Chapter 21- Alteration in Haematological function Flashcards
1
Q
What are some alterations in eythrocytes?
A
Anemias
Polycythemias
2
Q
What are Anemias?
A
Condition of too few erythrocytes or an insufficient volume of them in blood
3
Q
What are Polycythemias?
A
Erythrocyte (RBC) numbers or volume is excessive
4
Q
What are some alterations in Leukocytes?
A
Leukocytosis
proliferative disorders
5
Q
What is Leukocytosis?
A
Increased numbers of leukocytes (response to infections, etc.)
6
Q
What is an example of a proliferative disorder altering leukocytes?
A
Leukemia
7
Q
What is the purpose of clotting?
A
To stop bleeding
8
Q
What is clotting?
A
interaction between endothelium, platelets and clotting componenets
9
Q
What do clotting disorders result from?
A
Alterations in any of the three components
-Endothelium, platelets and clotting
10
Q
Anemia
A
reduction in the total number of RBCs in blood or decrease in quality or quantity of hemoglobin
11
Q
How is anemia classified?
A
Based on changes that affect cells’ size and hemoglobin content
12
Q
What do terms that end in ‘cytic’ refer to?
A
Cell size
13
Q
What do terms that end in ‘chromic’ refer to?
A
Hemoglobin content
14
Q
Anisocytosis
A
RBCs varying in size
15
Q
Poikilocytosis
A
RBCs assuming various shapes
16
Q
What is the main alteration caused by anemia?
A
Reduced oxygen-carrying capacity of blood
17
Q
What is the effect of reduced RBC concentration in blood?
A
Reduction in consistency and volume of blood
18
Q
What is the end result of reduced RBC concentration?
A
Decreased blood viscosity
Blood flows faster
19
Q
What is the effect of blood flowing faster as a result of anemia?
A
Increased heart rate
Increased stroke volume
20
Q
Hypoxemia
A
Reduced oxygen levels in the blood
21
Q
How does the body compensate for hypoxemia?
A
Dilation of vessels
—> Decreased systemic resistance
Increased blood flow through vessels
Increased blood flow to the heart
Increased heart rate and stroke volume
22
Q
Compensation of Hypoxemia can result in ____ ______?
A
Heart failure
23
Q
What are 3 kinds of Anemias?
A
- Macrocytic-Normochromic Anemias
- Microcytic-Hypochromic Anemia
- Normocytic-Normochromic Anemia
24
Q
What is Macrocytic-Normochromic Anemia?
A
Large stem cells (megaloblasts) in bone marrow that form unusually large RBC
25
Macrocytic-Normochromic Anemia: Hemoglobin?
Normal content of hemoglobin
26
What causes Macrocytic-Normochromic Anemia?
Ineffective RBC DNA synthesis
27
What leads to ineffective RBC DNA synthesis?
Vit B and Folic acid deficiencies
28
What is the result of ineffective RBC DNA synthesis?
Affected RBC die in circulation ultimately decreasing RBC concentration and leading to anemia
29
What is Eryptosis?
Premature death of damaged erythrocytes
30
What are some Macrocytic-Normochromic Anemias?
Pernicious Anemia
Folate Deficiency Anemia
31
What is the most common Macrocytic type of anemia?
Pernicious Anemia (PA)
32
What causes Pernicious Anemia?
Vit B deficiency
33
Pernicious
Highly injurious or destructive
34
What is Pernicious anemia?
An autoimmune condition that prevents your body from absorbing vitamin B12
35
How does pernicious anemia prevent the absorption of Vit B12?
PA individuals produce antibodies against parietal cells
---> Parietal cells produce intrinsic factor
---> Intrinsic factor required for Vit B12 absorption
36
What is Vitamin B12 needed for?
DNA synthesis in RBC
37
What past bacterial infection can cause Pernicious anemia?
H. pylori
38
How does Pernicious Anemia develop?
Slowly over 20-30 years
-Situation is severe when the patient gets treatment
39
Describe pernicious anemia early symptoms.
Non-specific and often ignored
40
What are Normal Hb lvels?
