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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
Thrombocytopenia can result from
Decrease in platelet production Increased sequestration (holding) of platelets in the spleen Decreased platelet survival
26
What are some specific ways that thrombocytopenia can be caused?
Drug-induced Heparin-induced Autoimmune disorders Thrombotic
27
Which drugs can cause thrombocytopenia?
Heparin (anticoagulant) Aspirin Atorvastatin Some antibiotics
28
Why might autoimmune diseases cause thrombocytopenia?
Platelets can be destroyed or their formation is inhibited by the immune system
29
Thrombotic Thrombocytopenic Purpura is a combination of what?
``` Thrombocytopenia Hemolytic anemia Renal failure Fever Neurologic abnormalities ```
30
Blood coagulation defects can result from
Deficiencies | Impaired function of one or more of the clotting factors
31
What are two examples of inherited bleeding disorders?
Von Willebrand disease Hemophilia
32
What is deficient in Von Willebrand disease?
Von Willebrand factor **The VWF gene provides instructions for making blood clotting protein Von Willebrand factor**
33
Which inheritance pattern is most common with Von Willebrand disease?
Type 1 - Autosomal dominant
34
What is deficient in hemophilia?
Factor VII gene **The F7 gene provides instructions for making a protein called coagulation factor VII**
35
Which inheritance pattern is most common with hemophilia?
X-linked recessive
36
Who is most effected by hemophilia?
Males (x-link recessive!!!)
37
What are two ways hemophilia can be aquired?
Liver disease Vitamin K deficiency
38
Liver disease can cause hemophilia because the
Synthesis of clotting factors is reduced
39
Vitamin K deficiency can cause hemophilia because
It is needed for normal coagulation activity
40
DIC stands for
Disseminated Intravascular Clotting ``` Disseminated = spread through organ/body Intravascular = in the vascular system ```
41
What are the characteristics of DIC?
Widespread coagulation and bleeding in the vascular compartment
42
DIC is a primary or secondary disease?
Secondary - it occurs as a complication of other conditions
43
DIC begins with ____ Resulting in ___ At the same time, what is reduced? ___ result and cause ___ Finally, what is the last result?
Massive activation of the coagulation sequence Systemic formation of fibrin (essential for blood clotting) Anticoagulants Microthrombi >>> 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)
44
If DIC is activated through the extrinsic pathway it involves Some examples would be
Tissue injury OB complications Trauma Bacterial sepsis Cancer
45
If DIC is activated through the intrinsic pathway it involves This is due to
Extensive endothelial damage ``` Viruses Infections Immune mechanisms Blood stasis Temp extremes ```
46
What is the most common cause of DIC?
OB disorders (50%)
47
Why are OB complications so high in causing DIC?
Tissue factors released may be the trigger of DIC
48
If a patient had a platelet disorder, what would you expect to see?
``` Bleeding - from gums Bruising Epistaxis (nosebleed) Blood stools ```
49
If a patient had purpura you would see what?
A rash of purple "spots" Small blood vessels burst causing blood to pool under the skin Purpura is spanish for purple :)
50
If a patient had petechiae you would see what?
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**
51
What would be the clue that a patient has Von Willebrand disease versus Hemophilia?
Bleeding from externally Hemophilia presents with internal bleeding, VW disease presents with bleeding outside of the body
52
With DIC, you'd expect to see
Petechiae Purpura Oozing from puncture sites Severe hemorrhage
53
Acute manifestations of DIC are mostly due to
Bleeding problems
54
What are the jobs of RBCs?
Carry O2 to the tissues >> via hemoglobin Carry CO2 Participate in acid-base balance (we just covered this! woohoo)
55
What hormone is critical for RBC production? | Where is it produced?
Erythropoietin In the kidney **made in response to decreased O2 levels**
56
Anemia is abnormally low Results in diminished
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**
57
Every type of anemia presents with (3)
Tachycardia (HR 100+ bpm) Fatigue Pallor
58
Effects of acute blood loss are mainly due to This can lead to
Loss of intravascular volume Cardiovascular collapse and shock
59
Acute blood loss leads to ___ which stimulates proliferation (rapid increase) of ___
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
60
Chronic blood loss leads to __
Iron deficiency anemia (70% of iron is found in hemoglobin, just a fun fact)
61
What are some causes of chronic blood loss?
GI bleeding | Menstrual disorders
62
Hemolytic anemia is
Disorder where blood cells are destroyed faster than they are created
63
Sickle cell anemia is what kind of disorder? What does it lead to?
Inherited and recessive Chronic hemolytic anemia Pain Organ failure
64
Sickle cells vs normal cells
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).
65
A mother and father are both carrier (heterzyg) - what are the chances the child will be a carrier or have the disease?
Carrier - 50% | Affected - 25%
66
Abnormal hemoglobin leads to a big risk of
Sickling (sickled cells)
67
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?
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
Two consequences of sickling are
1. Chronic hemolytic anemia | 2. Blood vessel occlusion
69
Factors associate with sickling
``` Cold Acidosis (too much acid) Dehydration Physical exertion Stress Infection Hypoxia ``` >>> all have decreased O2
70
Sickle cell anemia can manifest in many ways. What are the big ones?
