Heme/Onc Exam 2 Cards Flashcards
(318 cards)
1
Q
Proportion of deaths in the US caused by cancer
A
1 in 4
2
Q
Top three cancer causes
A
Breast or Prostate
Lung
Colon
3
Q
Top three cancer deaths
A
Lung
Breast/Prostate
Colon
4
Q
Why might there be more cancer cases but fewer deaths than in the past?
A
We are diagnosing more cancer, but those diagnosed are also living longer
5
Q
In addition to physical morbidity, what two things may cancer also be associated with?
A
Emotional distress and reduction of quality of life
6
Q
Primary prevention
A
Prevents a disease before it even starts - includes addressing risk factors and promoting health
7
Q
Secondary Prevention
A
Screening for early detection and treatment for those at risk
8
Q
Tertiary prevention
A
Rehabilitating, preventing complications and improving quality of life for those with illness
9
Q
Percent of cancer risk that likely comes form your environment
A
90-95% (Most of that from diet)
10
Q
2 cancers associated with lack of physical activity
A
Colon and Breast
11
Q
4 cancers associated with high fat diets
A
Breast, Colon, Prostate, Endometrium
12
Q
Cancer prevented by dietary fiber
A
Colon polyps, Colon cancer
13
Q
4 Carcinogens to remember
3 viruses and 1 substance
A
Epstein Barr virus
Alcohol
H. Pylorii
Hepatitis B or C
14
Q
Sensitivity
A
proportion of persons with the disease who test positive in the screen
15
Q
Specificity
A
Proportion of persons who do not have the disease who test negative in the screen
16
Q
Positive predictive value
A
Proportion of persons who test positive that actually have the disease
17
Q
Negative predictive value
A
Proportion testing negative that do not have the disease
18
Q
At what age should yearly mammograms begin
A
Age 40
19
Q
What and what are the screening guidlines for colon cancer
A
After 45, a colonoscopy every ten years or something else every five years
20
Q
Cervical cancer screening guidelines
A
Begin at age 21
Every 3 years from 21-29
Every 5 years from 30 to 65
Not recommended after 65
21
Q
CAUTION warning signs of cancer
A
Change in bowel or bladder habits
A sore that does not heal
Unusual bleeding or discharge
Thickening lump in breasts, testes, or elsewhere
Indigestion of difficulty swallowing
Obvious change in a mole
Nagging cough or hoarseness
22
Q
3 classic cancer warning signs
A
Nightsweats, Unexplained weight loss, Persistent low grade fever
Also chronic pain and persistent fatigue
23
Q
Clinical vs. Pathalogical staging
A
Clinical is based on physical exam, pathological is based onsurgical findings
24
Q
TNM system
A
Tumor, Node, Metastasis - localized to regional, to systemic
25
Stage IA lung cancer
Smaller than 3cm, no spread to lymph nodes
26
Stage IB lung cancer
3-4 cm not in lymph nodes or main bronchus
27
Difference between lung cancer stages IVA and IVB
IVA can be other lung OR site outside lungs
IVB is at least two sites outside lungs
28
Karnofsky performace index
Rates mobility and autonomy of cancer patients 0=Dead
100=Full independance
29
Tumor marker for gonadal germ cell tumor
Human chorionic gonadotropin (HCG)
Can also indicate pregnancy
30
Tumor marker for medullary thyroid cancer
Calcitonin
31
Tumor marker for hepatocellular carcinoma or germ cell cancer
a-fetoprotein
Can also be cirrosis
32
Tumor marker for colon, pancreas, lung, breast, and ovary
Carcinoembryonic antigen
Can also be pancreatitis, hepatitis, IBF, smoking
33
Lymphoma or Ewing's sarcoma marker
Lactate dehydrogenase
Can also be hepatitis or hemolytic anemia
34
Marker for prostate cancer
Prostate specific antigen
35
Marker for ovarian cancer and some lymphomas
CA-125
Can also be menstruation, peritonitis, or pregnancy
36
Marker for COLON cancer as well as pancreatic and breast
CA-19-9
Can be pancreatitis or UC
37
Agranulocytosis
Absence of granulocytes - Neutrophils, Eosinophils, Basophils
38
Proliferation stage
WBCs can divide but don't yet have special functions
39
Differentiation stage
WBCs have special functions but no longer multiply
40
Immature granulocytes that we should NEVER see in circulation
Metamyelocytes
41
Common progenitor of all non-lymphoid WBCs
Myeloblast
42
Absolute v. relative cell counts
Absolute = actual number of cells - more reliable
Relative = Percentage of total cells
43
3 places neutrophils might be hanging out
In the storage pool in the marrow (reserves)
Extramedullary either free in the bloodstream or attached to endothelial walls (marginal pool
44
Neutrophilic shift
Marginal pool neutrophils move to the general circulation
Can be due to stress
45
True neutrophilia