>120 g/L
41
What is the Hb level of someone with PA when they start to experience classic symptoms?
70-80g/L
42
What are the classic symptoms of Pernicious Anemia?
Fatigue
Paresthesia of feet and fingers
Abdominal pain
Nerve demyelination
43
Paresthesia
Tingling, prickling feeling
44
Are symptoms of PA reversible?
No
They are irreversible
45
What is Folate deficiency anemia?
Folic acid (folate) deficiency affecting RBC production
46
What is Folate's purpose?
Essential vitamin for RBC, RNA and DNA synthesis
47
What are folates?
Coenzymes that are required for synthesis of thymine and purines which affects RBC undergoing rapid cell reproduction
48
How do humans meet daily folate requirements?
Entirely depends on diet
49
What is the daily folate requirement?
50-200mg/day
50
What is folate deficiency anemia similar to appearance wise?
Malnourished appearance
51
What is a specific manifestation of folate deficiency anemia?
Scales on mouth and burning mouth syndrome
(red beefy tongue)
52
How is folate deficiency anemia treated?
Oral folate administration
53
if treatment/administration is effective what happens to folate deficiency anemia?
Anemia disappears in 1-2 weeks
54
What are Microcytic Hypochromic Anemias?
Abnormally small RBC
55
Microcytic Hypochromic Anemias: Hemoglobin?
Reduced amounts of Hb
56
What causes Microcytic Hypochromic Anemias?
iron metabolism disorders
-heme synthesis disorders
-globin synthesis disorders
57
What is the result of iron metabolism disorders?
iron deficeny anemia
Thalassemia
58
Thalassemia
An inherited disorder causing reduced hemoglobin concentration
59
What causes Iron deficiency anemia?
Chronic blood loss
Inadequate iron intake
Metabolic iron deficiency
60
Is chronic blood loss/inadequate iron intake related to iron metabolism dysfunction?
No
-They have no intrinsic dysfunction of iron metabolism
61
What is metabolic iron deficiency?
Dysfunction in iron metabolism
-insufficient iron delivery to bone marrow or impaired iron absorption into bone marrow
62
How much blood needs to be lost to cause iron deficiency anemia?
2-4mL/day
63
How does blood loss lead to iron deficiency anemia?
Normally iron can be recycled by blood loss disrupts this balance
64
What can cause iron deficiencies?
Medications causing GI bleeding
Surgical procedures
Insufficient iron intake
Pica
65
What kind of surgical procedures lead to iron deficiency?
Procedures that decrease transit time and absorption
66
What kind of mediactions cause GI bleeding and lead to iron deficiency?
Aspirin
NSAIDS
67
What is Pica?
disorder causing the eating of non-nutritional substances (dirt, chalk, paper)
68
Iron deficiency anemia is developped in how many stages?
3
69
Iron deficiency anemia development: Stage 1?
irons stores depleted
RBC production remains normal
70
Iron deficiency anemia development: Stage 2?
Insufficient iron transported to bone marrow
iron-deficient RBC production begins
71
Iron deficiency anemia development: Stage 3?
Hemoglobin-deficient RBC begin to replace normal RBCs that are being destroyed
-Anemia
72
What stage of IDA development does anemia occur?
Stage 3
73
At what level of Hb is Iron deficiency Anemia noticed?
70-80 g/L
-This is when the patient experiences classic symptoms
74
What are some symptoms of progressive reductions in Hb level?
Structural changes in epithelial tissue
Koilonychia
Glossitis
Dysphagia
75
Koilonychia
Finger nails become brittle and "spoon-shaped"
76
Glossitis
Tongue papillae atrophy
77
What is the dysphagia associated with reductions in Hb level caused by?
Web of mucus and inflammatory cells at esophageal opening
78
How is Iron deficiency anemia directly diagnosed?
iron stores measure by bone marrow biopsy
79
How is iron deficiency anemia indirectly diagnosed?
Measurement of serum levels of...
-Ferritin
-Transferrin saturation
-or Total iron-binding capacity
80
What is the first step in iron deficiency anemia treatment?