Severe hemolytic anemia Chronic hyperbilirubinemia (jaundice) Vaso-occlusive crisis (traffic jam of sickled cells blocking blood flow and causing pain)
71
Iron deficiency can result from
**It is very common** Dietary deficiency Increased demands Bleeding
72
As RBCs get old, they ____ and ___ is released; it is then reused to produce ___
Break down and iron is released >> the iron is used to produce more RBCs **It's the circle of liiiiiife**
73
If you wanted to increase your iron with food, your best bet would be to eat
Meat
74
What is a common cause of iron deficiency in the Western world? What are some examples?
Chronic blood loss - there isn't enough iron for recycling ``` GI bleeding Vascular lesions Intestinal polyps (lead to GI bleeding) Hemorrhoids Cancer ```
75
With a patient diagnosed with iron deficiency anemia, what would their symptoms be?
``` Fatigue Palpitations Dyspnea Angina (severe chest pain) Tachycardia ```
76
Vitamin B12 is responsible for
Red cell maturation and division | Prevention of abnormal fatty acids being incorporated into neuronal lipids
77
Where is B12 found?
In all food of animal origin
78
The rate of development for B12 deficiency anemia is
Slllloooooooowwwww
79
To be absorbed, B12 must bind to ___ which is secreted by ____
Intrinsic factor (protein that helps intestines absorb B12) Gastric parietal cells
80
If a patient has a gastrectomy or gastric bypass, are they more or less likely to develop B12 deficiency anemia?
More likely | >>> If there is less gut, there is less intrinsic factor for B12 to bind to = less B12 absorption
81
What symptoms would a patient with B12 deficiency anemia have?
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
Why would there be neuro changes in someone with B12 deficiency anemia?
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
Folic acid is required for And is found where?
DNA synthesis Red cell maturation Green, leafy veggies Fruits Meat
84
Symptoms of folic acid deficiency anemia is similar to which other anemia?
Vitamin B12 deficiency anemia >> B12 has neuro changes while folic acid doesnt
85
Common causes of folic acid deficiency
Malnutrition or dietary lack of folic acid | Increased alcohol consumption
86
Should a pregnant woman increase or decrease her intake of folic acid?
Increase | >> It helps prevent some birth defects
87
Aplastic anemia is a form of
Bone marrow failure
88
Aplastic anemia results in a reduction of
All three hematopoietic cell lines RBCs WBCs Platelets
89
A big cause of aplastic anemia is exposure to
High doses of > Radiation > Chemicals > Toxins that suppress hematopoiesis (formation of blood components)
90
Aplastic anemia can occur with infections such as
Viral hepatitis Infection mononucleosis Other viral illnesses
91
A patient with aplastic anemia might present with what symptoms?
``` Weakness Fatigue Pallor Petechiae Ecchymosis (discoloration of the skin caused by bleeding underneath) Bleeding from nose, gums, vagina, or GI Prone to infection ```
92
WBCs and lymphoid tissue function to
Protect the body against invasion of foreign agents
93
WBC and lymphoid tissue disorders can be due to
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
Leukopenia is the
Decrease in number of leukocytes (most often neutrophils) in the blood
95
Neutrophils play a critical role in what?
The host-defense mechanism against infection
96
What are the causes of Neutropenia?
Autoimmune Infection-related Drug-related
97
Autoimmune neutropenia results from
Antibodies directed against neutrophil cell membrane antigens or bone marrow progenitors **Either way, neutrophil count is decreasing**
98
What is the most common infection-related cause of neutropenia?
Viral diseases
99
Infections can produce neutropenia in what ways? In what way can it be aqcuired?
>Decreased neutrophil production >Loss of neutrophils by toxins >Problems resulting in neutrophil sequestration in the spleen (what a hog) Immunodeficiency syndrome
100
Drug-related neutropenia is
Idiosyncratic - reaction is different from the effect obtained in most people and that cannot be explained in terms of allergy
101
Neutropenia manifestations are
Mild skin lesions Stomatitis (inflamed/sore mouth) Pharyngitis (inflamed pharynx) Diarrhea ``` -Severe- Malaise Chills Fever Weakness Fatigue ```
102
Why would you not see symptoms in someone with severe neutropenia?
There aren't enough neutrophils to cause an inflammatory response
103
Which type of lymphoma is in the lymph nodes and spreads to the whole body?
Non-Hodgkin Lymphoma (NHL)
104
Which type of lymphoma is in a single node or group of nodes?
Hodgkin Lymphoma
105
NHLs represent a clinically diverse group of
B-cell, T-cell, or NK-cell origin
106
What are the causes of NHL?
Unknown, but linked to > EBV > Human T-lymphotropic virus > Helicobacter pylori
107
What are the most common NHL subtype?