Release of neutrophils from the storage pool
46
3 types of true neutrophilia
Spurious - We counted wrong
Primary - inherent defect
Secondary - due to another problem like infection
47
When might we see pseudoneutropenia
In the morning
48
Low neutrophil count at which we should worry about serious infection and at which we should consult
Under 500 - Worry
Under 1000 - Consult
49
2 myeloid growth factors for neutropenia
Filgrastim (Neupogen) - Only stimulates granulocytes
Sargramostin (Leukine) - stimulates granulocytes and macrophages
50
Distinguishing factor of eosinophils
Bi-lobed nucleus
51
Complication of eosinophilia and level at which we should be concerned about it
Tissue damage
Most likely to occur at over 1500/microliter
52
Where do most eosinophils live
in the tissues
53
3 organs most commonly affected by eosinophilia
Skin, airway, GI tract
54
2 things released by basophils
Heparin and Histamine
55
Common cause of primary basophilia
Chronic myelogenous leukemia
56
Hallmark of basophils
Blue-black (dark) granules
57
Special property that makes B and T cells different than other WBCs
They can still divide after diffierentiating
58
3 types of t cells and their roles
Helper/CD4 - antibody production and activation of other T cells
Cytotoxic/CD8 - Attack and destroy foreign cells including cancer cells and infected cells
Regulatory T cells - Turn off immune response of other T cells
59
Function of B cells
Present antigens to T cells, can become memory or plasma cells
60
Natural Killer cells
Lymphocytes that do not need stimulation to kill infected cells. Can recognize between foreign and self cells
61
What ages correspond with increased lymphocytes
Younger ages
62
Monoclonal lymphocytosis
More likely a malignancy than polyclonal
63
Polyclonal lymphocytosis
More often due to stress an infection or even a splenectomy
64
3 work ups for lymphocytosis
Repeat CBC, Do a peripheral blood smear, Perform flow cytometry
65
2 common procedures you should NEVER do on an immune compromised patient
Digital rectal exam
Foley catheter placement
66
To nutritional causes of lymphocytopenia
Zinc deficiency and Alcohol abuse
67
Nucleus shape of Monocytes
Kidney shaped or notched nucleus
68
5 potential etiologies for monocytosis
Bacterial infection complicated by neutropenia
Monocytic leukemia or lymphoma
Asplenia
Inflammatory or autoimmune conditions
Treatment with corticosteroids or colony stimulating factors
69
2 pharmacotherapies used for leukopenia
Broad spectrum antibiotics
Granulocyte colony stimulating or granulocyte-macrophage colony stimulating factors
70
3 Additional treatment options for leukopenia
Corticosteroid therapy, Correction of nutritional deficiencies, Splenectomy if unresponsive to all other treatments
71
Left shift
an absolute increase in neutrophils with an increase in bands and sometimes increases in even less mature forms
72
Hypersegmentation
Nuclei with 5 or 6 lobes rather than 3 or 4
73
3 places we might see hypersegmented neutrophils
Megaloblastic anemia, chemotherapy, myeloproliferative disorders
74
Dohle body
Irregularly shaped blue staining area associated with infections
75
Smudge or basket cell
Ruptured WBC remnant from fragile lymphocytes - associated with CLL
76
Platelet satellitosis
Platelets seen adering to WBCs - this leads to an artificially low PLT count
77
Flow cytometry
cells flow past a detector that can detect surface antigens and abnormal cells
78
3 goals of cancer treatment if the cancer cannot be eradicated
Palliation
Treatment of symptoms
Preservation of quality of life
79
Four main subtypes of cancer treatment
Sugery
Radiation
Chemotherapy
Biologic therapy
80
Percent of cancer patients that can be cured by surgery
40%
Surgery may not cure but may be helpful in managing cancer by removing bulk to enhance efficacy and preserving organ function
81
One condition that is needed for successful cancer removal surgery
Clear tumor borders
82
3 types of radiation therapy
Teletherapy - beams at a distance aimed at patient
Brachytherapy - Sources of radiation implanted in or near tumor
Systemic therapy - radionuclides are designed to hone in on site of cancer such as radioactive iodine for thyroid cancer
83
Systemic effects of local radiation therapy (4)
Fatigue, anorexia, nausea, vomiting
84
3 other localized cancer therapies
Radiofrequency ablation - Uses microwaves to cook tumors
Cryosurgery - uses cold to kill lesions
Chemoembolization - Localized administration of chemotherapeutic agents
85
4 types of chemotherapeutic agents
Conventional cytotoxic agents
Targeted agents
