Test 3 Flashcards
1
Q
What is the most common age and gender for a myeloproliferative disorder to present?
A
70 yr old male
2
Q
What is the prognosis for a person with MDS that transforms into a leukemia?
A
very poor
3
Q
What can a MDS progress into?
A
acute leukemia
4
Q
What blood problem can topoisomerase inhibitors create?
A
MDS
5
Q
What are the three common presenting symptoms in a patient with 5q syndrome?
A
macrocytic anemia
leukopenia
low/normal platelets
6
Q
How is 5q syndrome treated?
A
lenalidomide
7
Q
What is CMML?
A
chronic myelomonocytic leukemia
8
Q
What two drugs can be used to treat CMML?
A
azacytidine or imatinib
9
Q
What two cell lines are almost always seen during CML?
A
basophils
eosinophils
10
Q
What two labs are commonly high during CML?
A
LDH
uric acid
11
Q
How can one tell that CML is transitioning into AML?
A
splenomegaly
12
Q
What two drugs can be used in CML patients that have become resistant to imatinib?
A
dasatanib
nilotinib
13
Q
What is the major side effect of nilotinib?
A
prolong QT
14
Q
What is the most common mechanism of resistance to imatinib?
A
ABL mutations
15
Q
What is the Hb diagnostic level for PV in men?
A
greater than 18.5
16
Q
What is the Hb diagnostic level for PV in women?
A
greater than 16.5
17
Q
Would serum EPO be high or low in PV?
A
low
18
Q
What three drugs are used to treat PV?
A
hydroxyurea
interferon alpha
Anagrelide
19
Q
What age and sex is most commonly presenting with Essential Thrombocytosis?
A
60 yr old, female
20
Q
What two myeloproliferative diseases have very similar presenting symptoms?
A
essential thrombocytosis
polycythemia vera
21
Q
What three drugs are used to treat Essential Thrombocytosis?
A
Hydroxyurea
Anagrelide
Interferon-alpha
22
Q
What are the two common hallmarks of Primary Myelofibrosis?
A
marrow fibrosis
extramedullary hematopoiesis
23
Q
What modalities are used to prolong life during primary myelofibrosis?
A
none
24
Q
Does multiple myeloma effect men or women more often?
A
men
25
Does multiple myeloma effect AAs or caucasians more often?
AAs
26
What is the average age of mutliple myeloma Dx?
65
27
What are the two most common immunoglobulins to be produced during Multiple Myeloma? Which is more common?
IgA and IgG
IgG
28
What is the goal for stem cell transplants during Multiple Myeloma?
prolong life
29
What three drugs are used to treat multiple myeloma?
thalidomide
lenalidomide
carfilzomib
30
What is a plasmacytoma?
isolated malignant plasma cell tumor growing within axial skeleton or soft tissue
31
What is the most common site for a plasmacytoma?
upper respiratory tract
32
What is the mainstay of treatment for a plasmacytoma?
radiation
33
What is the stain for amyloidosis? What color does amyloidosis look like under fluorescent microscopy ?
congo red
red-green
34
What organ has the worst prognosis for the development of amyloidosis?
heart
35
Other than immunoglobulin, what is the main difference between mutliple myeloma and Waldenstroms Macroglobulinemia?
WM has greater than 10% lymphocytic involvement
36
What is the most common form of cancer in children under 14?
ALL
37
What is the most common form of cancer in children between 14 and 18?
Hodgkins Lymphoma
38
How hot and for how long does a fever indicative of Leukemia have to last for a child?
greater than 101F
longer than seven days
39
What are the two most common presentations of a childhood CNS tumor? Why?
vomiting and headaches
compression of CSF = increase ICP
40
How large must a lymph node from the epitrochlear region be to considered large in the epitrochlear region?
greater then 5 mm
41
How large must a lymph node from the inguinal region be to considered large?
greater than 15 mm
42
Adenopahy in what three regions is almost always considered abnormal?
epitrochlear, cervical, posterior auricular
43
Is ALL more common in AA children or white children?
caucasian
44
Is ALL more common in males or female children?
male
45
What is the peak age for ALL?
