Exam 3 Flashcards

1
Q

What is the function of Neutrophils?

A
  1. Phagocytic (engulfs and destroy)
  2. Capable of ameboid movement into the tissues (moves like an ameboid?)
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2
Q

What is the function of Eosinophils?

A
  1. Phagocytic
  2. destroy parasites
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3
Q

What is the function of Basophils?

A
  1. storage of histamine
  2. involved in immediate hypersensitivity reaction
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4
Q

What is the function of Monocytes?

A
  1. phagocytic
  2. cellular
  3. natural immunity
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5
Q

What is the function of Lymphocytes?

A
  1. antibodies
  2. cell-mediated immunity
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6
Q

What are selectins?

A

cell adhesion molecules

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7
Q

What are the Mobilization of Defenses for Leukocytes?

A
  1. Migration (movement between blood cell walls by adhering to different blood vessels)
  2. Diapedesis (movement by squeezing through endothelial cells)
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8
Q

What are the three phases of Phagocytosis?

A
  1. Migration & Diapedesis
  2. Opsonization & Recognition (tagging pathogens and recognizing them)
  3. Ingestion, digestion, and killing
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9
Q

What is the most common cause of leukocytosis?

A

increase in neutrophils in response to a bacterial infection

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10
Q

What is leukocytosis?

A

Total leukocyte count is more than 11.0X10 9/L in adult
(Increased leukocyte count)

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11
Q

What is leukopenia?

A

total leukocyte count is below 4.5 X 10 /L
(Decreased leukocyte count)

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12
Q

What is the most common cause of leukocpenia?

A

decrease in neutrophils

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13
Q

What is a “shift to the left”?

A

the early release of bands and metamyelocytes from the bone marrow into circulation(peripheral blood) in response to inflammation or an infection
(just everything starts earlier due to infection/inflammation… aka more to the left)

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14
Q

What are conditions linked to Reactive Chronic Neutrophilia?

A
  1. Leukocytes <50 X10 9/L (less than 50,000?)
  2. Shift to the left
  3. Presence of toxic granulation, Dohle bodies, and cytoplasmic vacuolization
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15
Q

What two conditions in tandem indicates severe infection?

A
  1. “a shift to the left” of the neutrophil population
  2. Granulocytosis
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16
Q

What does the suffix “-ocytsis” mean?

A

excess or too much

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17
Q

What does the suffix “-penia” mean?

A

deficiency or too little

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18
Q

What is a leukemoid reaction?

A
  1. a physiological response to a NON-CANCEROUS condition like stress or infection
  2. increased WBC (leukocytes) count [more than 50k cells/uL]
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19
Q

What symptoms are commonly associated with a Leukemoid reaction?

A
  1. leukocytosis with neutrophils (more than 50k)
  2. left shift
  3. toxic granulation
  4. dohle bodies
  5. cytoplasmic vacuolization (vacuoles in the cytoplasm)
  6. positive LAP reaction (neutrophils)
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20
Q

What is commonly found in ACQUIRED Neutrophil disorders?

A
  1. Hyper-segmentation (5-7 or more lobes)
  2. Hypo-granularity (less granules in the neutrophils)
  3. Pseudo Pelger-Huet Anomaly
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21
Q

What is commonly found in INHERITED Neutrophil disorders?

A
  1. Pelger-Huet Anomaly [bi-lobed poly]
    looks like a peanut; patient is unaffected by it
  2. May-Hegglin Anomaly
  3. Chediak-Higashi Syndrome
  4. Alder-Reilly Anomaly
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22
Q

What do dohle bodies look like?

A

Sky blue inclusions in the cytoplasm of neutrophils

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23
Q

What conditions would have dohle bodies in the blood smear?

A

infections, burns, myeloproliferative disorders, pregnancy and inflammation

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24
Q

What are dohle bodies composed of?