Hormonal therapies
Biologic therapies
86
Antimetabolites for chemotherapy (2)
Methotrexate, 5-fluorouracil
87
3 toxic manefestations of antimetabolites
Stomatitis, Diarrhea, Myelosuppression
88
MOA and class of Methotrexate
Competes and counteracts folic acid - Antimetabolite
89
MOA and class of 5-fluorouracil
Prevents thymidine formation - Antimetabolite
90
MOA of antimetabolites
Direct prevention of DNA synthesis
91
3 mitotic spindle inhibitors used in chemotherapy
Vincristine
Vinblastine
Paclitaxel
92
3 common toxic manifestations of mitotic spindle inhibitors
Alopecia, neuropathy, myelosuppression
93
MOA of alkylating agents
Get broken down by cells or spontaneously to form metabolites that covalently modify DNA bases and cross link strands
94
3 Alkylating agents
Cyclophosphamide
Chlorambucil
Cisplatin
95
Class and 3 side effects of cisplatin
Alkylating agent - Neurotoxicity (stocking glove syndrome), hearing loss, renal failure
96
Doxyrubicin
Antitumor antibiotic, binds to DNA and then generates free radicals to damage it
Bright Red
Cardiotoxic
97
Etoposide
Chemo drug, topoisomerase inhibitor that binds to topoisomerase II and causes breaks in the DNA
Can lead to secondary leukemia
98
Treatment for Chemo induced nausea
Ondensetron (Zophran)
99
Treatment for chemo induced neutropenia
Colony stimulating factors like filgrastatin (has a lot of side effects)
100
Mucositis
Oral soreness and ulcerations caused by 5-FU, methotrexate, and cytarabine
Use magic mouthwash to treat
101
Drug that especially causes chemo induced diarrhea and what to give for it
5-FU
Give Loperamide (Immodium) can give octerotide if no response to loperomide
102
Alopecia
Hair loss - chemo cap a controversial treatment
103
Most common side effect of chemotherapy
anemia
104
3 routine blood tests for patients of Chemo
CBC, CMP, PT/aPTT
105
3 presentations of chemo induced nausea
Acute
Delayed
Anticipatory
106
Effect of cancer on coagulation
Causes a hypercoagulable state
107
Paraneoplastic syndrome
Conditions that come with tumors but are not related to the mass effect of the tumor
108
Three ways paraneoplastic syndromes can arise
Initiated by a tumor product
Effect of destruction of normal tissue
Effects due to unknown mechanisms
109
Tumor will often present with ______________ symptoms before being recognized
Endocrine
110
Lambert-Eaton syndrome
Immune mediated neurologic syndrome characterized by muscle weakness of the limbs
111
Subacute cerebellar syndrome
Immune mediated degeneration of the cerebellum - dizzyness nausea and vertigo
112
2 Neurologic paraneoplastic syndromes
Lambert Eaton Syndrome and Subacute cerebellar syndrome
113
Dermatomyositis
Seen with Small cell and Non-small cell lung cancers leads to systemic inflammation of muscles and skin
114
Acanthosis nigricans
Thickening of skin with brown discoloration, often see in T2DM, with cancer you will more likely see it on mucous membranes
115
Neutropenic fever
Recurrent temp above 38 or single temp over 38.3 with a neutrophil count under 500 - often a result of chemotherapy
116
Symptoms of neutropenic fever
vague and mild at first but can rapidly progress to sepsis and death
117
Etiology of neutropenic fever
May be from viral, bacterial or fungal agents
118
PE Workup for a neutropenic fever
Thorough physical exam including access sites but NO digital rectal exam
119
Tx for neutropenic fever
2 Labs
3 Drugs
Take cultures, CXR, Labs and start empiric antibiotic therapy
Ceftazidime for Pseudomonas
Aminoglycoside for gram neg
Vancomysin for MRSA
120
Etiology of oncogenic spinal cord compression
Where does the tumor metastasize to and what are the three mechanisms by which it inflicts damage
Cancer metastasizes to the spinal cord resulting in trauma to the spinal cord from edema, hemorrhage, and pressure induced ischemia
121
Clinical presentation of oncogenic spinal cord compression and 4 things that make it worse
Back pain at the level of the tumor with some nerve root/spinal symptoms
Aggravated by lying down, weight bearing, sneezing or coughing
122
Progression of spinal cord compression symptoms(6)
LE weakness
Hyperreflexia
Motor/Sensory loss
Loss of reflexes
Loss of Bowel/Bladder function
Paraplegia
123
Diagnostic of choice and 3 treatments for oncogenic spinal cord compression
MRI to diagnose
High dose IV corticosteroids
Surgical decompression
Radiation
124
Hallmark of a malignant fracture is that it is _____________________
Atraumatic
125
Three mechanisms of oncogenic hypercalcemia
Tumors release osteolytic proteins
Tumors directly break down bone