Eliminate the source of blood loss
81
What is the second step in iron deficiency anemia treatment?
iron replacement therapy until serum ferritin level reaches 50 ug/L
(50 millionth of a gram/Liter)
82
What is Normocytic Normochromic anemias?
Normal size and normal hemoglobin content but insufficient in numbers
83
What are the four distinct normocytic normochromic anemias?
Aplastic
Posthemorrhagic
Hemolytic
Chronic inflammation
84
What is aplastic anmeia?
Infliltrative disorders of bone marrow
-Body stops producing enough new blood cells
85
What is posthemorrhagic anemia?
Sudden blood loss with normal iron states
86
What are the 3 subdivisions of hemolytic anemia?
Acquired
Hereditary
Hemolysis
87
What is acquired hemolytic anemia?
Destruction of RBC developed after birth
-Not congenital/hereditary
88
What is hemolytic hereditary anemia?
Destruction of RBC inherited
e.g Sickle cell anemia
89
What is hemolysis?
Destruction of RBC by erytosis
90
Erytosis
Premature death of damaged erythrocytes
91
What is chronic inflammation anemia?
Inflammatory disease
92
Leukocytosis
WBC count is higher than normal
-Causes
-Symptoms
93
Why does leukocytosis occur?
Normal protective response to physiological stressors
94
When does leukocytosis rarely happen?
Rarely in acute bacterial infection
95
When does leukocytosis commonly happen?
Acute viral infections
Especially Epstein-Barr virus
96
When do alterations in leukocytes affect people?
If there are too many or too few WBCs present in the blood
If WBCs in blood are structurally/functionally defective
97
What is Leukopenia?
WBC count is lower than normal
Absolute WBC count <4.0 x 10^9
98
Is the presence of Leukopenia normal?
NO its presence is never normal
99
What is the normal WBC count?
4.5 to 11.0 x 10^9/L
100
Increased risk of infection is associated with what?
Reduction in neutophils
101
When does infection increase rapidly?
Neutrophil count below 1.0 x 10^9/L
102
When does the possibility of life-threatening infection occur?
Neutrophil count below 0.5 X 10^9/L
103
What is Infectious mononucleosis? (IM)
Benign, acute, self-limiting characterised by infection of B-lymphocytes
104
What is a common cause of Infectious mononucleosis?
Epstein-Barr virus (EBV)
-85% of IM
105
What are other less common causes of IM?
HIV
Hepatitis A
Rubella
106
What percentage of people who get EBV, are infected during early childhood?
95%
107
Do most EBv cases develop into IM and why?
No, EBV rarely develops into IM because early infections asymptomatic and supply immunity to EBV
108
How is EBV transmitted in teenagers and young adults?
Through saliva
-aka Kissing disease
109
How does EBV infection begin?
Invasion of B cells which have receptors for EBV
110
Where does EBV infection first appear?
In throat area epithelial cells and spreads to lymphoid tissues
111
life-cycle of EBV
112
What is leukemia?
Malignant, very infec tious disorder of bone marrow and usually blood
113
What is the main feature of leukemia?
The main feature is the uncontrolled proliferation of malignant leukocytes causing decreased production of hematopoietic cells
114
What is common in leukemia?
Chromosomal abnormalities and translocations
115
How do WHO guidelines divide lymphoid neoplasms?
Five categories defined by cell of origin
116
What are the 5 categories of lymphoid neoplasms?
1. Precursor B-cell neoplasms (immature B cells)
2. Peripheral B-cell neoplasms (mature B cells)
3. Precursor T cell neoplasms (immature T cells)
4. Peripheral T cell and NK (natural killer) neoplasms (mature T cells and NK cells)
5. Hodgkin’s lymphoma (Reed-Sternberg cells)
117
What are hematopoietic cells?
Immature cells that can develop into all types of blood cells
118
What are the 2 kinds of leukemia?
Acute
Chronic
119
What is acute leukemia?
undifferentiated and immature cells (blast cells)
the onset of disease is rapid
short survival time
120
What is chronic leukemia?
more differentiated cells but not functionally normal
-slow progression
121
What do most lymphoid neoplasms arise from?