A

ribosomes and endoplasmic reticulum

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25
Where are dohle bodies? Hint: part of a cell, and where in the cell
periphery of the neutrophil
26
What would change in the morphology of neutrophils?
1. Dohle bodies 2. Toxic granulation 3. Vacuolization (vacuoles in the cytoplasm)
27
What does toxic granulation look like?
dark granulation in neutrophils
28
What is Myeloperoxidase deficiency?
lack in either the quantity or the function of myeloperoxidase dampens the function of neutrophils = inefficient or prolonged killing of bacteria
29
Chediak-Higashi Syndrome has high morality in _____ due to infection
children
30
What disease/syndrome has giant lysosomal granules (granulocytes, monocytes, lymphocytes, platelets)?
Chediak-Higashi syndrome
31
What is Chediak-Higashi Sydrome?
1. Rare autosomal recessive 2. Neutropenia w/ MPO deficiency (with prolonged and inefficient bacterial killing) 3. Defective chemotaxis 4. Lysosomal killing 5. Recurrent bacterial infections 6. High morality in children 7. Giant Lysosomal granules
32
What is the most common caused of defective O2 dependent killing? Why?
Myeloperoxidase deficiency-Chediak-Higashi failure to form bleach from H2O2
33
What is the result of an NBT test [Nitroblue tetrazolium blood test] with Myeloperoxidase deficiency-Chediak-Higashi? What is the purpose?
1. Changes Blue (Normal) [can kill bacteria] 2. To determine if it is Myeloperoxidase deficiency-Chediak-Higashi or chronic granulomatous disease, b/c they are commonly mixed up due to lack of bleach formation
34
What is Myeloperoxidase deficiency-Chediak-Higashi?
1. Defective O2 dependent killing due to H2O2 unable to produce Bleach (HOCl +H2O2) 2. Looks like Chronic Granulomatous Disease (is differentiated with NBT test coming back normal [CGD NBT test will get abnormal blue-black]) 3. Inclusion bodies in neutrophils, No other WBC anomalies 4. Asymptomatic with increased risk for candidiasis (fungal infection of Candida)
35
How is Pelger-Huet Anomaly diagnosed?
More than 70-90% of neutrophils have a bilobed nucleus
36
What unique to the neutrophils in Pelger-Huet Anomaly?
Hypo-segmented neutrophils Nucleus looks like a dumbbell or peanut shaped Pince-nez morphology (looks like it’s wearing round glasses) [ 3 blind mice lookin asssssss]
37
What is Pelger-Huet Anomaly?
1. Autosomal dominant, can be acquired by drugs or leukemia 2. 70-90% of neutrophils are Hypo-segmented (bilobed) neutrophils aka pince-nez morphology
38
What is commonly associated with Chronic Granulomatous Disease?
1. mutations in NADPH oxidase components 2. ineffective bacteria killing 3. failure to generate reactive oxygen species 4. Diagnose with Nitroblue tetrtazolium Test: abnormal remains yellow 5. no WBC anomalies
39
What are the characteristics of May-Hegglin Anomaly?
1. Autosomal dominant 2. Large blue cytoplasmic inclusions found in neutrophils, eosinophils, basophils and monocytes 3. Blue inclusions resemble dohle bodies but are larger 4. This anomaly can be associated with low platelet counts 5. Cytoplasm in the neutrophils contain granule free areas and fibrins of RNA
40
What is unique about Alder-Reilly Anomally?
Cytoplasmic inclusions are composed of *precipitated mucopolysaccharide*
41
What are characteristics of Alder-Reilly Anomally?
1. Autosomal Recessive 2. Dark Azurophilic Granules in the cytoplasm of neutrophils 3. Eosinophils and Basophils are also affected 4. Possibly abnormally granulated and vacuolated lymphocytes 5. Toxic granulation-like in the granules 6. Cytoplasmic inclusions are composed of *precipitated mucopolysaccharide*
42
What is lympocytosis?
an increase in the number of circulating lymphocytes
43
How do you determine absolute lymphocyte count?
multiply the percentage of lymphocytes (obtained from the peripheral differential) by the total WBC count from the complete blood count or CBC. lymphocyte % x total WBC count
44
What is the most common cause of acute lymphocytosis?
infectious mononucleosis
45
What generally causes ABSOLUTE lymphocytosis?
1. acute viral infections (ex. infectious mononucleosis, hepatitis and CMV infection) 2. pertussis 3. Protozoal infections, such as toxoplasmosis 4. Chronic intracellular bacterial infections such as TB or Brucellosis 5. Chronic Lymphocytic Leukemia