B-cell lymphomas
108
B-cell lymphomas predominantly affect
Lymph nodes ``` Also > Spleen > Bone marrow > Peripheral blood > Head and neck region > GI tract > Skin ```
109
What would a patient with Non-Hodgkin Lymphoma present with?
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
Which lymph nodes are often involved with NHL?
Retroperitoneum Mesentery Pelvis
111
Many low-grade lymphomas eventually transform into
More aggressive forms of lymphoma/leukemia
112
Hodgkin lymphoma has the presence of what abnormal cell?
Reed-Sternberg cell
113
Who is more likely to get HL?
People from 15-40, and then 55+
114
What are the possible causes of HL?
Exposure to carcinogens and viruses Genetic and immune mechanisms
115
A patient with HL might present with what?
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
Leukemias are malignant ___ of cells originally derived from ____
Neoplasms (abnormal growths) Hematopoietic precursor cells (cells that create new blood cells)
117
Leukemias are characterized by
Diffuse replacement of the bone marrow with unregulated, proliferating, immature neoplastic cells
118
Your patient is a child with cancer, based off of statistics, what kind of cancer is she likely to have?
Leukemia (makes up 29% of cancer cases in children)
119
Leukemias are commonly classified according to
Their predominant cell type > Lymphocytic or Myelocytic Whether it is acute or chronic
120
Lymphocytic leukemias involve ____ lymphocytes and progenitors that originate in ___
Immature Bone marrow
121
Myelogenous leukemias involve the ___ stem cells in bone marrow and interfere with ___
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
Which leukemia grows VERY fast?
Acute leuk
123
Which leukemia grows slowly
Chronic **occurs in mature cells!**
124
What causes leukemia?
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
ALL stands for AML stands for
Acute lymphocytic leukemia Acute myelogenous leukemia
126
ALL is composed of
Precursor B or precursor T cells
127
Approximately 90% of people with ALL have
Numeric and structural changes in the chromosomes of their leukemic cells
128
ALL most frequently affects who?
Children 1/3 of cases are adults
129
AML affects what kind of cells?
Myeloid precursor cells in the bone marrow
130
Most AMLs are associated with acquired
Genetic alterations
131
AML has an accumulation of This leads to
Undifferentiated blast cells Anemia Neutropenia Thrombocytopenia
132
AML affects who?
Older adults 1/4 of cases are children
133
Both ALL and AML present with what symptoms?
``` Fatigue (from anemia) Low grade fever Night sweats Weight loss Bleeding Bone pain Infection Generalized lymphadenopathy (lymph node swelling) ```
134
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)
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
What symptoms are specific to AML?
Malignant cells infiltrate skin, gum, and other soft tissues
136
What happens when leukemic cells cross the blood brain barrier?
Causes CNS symptoms **more common in ALL**
137
CLL stands for CML stands for
Chronic Lymphocytic Leuk Chronic Myelogenous Leuk
138
CLL is a
Clonal (arising from a single cell) malignancy of B lymphocytes
139
CML is a disorder of
The pluripotent hematopoietic progenitor cell
140
CML harbors the
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
Both chronic leukemia primarily affect
Older people
142
CLL is related to
Progressive infiltration of bone marrow and lymphoid tissues by neoplastic lymphocytes
143
Which chronic leukemia is indolent and often astymptomatic?
CLL
144
Lab work for CLL shows
Increased lymphocytes
145
What happens as CLL progresses?
Lymph nodes gradually increase in size
146
In more aggressive CLL, you would see a patient with
Lymphadenopathy Hepatosplenomegaly (liver and spleen swollen) Fever ABD pain Weight loss Anemia Thrombocytopenia (low blood platelet count)
147
What are the three phases of CML?
Chronic Accelerated Terminal blast crisis
148
Which stage of CML is slow with weakness, weight loss, and anemia as the symptoms?
Chronic
149
Which CML stage is characterized by enlargement of spleen?
Accelerated
150
Which CML stage is the evolution to acute leukemia?
Terminal blast crisis
151
Terminal blast crisis is characterized by
Myeloid precursors Splenomegaly (enlarged spleen) Infiltrates of leukemic cells in skin, lymph nodes, bones, CNS
152
Multiple myeloma has high __ rates and occurs mostly in
High morbidity and mortality rates | Older people
153
What are some risk factors of multiple myeloma?
Chronic immune stimulation (causes immune exhaustion) Autoimmune disorders Exposure to ionizing radiation Occupational exposure to pesticides or herbicides
154
Multiple myeloma is characterized by
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
Multiple myeloma is associated with
Chromosomal abnormalities
156
In multiple myeloma, one of the characteristic features of proliferating osteoclasts (degrade bone) is
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
Do antibodies increase or decrease in multiple myeloma?
Decrease
158
The main sites that show manifestations of multiple myeloma are
Bones and bone marrow
159
In cases of multiple myeloma, there are proliferating osteoclasts that lead to ____ and predisposes the person to
Bone reabsorption and destruction Pathologic fractures and hypercalcemia
160
In multiple myeloma, what is one of the first symptoms to occur?
Bone pain

RMC Patho Exam 2 (6 decks)

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