2-3 years old
46
In what child population is ALL most common?
hispanic
47
What are the two most common risk factors for the development of a child with ALL?
pre-natal Xrays
post-natal radiation
48
What two characteristics are the standard risk for ALL?
1-10 years old
WBCs below 50K
49
What two characteristics are the high risk for ALL?
below one or greater than ten
WBCs above 50K
50
By day 28, what level of lymphoblasts is wanted?
below 5%
51
What is the most common childhood brain tumor?
astrocytoma
52
What are astrocytomas associated with?
NF1
53
What is the etiology of LiFraumeni Syndrome?
p53 mutations
54
What are the four associated diseases that accompany Wilms Tumors?
Hemihypertrophy
Aniridia
Denys-Drash
Beckwith-Wiedemann
55
What type of tissue does a neuroblastoma develop from?
any neural crest tissue
56
How could a neuroblastoma in the cervical region develop?
Horner Sydrome
57
What protein is mutated in a neuroblastoma?
N-myc
58
What is the most common mesenchymal tissue in children?
Rhabdomyosarcoma
59
Are Ewing's Sarcomas more common in male or females? What ages?
males
11-15
60
What are TH2 reactions?
antibody mediated
61
What are TH1 reactions?
cell mediated
62
What is the sex and age for AML presentation?
65 year old male
63
Is CML more common in males or females?
males
64
What is the age for the average onset of AML?
65 years old
65
Which form of myelegnous leukemia is known for infiltrating the skin?
AMML
66
Is organomegaly common in AML?
no
67
What two forms of AML have Auer rods?
M2 and M3
68
Which AML cellular subtype is known for invading the gingiva?
monocytic
69
What sex is more affected by mantle cell lymphoma? What age?
male
50-60
70
What GI organ does mantle cell lymphoma commonly effect?
small BOWEL
71
Does Mantle Cell lymphoma possess CD23?
no
72
What is the median age of deveopment for the sporadic form of Burkitts Lymphoma?
30
73
In the endemic form of Burkitts lymphoma, what are three common locations other than the jaw for cancer to develop?
ovaries, breast, kidneys
74
Does HTLV1 infect CD4 or CD8 cells?
CD4
75
What is the new antibody for HTLV1? What CD molecule does it target?
Alemtuzumab
CD52
76
What are two characteristics of the nucleus of cells infected with HTLV1?
folded
cerebriform
77
What is the difference between Sezary Syndrome and Mycoises Fungoides?
Sezary syndrome involves leukemia as well
78
What are two common presentation sites for T-cell leukemia?
Mediastinal mass
CNS issues
79
According to Fang, what are the three markers for T-cell leukemia?
CD34, TdT, CD1a
80
What is ALCL?
anaplastic large cell lymphoma
81
Which marker has a poor prognosis in ALCL? Good Prognosis?
good = ALK1 positive
bad = ALK1 negative
82
What is the shape of cells during ALCL?
kidney shaped
83
What CD molecule can ALCL stain for?
CD30
84
What can produce pain in lymph nodes with a B-cell lymphoma?
alcohol
85
Beta 2 microglobulin is normally apart of what molecule?
MHC one
86
In what two conditions can Beta 2 microglobulin be elevated?
multiple myoloma
lymphoma
87
According to Heddinger, what is stage one?
one nodal region
88
According to Heddinger, what is stage two?
two nodal regions on the same side of the diaphragm
89
According to Heddinger, what is stage three?
both sides of diaphragm
90
According to Heddinger, what is stage four?
extra-nodal site
91
What is the most common type of Hodgkins Lymphoma?
nodular sclerosing
92
What is the adverse prognostic number for albumin regarding Hodgkins Disease?
less than four
93
What is the adverse prognostic number for age regarding Hodgkins Disease?
greater than 45
94
What is the adverse prognostic number for hemoglobin regarding Hodgkins Disease?
less than 10.5
95
What is the adverse prognostic number for WBC regarding Hodgkins Disease?
greater than 15,000
96
What is the most common treatment for Hodgkins?
radiation
97
What is more aggressive, B-cell or T-cell?