Increased absorption of calcium from active vitamin D metabolite
126
MCC of hypercalcemia in cancer patients
Parathyroid hormone-related peptide secreted by cancer cells
127
Mnemonic for hypercalcemia symptoms
Bones - remodeling, fracture risk
Stones - Kidney stones
Groans - Abdominal cramping, nausea and constipation
Moans - Lethargy, depression, psychosis
128
2 EKG signs of hypercalcemia
QT and ST depression
129
4 treatments for hypercalcemia
Hydration and diuresis
Bisphosphonates
Calcitonin
Hemodialysis
130
Tumor lysis syndrome occurrence and most commonly associated cancer
Occurs 1-3 days after radiochemotherapy
Most common in hematologic malignancies, especially Burkitt lymphoma
131
Effects of tumor lysis sundrome
Releases large amounts of cell contents such as phosphorus and potassium into the bloodstream, can cause AKI, cardiac arrhythmias or hyperkalemia
132
2 treatments for tumor lysis syndrome
IV hydration and electrolyte abnormality correction may require emergency hemodialysis
133
Effect of hyperkalemia on EKG
Causes peaked T waves
134
Why do cancer related effusions most often develop
Direct involvement of the serous surface with the tumor
135
2 MCC of malignant pericardial of pleural effusions
Lung or breast cancer
136
4 MCC of malignant ascites
Ovarian, Colorectal, Stomach and Pancreatic cancers
137
3 signs of cardiac tamponade
Narrowed pulse pressure, distended neck veins, muffled heart sounds
138
Diagnostic tool of choice and treatment of choice for cardiac tamponade
Use a transthoracic echocardiogram to diagnose
Use an echo guided percutaneous pericardiocentesis to treat
139
Superior Vena Cava Syndrome - with MCC
Results from direct obstruction of the superior vena cava by malignancies - most commonly caused by bronchogenic carcinoma
140
Physical exam findings for SVC syndrome (3)
Nonpitting edema of the neck, tongue and facial swelling, distended neck arm and chest veins
141
Diagnostic tool of choice and 2 treatments for SVC syndrome
Chest CT with contrast for diagnosis
Glucocorticoids to decrease inflammatory response to tumor
Stenting chemo and radiation alse effective
142
3 elements of Virchow's triad
Stasis, Vessel Wall Injury, HYpercoagulability
143
How can cancer cause each of the virchow's triad elements
Malignancy causes a hypercoagulable state
Neoplastic cells can cause intimal injury
Obstructive tumors can cause venous stasis
144
5 signs of a cancer related thromboembolic event
Low grade fever, tachypnea, tachycardia, pleural rub, unilateral LE swelling
145
2 tools to diagnose thromboembolic events in cancer patients
Ventilation perfusion scan
Spiral chest CT with contrast
146
3 therapy options from first line to last resort for cancer related thromboembolic events
Anticoagulation with LMW Heparin
Rivaroxaban for 21 days PO
Thrombolytic therapy if there is hemodynamic compromise and RV failure
147
What cancer complication may be the initial finding/complaint in a cancer patient
Development of an effusion
148
3 indications for bone marrow aspiration or biopsy
Potential for cancer, blood, or bone
marrow disorder, fever of unknown origin, unexplained splenomegaly
149
Contraindication for bone marrow aspiration/biopsy
Severe bleeding disorders but NOT low PLT count
150
3 sites to avoid when taking a bone marrow biopsy
Infection, injury, excess adipose tissue
151
Preferred and alternate bone marrow biopsy sites
Preferred - posterior iliac crest
Alternate - Anterior iliac crest
152
Sites for aspiration only and indications for use
Tibia - commonly used for infants under 12 months
Sternum only for 12+ years old and morbidly obese patients
153
Etiology of Acute Lymphoblastic Leukemia (ALL)
Originates in a single lymphoblast (lymphocyte progenitor) and often results from a chromosomal translocation
154
4 qualities of A.L.L. mutant lymphoblasts
Rapid proliferation
Reduce normal cell proliferation
Don't differentiate
Resist apoptosis
155
4 places where ALL lymphoblasts accumulate
liver, spleen, meninges, and lymph nodes
156
5 risk factors for ALL
5% genetic
In utero radiation exposure
Chemicals
High birth weight
Lack of exposure to infections - immune system needs to be activated
157
Epidemiology of ALL
Most common ages are under 5 or over 60
More common in caucasians that african americans
158
5 symptoms of ALL
Fever of unknown origin, Pancytopenia, Lymphadenopathy, Bone pain (deep and difficult), Painless testicular swelling
159
Leukostasis of ALL with three associated symptoms
Too many WBCs cause circulation issues leading to HA, altered mental staus, priapism, etc.
