturation of all blood cells

122
Q

Which leukemia grows VERY fast?

A

Acute leuk

123
Q

Which leukemia grows slowly

A

Chronic

occurs in mature cells!

124
Q

What causes leukemia?

A

The cause is largely unknown, but there are a few things

> Increased incidence with exposure to high levels of radiation

Exposure to
> Benzene (human carcinogen)
> Many unknown toxins (so helpful)
> Drugs
> Chemicals
> Gases
125
Q

ALL stands for

AML stands for

A

Acute lymphocytic leukemia

Acute myelogenous leukemia

126
Q

ALL is composed of

A

Precursor B or precursor T cells

127
Q

Approximately 90% of people with ALL have

A

Numeric and structural changes in the chromosomes of their leukemic cells

128
Q

ALL most frequently affects who?

A

Children

1/3 of cases are adults

129
Q

AML affects what kind of cells?

A

Myeloid precursor cells in the bone marrow

130
Q

Most AMLs are associated with acquired

A

Genetic alterations

131
Q

AML has an accumulation of

This leads to

A

Undifferentiated blast cells

Anemia
Neutropenia
Thrombocytopenia

132
Q

AML affects who?

A

Older adults

1/4 of cases are children

133
Q

Both ALL and AML present with what symptoms?

A 
Fatigue (from anemia)
Low grade fever
Night sweats
Weight loss
Bleeding
Bone pain
Infection
Generalized lymphadenopathy (lymph node swelling)
134
Q

Explain why these things happen with AML and ALL

  1. Weight loss
  2. Bleeding
  3. Bone pain
  4. Infection
  5. Lymphadenopathy, splenomegaly (enlarged spleen), and hepatomegaly (enlarged liver)
A
  1. Rapid proliferation and hypermetabolism of leukemic cells
  2. Low platelet count
  3. Bone marrow expansion
  4. From neutropenia
  5. Infiltration of leukemic cells
135
Q

What symptoms are specific to AML?

A

Malignant cells infiltrate skin, gum, and other soft tissues

136
Q

What happens when leukemic cells cross the blood brain barrier?

A

Causes CNS symptoms

more common in ALL

137
Q

CLL stands for

CML stands for

A

Chronic Lymphocytic Leuk

Chronic Myelogenous Leuk

138
Q

CLL is a

A

Clonal (arising from a single cell) malignancy of B lymphocytes

139
Q

CML is a disorder of

A

The pluripotent hematopoietic progenitor cell

140
Q

CML harbors the

A

Philadelphia chromosome

> Forms when chromosome 9 and chromosome 22 break and exchange portions

> Creates an abnormally small chromosome 22 and a new combination of instructions for your cells that can lead to the development of CML

141
Q

Both chronic leukemia primarily affect

A

Older people

142
Q

CLL is related to

A

Progressive infiltration of bone marrow and lymphoid tissues by neoplastic lymphocytes

143
Q

Which chronic leukemia is indolent and often astymptomatic?

A

CLL

144
Q

Lab work for CLL shows

A

Increased lymphocytes

145
Q

What happens as CLL progresses?

A

Lymph nodes gradually increase in size

146
Q

In more aggressive CLL, you would see a patient with

A

Lymphadenopathy
Hepatosplenomegaly (liver and spleen swollen)
Fever
ABD pain
Weight loss
Anemia
Thrombocytopenia (low blood platelet count)

147
Q

What are the three phases of CML?

A

Chronic
Accelerated
Terminal blast crisis

148
Q

Which stage of CML is slow with weakness, weight loss, and anemia as the symptoms?

A

Chronic

149
Q

Which CML stage is characterized by enlargement of spleen?

A

Accelerated

150
Q

Which CML stage is the evolution to acute leukemia?

A

Terminal blast crisis

151
Q

Terminal blast crisis is characterized by

A

Myeloid precursors
Splenomegaly (enlarged spleen)

Infiltrates of leukemic cells in skin, lymph nodes, bones, CNS

152
Q

Multiple myeloma has high __ rates and occurs mostly in

A

High morbidity and mortality rates

Older people

153
Q

What are some risk factors of multiple myeloma?

A

Chronic immune stimulation (causes immune exhaustion)
Autoimmune disorders
Exposure to ionizing radiation
Occupational exposure to pesticides or herbicides

154
Q

Multiple myeloma is characterized by

A

Proliferation of malignant plasma cells in bone marrow and osteolytic bone lesions throughout the skeletal system

(excessive production and improper function of certain cells)

155
Q

Multiple myeloma is associated with

A

Chromosomal abnormalities

156
Q

In multiple myeloma, one of the characteristic features of proliferating osteoclasts (degrade bone) is

A

Unregulated production of a monoclonal antibody** “M protein”

**Lab-produced molecules that act as substitute antibodies that can restore, enhance or mimic the immune system’s attack on cells

157
Q

Do antibodies increase or decrease in multiple myeloma?

A

Decrease

158
Q

The main sites that show manifestations of multiple myeloma are

A

Bones and bone marrow

159
Q

In cases of multiple myeloma, there are proliferating osteoclasts that lead to ____ and predisposes the person to

A

Bone reabsorption and destruction

Pathologic fractures and hypercalcemia

160
Q

In multiple myeloma, what is one of the first symptoms to occur?

A

Bone pain

RMC Patho Exam 2 (6 decks)

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