46
What generally causes RELATIVE lymphocytosis?
1. Age is less than 2 years old 2. Acute viral infections 3. Connective tissue diseases 4. Thyrotoxicosis 5. Splenomegaly with splenic sequestration of granulocytes 6. Exercise 7. Stress
47
What is the morphology of reactive atypical lymphocytes?
1. a dark blue cytoplasm with a clear halo around the nucleus (also known as plasma cells) 2. scalloped cell borders (when near RBCs) 3. either small or large (...anyways)
48
What causes Infectious Mononucleosis? *
*Epstein Barr Virus* (EBV)
49
What should a young patient with Infectious Mononucleosis NOT DO??
PLAY SPORTS (fatal!!! can rupture spleen)
50
What are characteristics of Infectious Mononucleosis?
1. atypical lymphocytes are present 2. elevated lymphocyte count (differential) 3. elevated liver enzymes (enlarged spleen presses against the liver, releasing liver enzymes) 4. young patient that has fatigue, swollen glands and sore throat
51
What is the difference between leukemia and lymphoma?
Leukemia = monotonous/uniform nalignant lymphocytes (due to cloning) Lymphoma = atypical lymphocytes
52
In what diseases are reactive changes in lymphocytes (abnormal lymphs) present?
1. Infectious Mononucleosis 2. Cytomegalovirus (CMV) 3. Rubella 4. Hepatitis
53
What tests/examinations are ordered for diagnosis of leukemia?
1. CBC and Differential 2. Abnormal blood count (Usually increase in WBC’s) 3. Bone marrow analysis and peripheral smear examination
54
What are the factors included in diagnosis of leukemia?
1. Abnormal Cells (differentiate btwn ALL and AML) 2. Cell surface markers that determines B or T cell origin of the lymphoblasts (using uses multiparameter flow cytometry) 3. Clone specific chromosome abnormality (CML vs. AML) 4. Molecular changes
55
What are the factors included in diagnosis of leukemia?
1. Abnormal Cells (differentiate btwn ALL and AML) 2. Cell surface markers that determines B or T cell origin of the lymphoblasts (using uses multiparameter flow cytometry) 3. Clone specific chromosome abnormality (CML vs. AML) 4. Molecular changes
56
What Clone specific chromosome abnormality is found in CML?
1. Philadelphia chromosome 2. BCR-ABL fusion gene
57
What is the most important pretreatment prognostic information for AML?
any abnormalities found in chromosomal analysis of the leukemic cell
58
What is a worse prognosis for leukemia, acute or chronic?
acute!!! it's sudden, not regulated, and will generally has more immature cells
59
At what age are acute leukemia patient's diagnosed?
any age
60
At what age are chronic leukemia patient's diagnosed?
adults (usually)
61
What is the clinical onset (future development) of acute leukemia?
sudden
62
What is the clinical onset (future development) of chronic leukemia?
insidious
63
What is the course (untreated) of acute leukemia? [life expectancy?]
6 months or less
64
What is the course (untreated) of chronic leukemia? [life expectancy?]
2-6 years
65
What type of leukemic cells does acute leukemia have?*
immature (>30% are blasts)
66
What type of leukemic cells does chronic leukemia have?*
more mature cells
67
What level of anemia and thrombocytopenia is common with acute leukemia?
prominent, prominent
68
What level of anemia and thrombocytopenia is common with chronic leukemia?
mild, mild
69
What levels of WBC count are common with acute leukemia?
variable
70
What levels of WBC count are common with chronic leukemia?
increased
71
What level of lymphadenopathy and splenomegaly is common with acute leukemia?
mild, mild
72
What level of lymphadenopathy and splenomegaly is common with chronic leukemia?
present (prominent), present (prominent)
73
What is the FAB classification of M0 mean?
a Leukemia patient with Acute myeloblastic w/o cytologic maturation
74
What is the FAB classification of M1 mean?
a Leukemia patient with Acute myeloblastic w/ minimal maturation
75
What is the FAB classification of M2 mean?
a Leukemia patient with Acute myeloblastic w/ maturation
76
What is the FAB classification of M3 mean?
a Leukemia patient with Acute promyelocytic
77
What is the FAB classification of M4 mean?
a Leukemia patient with Acute myelomonocytic
78
What is the FAB classification of M5 mean?
a Leukemia patient with Acute Monocytic