T-cell
98
Which BCL molecule is present is Small lymphocytic leukemia?
BCL-3
99
Which BCL molecule is present in diffuse large b-cell lymphoma?
Bcl-6
100
Which BCL molecule is present during Mantle Cell Pymphoma?
bcl-1
101
What are the three prognostic factors for DLCL?
stage III/IV
over 60
elevated LDH
102
What is Richters Transformation?
Follicular lymphoma transforming into DLCL
103
What two viruses are known to cause lymphoma in HIV patients?
HHV8
EBV
104
Which leukemia present most like a lymphoma?
CLL
105
What are the two most common signs of CLL on physical exam?
lymphoma
splenomegaly
106
Which three immunoglobulins are known to be low during the late stages of CLL?
IgA/IgG/IgM
107
CLL will be positive for which three CD molecules?
CD5/CD20/CD23
108
Is CLL curable?
no
109
What neutrophil anamoly can present during MDS?
pseudo-pelger huet
110
What is Sweet's Syndrome?
febrile neutrophilic dermatosis
111
What type of patient most often presents with a 5q syndrome?
elderly female
112
Other than RBCs, what two cell lines also increase in their numbers during PV?
WBCs and platelets
113
What amino acids are mutated during a JAK2 mutation?
V617F
114
What disease does a plasmacytoma often progress to?
multiple myeloma
115
What organ is most commonly effected by amyloidosis?
kidney
116
What organ gives the worst prognosis for amyloidosis?
heart
117
What percent of lymphocytes have to be monoclonal to Dx Waldenstroms?
greater than 10%
118
What type of bone pain is indicative of cancer in kids?
wakes them from sleep
119
What is the most common solid tumor of childhood?
brain tumor
120
What type of molecules do neuroblastomas secrete?
catecholamines
121
Do osteosarcomas effect long bones or all bones?
long bones
122
Does Ewing's Sarcoma effect long bones or all bones?
all bones
123
When are antibiotics used during AML therapy?
ablating bone marrow
124
Which form of AML is most likely to produce DIC?
M3
125
One will almost always find a large spleen during what malignancy?
CML
126
How does a T-cell leukemia spread in the CNS?
via meninges
127
What is considered higher risk, B-ALL or T-ALL?
T-ALL
128
What two types of malignancies is EBV associated with?
Burkitts
Nasopharyngeal carcinoma
129
What two proteins undergo fibrosis in the bone marrow during MDS?
collagen and fibrillin
130
What drug can cause an MDS?
ethanol
131
What is the MOA of anagrelide?
inhibits platelet production
132
Does T-cell ALL effect adolescents or children more often?
adolescent
133
Does T-cell ALL effect males or females more often?
males
134
Is an astrocytoma a low-grade or high-grade tumor? What type of tumor?
low grade
glioma
135
What type of brain tumor does not respond to radiation?
choroid plexus tumors
136
Which ethnicity has a higher prevalence of Wilms Tumor?
AAs
137
Which ethnicity has a lower prevalence of Wilms Tumor?
Asians
138
What is the most common extra-cranial solid tumor in children?
neuroblastoma
139
Are Ewings sarcomas amenable to radiation?
no
140
Over the age of what is a poor prognosis for ALL?
35
141
Longer than how many weeks for remission of ALL is a poor prognostic factor?
4 weeks
142
Which lymphoma is known to involve the liver?
mantle cell
143
According to Fang, where is the most common site for a sporadic Burkitt lymphoma to arise?
ileo-cecal region
144
Where is the plaque phase of Sezary/Mycosis Fungoides located?
epidermis
145
Where is the tumor phase of Sezary/Mycosis Fungoides located?
dermis
146
Which leukemia or lymphoma displays a bimodal distribution of the ages it effects?