160
4 tests in an initial workup of ALL
CBC, CMP, Blood culture if infection is suspected
Imaging depending on symptoms (CXR if pulmonary, MRI/CT if neuro)
161
CBC results suggestive of ALL
High percentages of large unstained cells and lymphocytes - all counts and other percentages are low
162
3 items for further workup in ALL
Peripheral smear, LDH levels (evidence of tissue destruction), CT Chest with contrast
163
Biggest concern in patients with leukostaisis
Brain bleed
164
Why do we want a lumbar puncture in ALL
Spinal infiltration of disease will require different chemotherapy
165
2 antigens expressed by ALL cells
CD19 and CD10 antigens
166
Definitive diagnosis for ALL
Bone marrow aspiration and biopsy with greater than 20% lymphoblasts
167
4 initial treatment steps for an ALL patient
Refer to Heme/Onc
Screen for infection if febrile
Induction chemotherapy
CNS prophylaxis
168
Induction chemotherapy
Multidrug therapy over the course of 4-6 weeks, goal is to induce remission at 65-85%
169
ALL CNS prophylaxis
Intrathecal chemotherapy given through a spinal tap to prevent remission
170
Consolidation/Intensification therapy for ALL
For young patients who respond well
Readminister induction regimen or higher after hematopoiesis is restored
4-8 months to increase remission time
171
Maintenance therapy for ALL
For young patients who tolerate chemo
Less intensive regimen daily or weekly for 2-3 years
172
ALL therapy for older patients who tolerate chemo less well
Allogenic stem cell transplant (from a donor). Once given IV cells with find their way to the bone marrow
173
Apheresis
Removal and replacement of blood that includes removing an element of it along the way
Plasmapheresis removes plasma
Leukopheresis removes WBCs
174
Cure rate and recurrence of ALL
Cure: 90% in children 50% in adults
Recurrence is usually within the first two years
175
4 criteria for poor ALL prognosis
Chromosomal abnormalities
Age over 60
WBC count over 100,000
Failure to achieve remission in 2 weeks of therapy
176
Normal WBC count
4,500-11,000 per microliter
177
Chronic Lymphocytic Leukemia
Malignant lymphoid neoplasm characterized by the accumulation of long lived, functionally incompetent, mature B cells
178
Epidemiology of CLL
Most common form of leukemia, 90% of cases occur after age 50
179
4 clinical symptoms of CLL
Lymphadenopathy, recurrent infections, hepatosplenomegaly, anemia/thrombocytopenia
180
CBC findings for CLL
WBC over 20,000 with isolated absolute lymphocytosis may have decreased RBC and platelets
181
Peripheral smear findings for CLL
Many lymphocytes with some smudge cells and possibly prolymphocytes
182
5 stages of CLL
Low Risk
0 - Lymphocytes over 15,000 and 40%
Intermediate Risk
I - Enlarged nodes in any site
II - Hepatomegaly or splenomegaly
High Risk
III - Anemia
IV - Thrombocytopenia
183
Treatment for low risk CLL
Observe and treat when symptoms appear
184
Treatment for high or intermediate risk CLL (3 options)
Chemo and growth factors to decrease post-chemo neutropenia
Allogenic stem cell transplant - reserved for chemo failure and not suited to elderly patients
Splenectomy for refractory splenomegaly and ppancytopenia
185
Obstructive lymphadenopathy
Enlarged lymph nodes compress internal organs
186
4 complications of CLL
Obstructive lymphadenopathy
Transformation into aggressive large cell lymphoma
Autoimmune hemolytic anemia
Thrombocytopenia
187
Prognosis for CLL
Low risk - 10-15 years
Intermediate/High risk - 90% survive 2 years 70% survive 5 years
188
Acute myelogenous leukemia
Results from an arrest in the early development of myeloid precursors
Rapid proliferation of myeloblasts with no differentiation
189
Progeny of a myeloblast
Non-lymphocyte WBCs
(Granulocytes and Monocytes)
190
Pathogenesis of AML
Chromosomal translocation and other genetic mutations
191
Most common risk factor for AML and 3 other risk factors
Myelodysplastic syndrome
Down's syndrome
Environmental exposure
Chemotherapeutic agents
192
Median age of onset for AML
70 years
193
Auer rod
Eosinophilic, needle like inclusion in the cytoplasm of myeloblasts - confirmatory for AML
194
CBC findings for AML
Decreased RBC, Platelet, Neutrophil - WBC may be normal, high, or low
195
If you don't see any Auer rods in a peripheral smear, how else can you confirm AML
Using flow cytometry to differentiate between myeloid and lymphoid antigens on cell surfaces
196
How might a CMP be useful for cancer assessment
Look at Liver and Kidney function
197
How might blood cultures be useful in a cancer workup
look for signs of infection
198
How might a lactate dehydrogenase level be useful in a cancer workup
Shows increase in tissue destruction
199
What are we looking for in a bone marrow biopsy for AML
Predominant blasts
200
Lumbar puncture for AML
Only if there are symptoms - CNS infiltration is rare
201
Autologous stem cell transplant
Uses patients own cells removed and then replaced after therapy
202
Treatment for AML
Induction chemotherapy (induces remission in 80-90% of patients under 60 and 50-60% of patients over 60)
Post remission chemo or stem cell replacement (allogenic preferred)
203
Chronic Myeloid Leukemia (CML)
Dysregulated production and uncontrolled proliferation of mature and maturing granulocytes
204
Hallmark of CML
Translocation between 9 and 22 known as the philadelphia chromosome
205
Average age of onset for CML
55 years old, may be brought on by ionizing radiation exposure
206
3 phases of CML
Chronic phase (3-5 years) - WBCs differentiate (usually detected in this stage
Accelerated Phase
Terminal phase - Fatal blast crisis
207
5 clinical presentations of CML
Fatigue/weight loss
Low grade fever/night sweats
Hepatosplenomegaly
Bone tenderness
Allergy-like symptoms due to basophil overproduction (flushing, pruritis, etc.)