B-cells and T-cell differentiation pathways
122
What kind of disorders are leukemias?
Clonal disorders are driven by genetically abnormal stem-like cancer cells (SLCCs)
123
During leukemia, what are the abnormal WBCs called?
Leukemic blasts
124
What do leukemic blasts do?
Fill bone marrow and spill into the blood
125
What happens when normal bone marrow cells stop functioning during leukemia infection?
Pancytopenia
126
What is Pancytopenia?
Reduction in production of all blood cellular components
127
After pancytopenia, what occurs during leukemia infection?
Translocations occur
128
What kind of translocation can occur during leukemia infection?
Philadelphia chromosome translocations
129
What chromosomes are involved in Philadelphia chromosome translocations?
Chromosome 9 and 22
130
What kind of leukemia has a 95% chance of Philadelphia chromosome translocations?
Chronic myeloid leukemia
131
What kind of leukemia has a 30% chance of Philadelphia chromosome translocations?
Acute Lymphocytic Leukemia
132
What is the result of leukemia chromosome mutations?
Unique protein (BCR-ABL) results in reduced apoptosis and release of immature cells into circulation
133
What are the two types of acute leukemia?
ALL: Acute Lymphocytic Leukemia
AML: Acute Myeloid Leukemia
134
What cells are affected by Acute Lymphocytic Leukemia?
B-cells
T-cells
NK cells
135
What cells are affected by Acute Myeloid Leukemia?
RBC
Platelets
Eosinophils
Neutrophils
Basophils
136
Who are most cases of ALL?
Children
1-10 years old
137
Who are most cases of AML?
Older adults
138
AML has a decreased rate of what?
Apoptosis and differentiation in affected cells
139
Because of decreased apoptosis/differentiation AML people blood becomes?
Populated with dysfunctional blood cells
140
Signs/symptoms of Acute leukemia?
Fatigue
Bleeding
Fever
141
What causes acute leukemia fatigue?
Anemia
142
What causes acute leukemia bleeding?
Low platelet count
143
What causes acute leukemia fever?
Infections caused by dysfunctional immune response
144
What are the 2 kinds of chronic leukemia?
CLL: Chronic Lymphocytic leukemia
CML: Chronic Myeloid leukemia
145
What is Chronic Lymphocytic leukemia
Slow-growing cancer with too many immature lymphocytes found cells in bone and blood
146
What is the most common leukemia in the Western world IN ADULTS?
Chronic Lymphocytic leukemia
147
What is Chronic Myeloid Leukemia?
Slow developing cancer
Too many myeloid cells (RBC and Platelets) being made in bone marrow
148
What is the main deficit in CLL?
B cells fail to mature into Plasma cells
149
What affects 60% of patients with CLL?
Inability to synthesize antibodies.
150
What is the main effect of CML?
Philadelphia chromosome and presence of BCR-ABL protein (present in 95% of CML patients)
151
What is the only known cause of CML?
exposure to ionizing radiation
152
What are the 3 CML phases?
1. Chronic Phase
2. Accelerated Phase
3. Terminal Blast Phase
153
What is the chronic phase (CML)?
Lasts 2-5 years
May be asymptomatic
154
What is the accelerated phase of CML?
Lasts 6-18months
Proliferation of malignant cells
Splenomegaly
Infections
155
Splenomegaly
Enlarged spleen
156
What is the terminal phase of CML?
Survival 3-6 months
Rapid and progressive leukocytosis (increased WBC count)
157
What does the spleen control?
Blood level of white blood cells, red blood cells and platelets
158
How is CLL diagnosed?
Tests for accumulation of monoclonal B cells in blood
159
What are monoclonal B cells?
condition in which a higher-than-normal number of identical B cells are found in blood.
160
Is there a survival advantage for immediate versus delayed treatment of CLL?
No
161
How is Progressive CLL treated?
Chemotherapy
-Its not curative
162
What has achieved prolonged disease-free treatment of CLL?