79
What is the FAB classification of M6 mean?
a Leukemia patient with Erythroleukemia
80
What is the FAB classification of M7?
a Leukemia patient with Acute Megakaryoblastic
81
What is the WHO classification for acute leukemia?*
if there is more than 20% blast
82
What are environmental factors associated with an increased risk of Leukemia?
1. Ionizing radiation 2. Chemicals (ex. benzene) 3. Drugs (ex. alkylating) 4. Viruses (ex. Epstein Barr, HTLV-2)
83
What are the two types of Acute Leukemia?
1. Myeloid (granulocytes) 2. Lymphoid (lymphocytes)
84
Is the ME (myeloid) ratio in erythro-leukemia, high or low?
Low, increased erythrocytes means decreased myelocytes (-/+)
85
In what type of leukemia, are Auer rods present?
AML
86
Where is the translocation of the chromosomes in Philadelphia Chromosome in CML?
22 and 9
87
In an uncomplicated case of Infectious mononucleosis, what is affected?
lymphocytes (atypical lymphs)
88
Reactive lymphocyte seen in blood smear of infectious mononucleosis, is derived by?
T-Lymphocytes
89
Where would you see increased basophils?
CML
90
What is Chronic Lymphocytic Leukemia (CLL) defined as?
Accumulation of Monoclonal B cells
91
What type of leukemia is Hairy cell leukemia?
chronic lymphocytic leukemia
92
What disease most likely associated with granulocytes hyposegmentation?
Pelger-Huet Anomaly
93
What is Chediak-Higashi syndrome is associated with?
a membrane defect of lysosomes
94
What is Alder-Reilly Anomaly associated with?
mucopolysaccharide
95
What is May-Hegglin Anomaly associated with?
dolhe bodies and giant platelets
96
What is normal myeloblast morphology?
large in size and have prominent nucleoli
97
How is myeloblastic and lymphoblastic linage confirmed?
cytochemical staining and immunophenotyping
98
What is normal lymphoblast morphology?
small to medium sized blasts with dense chromatin and the nucleoli are less distinctive
99
If the patient has a poor leukemia diagnosis, What type of cells are found in the blood smear?
blast cells
100
What is the use of flow cytometry for leukemia patients?
1. analyzing cell surface 2. cytoplasmic antigen expression in leukemia blasts [important for therapeutic decisions]
101
What are chronic myeloproliferative syndromes?
acquired clonal disorders from the bone marrow that are classified by extra or vast amount of proliferation of one or more of the non lymphoid cell lines (myeloblastic)
102
What is a characteristic of both chronic myeloproliferative syndromes and infectious mononucleosis?
enlarged spleen (splenomegaly)
103
What syndromes are listed under chronic myeloproliferative syndromes ?
1. chronic myelogenous leukemia 2. polycythemia vera 3. essential thrombocythemia 4. chronic idiopathic myelofibrosis 5. chronic neutrophilic leukemia 6. chronic eosinophilic leukemia 7. hyper eosinophilic syndrome 8. an unclassifiable myeloproliferative disease
104
What is an abnormalities found in the blood of a Chronic Myelogenous Leukemia patient?
1. an intense increase in the WBC 2. neutrophilic leukocytosis with immature forms 3. increased basophils (8-12) 4. increased eosinophils 5. increased monocytes 6. increased granulocytes and thrombocytes (hyperactivity in the BM)
105
What is the only cure for Chronic Myelogenous Leukemia?
Bone Marrow Transplant
106
What does the BCR-ABL fusion gene do in Chronic Myelogenous Leukemia?
a protein is continuously made that stimulates granulocyte CML
107
What are common symptoms of Chronic Myelogenous Leukemia?
1. fatigue 2. feeling full in the upper abdomen region due to an enlarged spleen 3. night sweats 4. weight loss 5. tenderness of the bone 6. bones feeling achy 7. being anemic
108
What are the three phases of Chronic Myelogenous Leukemia, in order from safest to dangerous?
1. Chronic 2. Accelerated 3. Blast
109
What is the most common acute leukemia in adults?
Acute Myelogenous Leukemia
110
What is the speed of Acute Myelogenous Leukemia, if untreated?
quick
111
What is the most common risk factor for Acute Myelogenous Leukemia?
myelodysplastic syndrome
112
What are risk factors of Acute Myelogenous Leukemia?
1. myelodysplastic syndrome 2. myelofibrosis 3. aplastic anemia
113
What tests are ordered for Acute Myelogenous Leukemia?