Hodgkins lymphoma
147
What is the typical lymphocyte count of CLL?
greater than 5K
148
What is seen during Stage 1 CLL?
lymphadenopathy
149
What is seen during Stage 2 CLL?
splenomegaly
150
What is seen during Stage 3 CLL?
Hb under 11
151
What is seen during Stage 4 CLL?
platelets under 100K
152
Which fungus can cause SVC syndrome?
Histoplasma capsulatum
153
A total neutrophil count of less than what raises suspicion for neutropenic fever?
500
154
What is the most common source of bacteria during neutropenic fever?
endogenous gut flora
155
What type of organism is rarely the primary causative agent of neutropenic fever?
fungus
156
Which two fungi are the most common to cause complications from neutropenic fever?
candida and aspergillus
157
What organism is the most common cause of venous catheter infection?
Candida
158
What is Lhermitte's Sign?
pain down spine during neck flexion
159
What are the three metabolic abnormalities seen during TLS?
hyperkalemia
hyperphosphatemia
hypocalcemia
160
Would TLS produce metabolic acidosis or metabolic alkalosis?
acidosis
161
Deposition of what electrolyte can cause arrhythmia problems?
phosphate
162
Which two metabolites precipitate causing renal stones during TLS?
uric acid
calcium phosphate
163
What are the three treatments for TLS?
hydration
allopurinol
Rasburicase
164
What drug is used before the onset of TLS?
allopurinol
165
What drug is used after the onset of TLS?
Rasburicase
166
What rxn does Rasburicase catalyze?
uric acid to allantoin
167
What is the best test for a PE?
CT Pulmonary Angiography
168
What is the Tx for a hemodynamically stable patient having a PE?
warfarin
169
What is the Tx for a hemodynamically unstable patient having a PE?
fibrinolytic
170
What is the treatment of Waldenstroms Macroglobulinemia?
plasmapharesis
171
What two types of drugs are known to cause a myelodysplastic syndrome?
alkylating agents
topoisomerase inhibitors
172
According to Hughes, how do alkylating agents progress through a MDS?
MDS and then leukemia
173
According to Hughes, how do topoisomerase inhibitors produce an MDS?
present as leukemia (AML)
174
How are hypocellular MDS treated?
immunosuppressives
175
What is the only chance at cure for an MDS?
bone marrow transplant
176
In which Myeloproliferative Disorder is it common to see leukocytosis (> 100,000)?
CML
177
When is anagrelide contraindicated ?
pregnancy
178
In patients with a JAK2 mutated Essential Thrombocytosis, what molecule and cell type are increased?
Hb and neutrophils
179
Which leukemia can present with arthralgia?
AML
180
Does Burkitts respond will to therapy?
yes
181
What are the three complications of radiation during Hodgkins Lymphoma?
lung cancer
breast cancer
heart disease
182
Is NHL more common in men or women?
men
183
What is the most causative agent of gram positive infections during neutropenic fever?
S. epidermidis
184
Which gram-negaives are most likely to cause severe neutropenic fever?
E. coli and pseudomonas
185
What virus is most likely to arise during neutropenia?
herpes
186
Is the prognosis for 5q syndrome good or bad?
good
187
Whick leukemia presents like a lymphoma?
CML
188
What are the two associated viruses of Hodgkins Lympoma ?
EBV and HIV
189
Can DLCL be cured? What therapy?
yes
CHOP
190
A lymphocyte count greater than 5K should have a patient worked up for what?
CLL
191
Which viral infection can produce an MDS?
HIV
192
Which has a shorter latency period, topoisomerase inhibitor or alkylating agent?
topoisomerase inhibitor
193
Does 5q Syndrome have a good or bad prognosis?
good
194
A hypocellular MDS resembles what other disease?
aplastic anemia