Symptoms get worse in accelerated phase
208
CBC findings for CML
Average white count with granulocytosis in the chronic phase and decrease in PLT and RBC with myeloblasts in the accelerated phase
209
Leukocyte Alkaline Phosphatase
Marker for WBC destruction
210
Leukocyte alkaline phosphatase level in CML
Low due to granulocyte resistance to Apoptosis
211
Symptoms of leukostasis
Usually neurologic symptoms
212
Percentage of blasts in the blast stage of CML
Over 20%
213
3 tissues that secrete alkaline phosphatase
Liver, Stomach and Bone
214
Test to identify philadelphia chromosome
PCR for bcr/abl DNA segment
215
Therapy for chronic phase CML
Single drug chemotherapy using a tyrosine kinase inhibitor - cancer cells are often "addicted" to this gene
216
Hematologic remission of CML
Often within 3 months
Normal CBC
217
Cytogenic remission of CML
Seen in 3-6 months, normal fromosome returns with less than 10% of cells testing positive for Philadelphia chromosome
218
Molecular remission of CML
Negative PCR for the bcr/abl mRNA
219
How long should therapy be continued for CML after molecular remission
2 years
220
Therapy for CML accelerated or blast phase
Tyrosine kinase inhibitor AND multidrug chemotherapy
Stem cell transplant may also be considered, especially if resistant to TKI
221
Multiple myeloma
A neoplastic proliferation of plasma cells that produce nonfunctional immunoglobulins
222
Preceding condition to multiple myeloma
MGUS - Monoclonal Gammopathy of Undetermined Significance - results from abnormal plasma cell response to antigenic stimulation
223
Epidemiology of Multiple Myeloma
Median onset is 65 years
Occurs more in men
occurs most in african americans
224
5 pathophysiologic aspects of Multiple Myeloma
Diminished hematopoiesis due to overgrowth of plasma cells
Lack of adequate response to infection because neoplastic plasma cells are monoclonal
Increase in Osteoclastic activity, bone tumor formation and hypercalcemia
Myeloma proteins are an antibody secreted by plasma cells that harms organs
Infiltration of tissues leads to plasmocytomas
225
3 skeletal presentations of multiple myeloma starting with MC
Bone pain in weight bearing back hips and ribs
Spinal cord compression
Pathologic fracture
226
RBC rouleaux formation
RBCs form into strings - a result of increased fibrin from multiple myeloma
227
4 Proteins we look for in MM and their significance
Paraprotein (M-protein) found via serum protein electrophoresis
Bence Jones protein found via 24 hout urine collection with urine protein electrophoresis
Low levels of non-myelomatous Ig
Beta-2 microglobulin DIRECTLY related to tumor burden
228
Imaging for MM (3)
X ray CT MRI
X-rays for pathologic fracture (Skull, spine, and long bones)
CT for neoplastic bone disease
Spine MRI for spinal nerve compression
229
Treatment for MM
Disease is uncurable but we can prolong life and improve symptoms
Triple agent chemotherapy
Stem cell transplant for young patients
Palliative, localized radtiation
230
Treatment for MM pathologic fractures
Stabilize the bone and irradiate the lesion
231
Treatment for MM vertebral body collapse
Vertebroplasty or Kyphoplasty - consult orthopedics
232
3 treatments for spinal cord compression in MM
IV steroids, Radiation, Consult neurosurgery
233
Prognosis for MM
median is 3 years to live - better for younger patients
234
What happens to lymph nodes as we age?
They grow until about 12 years and then begin to atrophy
235
Normal lymph node sizes in children for 3 nodes
Anterior cervical less than 2 cm
Axillary less than 1 cm
Inguinal less than 1.5 cm
236
Normal lymph node size in adults
Less than 1 cm at any location
237
5 characteristics of a lymph node that need to be documented
Size: 2 numbers (ie. 1.5x1)
Location: Examine all locations
Consistency: Hard, firm, rubbery, soft
Tenderness
Fixation: Is it mobile
238
What does a swollen NON tender lymph node indicate
Malignancy instead of infection
239
What do we mean if we say that lymph nodes are "matted"
They are stuck to each other
240
What do hard nodes indicate?
Fibrotic cancers
241
What do firm/rubbery nodes indicate?