1. bone marrow assessment 2. flow cytometry 3. molecular tests (karyotyping, FISH, PCR, and DNA sequencing)
114
How is Acute Myelogenous Leukemia diagnosed?*
1. 20% of cells in BM/blood smear is myeloblast 2. auer rods are seen in the myeloid blast cytoplasm (large crystalline cytoplasmic inclusion bodies) (3. big nucleus)
115
What is the most common (chronic) leukemia found in adults?
Chronic Lymphocytic Leukemia (CLL)
116
Is hairy cell leukemia common or rare?
rare
117
What is the speed of hairy cell leukemia?
slow! or severity stays stagnant
118
What will hairy cell leukemia look like microscopically?
1. too many abnormal B cells 2. the B cells have *five lone projections* "hairy"
119
What is the treatment for hairy cell leukemia?
chemotherapy
120
What is used to diagnose hairy cell leukemia?*
TRAP stain
121
What is the pathology of Chronic Lymphocytic Leukemia (CLL)?
BM to the blood to the lymph nodes, spleen, and liver
122
What is abnormal about the lymphocytes in Chronic Lymphocytic Leukemia (CLL)?
1. cells last longer and will crowd out normal cells 2. do not fight infection well (dysfunctional)
123
What is uniquely found in the Chronic Lymphocytic Leukemia (CLL) blood smear?*
*Smudge cells*
124
What is the treatment for Chronic Lymphocytic Leukemia (CLL)?
1. chemotherapy 2. targeted therapy 3. stem cell replacement 4. monoclonal antibodies
125
What is the most common cancer in children?
Acute lymphoblastic Leukemia (ALL)
126
What are the symptoms of Acute lymphoblastic Leukemia (ALL)?
1. bone pain 2. fever 3. pale skin 4. anemia 5. weakness 6. fatigue 7. lumps caused by swollen glands
127
What is the cause of Acute lymphoblastic Leukemia (ALL)?
mutations in the bone marrow cells, development of too many lymphoblasts that crowd out the normal cells
128
What are lymphoblasts?
Bone marrow produces immature cells that develop into leukemic cells
129
What are risk factors of Acute lymphoblastic Leukemia (ALL)?
1. Previous cancer treatment 2. exposure to radiation 3. genetic disorders
130
How is the diagnosis of Acute lymphoblastic Leukemia (ALL) determined?
bone marrow biopsy and blood tests
131
How do Myeloproliferative disorders emerge?
a malignant transformation of a single multipotential stem cell that is responsible for the differentiation of granulocytes, monocytes, erythrocytes and platelets
132
What is a common characteristic of Myeloproliferative disorders? What is it?
1. Panhyperplasia of the bone marrow 2. hematopoietic tissue of the marrow cavity is replaced with adipose or fat tissue
133
What are patients with Myeloproliferative disease predisposed to?
leukemic transformation
134
What is diagnostic for Polycythemia Vera?
Elevation of the hematocrit Males: 58% or higher Females: 52% or higher
135
What gene mutation do 90% of Polycythemia Vera (PV) patient's have?
V617F mutation in the JAK2 gene
136
What kind of phase are 20% of Polycythemia Vera (PV) patients in?
"spent" phase with marrow fibrosis, splenomegaly getting worse and pancytopenia
137
What is treatment for Polycythemia Vera (PV)?
1. frequent phlebotomy and/or 2.use of cytotoxic myelosuppressive agents (chemical RBC suppressant)
138
What is Idiopathic Myelofibrosis?
Anemia with: 1. teardrop poikilocytosis 2. elevated platelet counts 3. leucoerythroblastic blood picture 4. elevated bone marrow fibrosis 5. enlarged spleen
139
What is found in a peripheral blood smear of a patient with Idiopathic Myelofibrosis?
1. Giant, bizarre platelets (large dysfunctional platelets) 2. small platelets
140
What symptoms are seen in Progressive State of Idiopathic Myelofibrosis?
1. Weight loss 2. hypertension 3. pancytopenia
141
What is the cure for Idiopathic Myelofibrosis?
bone marrow transplant
142
What is essential thrombocytosis?
elevated platelet count (up to greater than 1mil)
143
What are clinical features of essential thrombocytosis?
1. thrombosis 2. hemorrhage from dysfunctional platelets! (abnormal increased platelets are prone to be dysfunctional)
144
What should high risk patients for essential thrombocytosis receive?
cytoreductive therapy with hydroxyurea
145
What medicine prevents stroke, but thins out blood?
aspirin
146
How do you differentiate from AML and ALL?
myeloperoxidase stain
147
What's the difference between AML and ALL?
AML: ADULTS!, Auer Rod ALL: KIDS!, lymphoblasts