Stem cell transplants
163
How is CML treated?
Not very treatable
-Current modalities do not cure the disease
164
What is Lymphadenopathy? (LO)
Enlarged lymph nodes due to an increase in lymphocytes and monocytes
165
Lymphadenopathy enlarged lymph nodes are?
Palpable and painful to touch
166
What does localized LO indicate?
Drainage from an infected area
167
When does Generalized LO occur?
Occurs less often
With the occurrence of infection or malignant disease in adults
168
What are the causes of Lymphadenopathy (LO)?
1. Neoplastic disease
2. Immune or inflammatory conditions
3. Endocrine disorders
4. Lipid storage diseases
169
Malignant
very virulent or infectious
170
What are the 2 classifications of malignant lymphomas?
Hodgkin's
Non-Hodkin's
171
What are the basic groups of Hodgkin's and Non-Hodkin's?
B-cell neoplasms
T-cell neoplasms
NK-cell neoplasms
172
What are maliganant lymphomas?
Proliferation of malignant lymphocytes in lymph system
173
Lymphomas are the result of what?
Genetic mutation of infection which causes cell with uncontrolled and excessive growth to accumulate in lymph nodes
174
What is the most common blood cancer in Canada?
Lymphoma
175
How much has the Canadian incidence of Lymphoma increased since 1970?
Double since 1970
176
What has the Canadian incidence of lymphoma increased?
Immune disorders
Diet
metabolic syndrome
Environmental factors
177
What are Hodgkin's lymphoma?
Malignant lymphoma that progresses from one group of lymph nodes to another
178
Presence of what cells are used to diagnose HL but don't always appear with cancer?
Unique B cells called Reed-Sternberg cells
179
Which gender is the incidence of Hodgkin's lymphoma higher?
Male
180
What is the median age for HL?
Median = 64
Peaks: 20-40 and 60-70 yoa
181
What are Reed-Sternberg cells?
Large binuclear B cells
182
What type of cell is accepted as the malignant transformed lymphocyte in Hodgkin's Lymphoma (HL)?
Reed-Sternberg cells
183
What is the triggering mechanism for the transformation of Reed-Sternberg (RS) cells in Hodgkin's Lymphoma (HL)?
The triggering mechanism for cell transformation is unknown
184
What is Hodgkin's Lymphoma derived from?
B cells that have not undergone Successful Immunoglobulin Rearrangement (SIR)
185
What is SIR?
Proper alignment of the two light chains and two heavy chains of an antibody
186
What does proper SIR induce?
Apoptosis
187
What is the survival of SIR linked to?
EBV providing factors that expand tissue growth
188
What is the first sign of HL?
Enlarged painless lymph node on neck
189
What are the common systemic symptoms of HL?
Fever without infection
Drenching night sweats
Itchy skin (Pruritus)
Weight loss
-Combined appearance of these symptoms = poor prognosis
190
How is HL diagnosed with blood work?
Complete blood count (CBC), sedimentation rate and lymph node biopsy
191
What are Non-Hodgkin's Lymphoma?
Heterogenous group of lymphoid tissue neoplasms with differing patterns of activity and responses to treatment
192
What are WHO classifications of NHL?
cell neoplasms: originate from B cells at various levels of differentiation
T cell neoplasms: originate from T cells at various levels of differentiation
NK-cell neoplasms: originate NK cells at various levels of differentiation
193
How are NHL and HL differentiated from one another?
NHL lack Reed-sternberg cells
194
What is the best way to describe NHLs?
A progressive clonal expansion of B, T and NK cells
195
What percentage of NHLs are from B-cells?
90%
196
What activates oncogenes during NHLs?
Translocations
197
How does NHL spread?
Unpredictable manner and spreads early (unlike HL)
198
What is the most common NHL?
Diffuse B cell NHL
199
What are risk factors of NHL?
Old, male and white
Autoimmune disease
Infection of cancer-related virus (HIV, EBV, Hep C)
Immune suppressants bc of Organ transplant
Gastric infection with H. pylori