Lymphomas/ chronic leukemia
242
What do softer nodes indicate
Acute leukemia or inflammation
243
Lymphadenopathy management in children
Clindamycin in high MRSA areas
Cefalexin or Augmentin in low MRSA areas
Add azithromycin for cat scratches
244
Lymphadenopathy management in children
Workup to rule out malignancy and refer for node biopsy if needed
245
Non-hodgkin lymphoma
Overgrowth of lymphocytes or their precursor in the lymphatic tissue - MC B cells 85%
246
5 Etiologies for Non-hodgkin lymphoma
Chromosomal translocations, Infection, Environmental factors, Immunodeficiency status, Chronic inflammation
247
Epidemiology of Non-Hodgkin lymphoma
Average age of onset 50 to 60 years old
Caucasians are most likely to get
Slightly more common in males
248
Two types of non-hodgkin clinical presentation
Indolent - Slow gorwing
Aggressive - Fast growing
249
Clinical presentation of indolent non-hodgkin lymphoma
Painless and slow growing lymphadenopathy, nodes may grow and then regress. Hepatosplenomegaly and cytopenias
250
B symptoms
Systemic symptoms seen with a cancer (A symptoms indicates a lack of systemic symptoms)
251
Clinical presentation of aggressive non-hodgkin lymphadenopathy (3)
Fast gowing and painless but may compress other structures such as the SVC. Systemic "B" symptoms also occur. Hepatosplenomegaly
252
CBC and peripheral smear findings for non hodgkin lymphoma
CBC normal until infiltration of the bone marrow causes pancytopenia
Smear normal
253
CMP and LDH levels with non-hodgkin lymphoma
Increased BUN/Creatinine with hydronephrosis
Increased LFT with hepatic involvement
Increased alkaline phosphatase with bone/liver involvement
Increased LDH with serious disease
254
Imaging for non-hodgkin lymphoma
CXR for mediastinal mass/nodes
CT with contrast to evaluate lymph node involvement
255
Diagnostic test for non-hodgkin lymphoma and its indication
Excisional lymph nod biopsy indicated by a node greater than 2.25cm squared or 2 cm in a single diameter
Biopsy of a peripheral node is preferred
256
Bone marrow biopsy for non-hodgkin lymphoma
Must be bilateral due to patchy nature of the disease
257
Three viruses we want to check for in non-hodgkin lymphoma
HIV, HCV, HBV
258
Where do we need to CT scan for non-hodgkin lymphoma
Neck to Peolvis so that we can see what areas are involved
259
Tests required to make an Ann arbor stage determination for non-hodgkin lymphoma
PET or CT of chest abdomen and pelvis and bilateral bone marrow biopsy
260
Ann arbor staging for Lymphoma
I - Only one tumor
II - More than one tumopr but all on the same side of the diaphragm
III - Tumors on both sides of the diaphragm
IV Disseminated tumors - an extralymphatic organ without the associated node
261
Letters used in ann arbor stage classifications
Represent organs and areas of the body - ie. E for extralymphatic site
B is for systemic symptoms
262
Treatment for indolent Non-hodgkin lymphoma
Incurable if disseminated at time of diagnosis, treatment consists of chemotherapy and is only recommended for symptomatic patients
263
Prognosis for indolent non-hodgkin lymphoma
10-15 years after diagnosis
264
Treatment for aggressive non-hodgkin lymphoma
Chemotherapy with or without local radiation therapy, stem cell transplant
265
4 poor prognostic factors for aggressive non-hodgkin lymphoma
Age over 60
Increased LDH
Poor response to standard therapy
Ann arbor stages 3 or 4
266
Hodgkin Lymphoma
A malignancy of B lymphocytes within the lymph tissue characterized by the presence of Reed-Sternberg cells
267
Reed Sternberg cells
Large abnormal lymphocytes that may contain more than one nucleus
268
Two viral triggers for Hodgkin Lymphoma
Epstein Barr Virus
HIV
269
2 Peaks for occurrence of hodgkin lymphoma
Gender and racial tendencies
Around 20 and around 50
More common in males, caucasians, and African Americans
270
Presentation of Hodgkin lymphoma
Painless swollen lymph node with migration to other nodes in a contiguous pattern
B symptoms - fever, night sweats, etc.
271
4 potential signs of Hodgkin lymphoma (not always present
Generalized pruritis
HSM
Mediastinal mass
Pain in swollen nodes with alcohol consumption
272
Diagnostic test for Hodgkin lymphoma
Lymph node excisional biopsy - look for Reed-Sternberg cells
273
Bulk
Lymph node over 10cm or mediastinal mass over 1/3 thoracic diameter
274
Treatment for hodgkin lymphoma
Multidrug chemotherapy with possible radiation for stages 1 and non-bulky 2
Consider high dose chemo and stem cell transplant for relapse
275
Prognosis and age risk factor cuttoff for Hodgkin lymphoma
Poor prognostics after age 45
90% survive ten years with I or non-bulky II
50-60% for later stages
276
Gene mutation leading to polycythemia vera
JAK2
277
Peak incidence age for polycythemia vera
50-70
278
Three main consequences of Polycythemia Vera
Increased blood viscosity
Pruritis from basophilia that gets worse after a warm shower of bath
Bleeding from platelet dyfunction
279
3 symptoms caused by increased blood viscosity
HA
Vertigo
Intermittent claudication
280
One thing thought to trigger polycythemia vera
ionizing radiation
281
Plethora
Reddish uneven complexion of the face, palms, nail beds and mucosa due to an excess of blood
282
Confirmatory tests for polycythemia vera
Erythropoietin level - should be low
Genetic testing for JAK2 mutation
283
Normal HCT for men and women with Polycythemia Vera
54% for males
51% for females
HCT will be high with very low EPO
284
mL in 1 unit of blood
500mL
285
Goal of therapeutic phlebotomy in polycythemia vera
Reduce HCT to under 45
286
How much does removal of 1 unit of blood reduce a patient's hematocrit
By 3%
287
3 lifestyle modifications for polycythemia vera patients
Stop smoking
Manage CV risk factors
Manage hypoxic conditions such as COPD
288
Treatment plan for polycythemia vera
Remove 1 unit of blood per week until the patient's HCT is below 45%
Administer aspirin if not contrindicated by blood loss
289
Iron replacement for polycythemia vera patients
DO NOT GIVE IRON
290
2 medications for polycythemia vera
Hydroxyurea - suppresses bone marrow contraindicated in pregnancy
Ruxolitinib - Suppresses JAK1/JAK2 - Indicated for failure of phlebotomy can cause myelofibrosis and splenomegaly
291
Low risk PV patients
Under 60 with no known hx of thromboembolism
292
Therapy for low risk PV patients
Phlebotomy, ASA, and lifestyle modifications - only use cytoreductive therapy if treatment is not successful or tolerated
293
Therapy for high risk PV patients
Phlebotomy, ASA, Lifestyle modifications, and Hydroxyurea
294
MC cause of death and 1 other potential complication from polycythemia vera
MCC of death = Thrombosis
Myelofibrosis is also a complication
295
Essential Thrombocytosis
Disorder of increased megakaryocyte production
296
3 genetic mutations that cause essential thrombocytosis
MC is JAK2
CALR - Calrectulin
MPL - Myoproliferative Leukemia virus oncogene
297
Average age for dx of essential thrombocytosis
50-60
298
5 symptoms of essential thrombocytosis
Thrombosis (DVT or Mesenteric)
Headache
Transient Ischemic attacks (dizziness etc.
Bleeding
Microvascular occlusion leading to pain
299
CBC and peripheral smear for ET
elevated PLT can be as much as 2million with large platelets
300
3 factors the increase the risk of thrombosis in ET
Over 60 years old
hx of thrombosis
JAK2 mutation
301
High risk ET
Hx of thrombosis and or over 60 with JAK2 mutation
302
Intermediate risk ET
Over 60 w/o JAK2 or Hx of thrombosis
303
Low risk ET
Under 60 with JAK2 and no thrombosis hx
304
Very low risk ET
Under sixty, no JAK2 and no hx of thrombosis
305
Management of low risk or very low risk ET
Observation and ASA 81mg daily - NO NSAIDs
306
Management of Intermediate and High risk ET
Administer Hydroxyurea with a target PLT of 100-400k
307
Prognosis for ET
Over 15 years with adequate treatment
308
4 potential etiologies for secondary erythrocytosis
Tissue hypoxia
Decreased renal perfusion
EPO secreting tumors
Testosterone administration
309
One symptom that differentiate secondary erythrocytosis from PV
No splenomegaly is present in SE
310
4 symptoms of potential secondary erythrocytosis
Low arterial oxygen
HA and lethargy
Clubbing of fingers
Ruddy complexion
311
Acrocyanosis
Hypoxia of the extremities turning them blue
312
Reactive thrombocytosis
An elevated PLT that is secondary to another disorder
313
2 main causes of increased megakaryocyte proliferation and maturation
Inflammatory cytokines or stimulation of RBC progenitors
314
3 conditions that can cause inflammatory cytokine release and therefore RT
Infection
Chronic inflammation
Malignancy
315
3 conditions that can cause stimulation of RBC progenitors
Hemorrhage, iron deficiency, hemolysis
316
3 main causes of RT
Increased megakaryocyte proliferation and maturation
Accelerated PLT release
Reduced platelet turnover from asplenia
317
3 symptoms each associated with a malignancy, inflammation, and bleeding
Inflammation - Pain, Swelling, Redness
Malignancy - Fever and weight loss, night sweats
Bleeding - Anemia, Fatigue, Visible blood
318
4 Lab workups for ET if you suspect an inflammatory condition
Erythrocyte sed rate
C reactive protein
Antinuclear antibody
Rheumatoid factor
