Test 3 Flashcards

1
Q

What is the most common age and gender for a myeloproliferative disorder to present?

A

70 yr old male

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2
Q

What is the prognosis for a person with MDS that transforms into a leukemia?

A

very poor

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3
Q

What can a MDS progress into?

A

acute leukemia

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4
Q

What blood problem can topoisomerase inhibitors create?

A

MDS

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5
Q

What are the three common presenting symptoms in a patient with 5q syndrome?

A

macrocytic anemia

leukopenia

low/normal platelets

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6
Q

How is 5q syndrome treated?

A

lenalidomide

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7
Q

What is CMML?

A

chronic myelomonocytic leukemia

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8
Q

What two drugs can be used to treat CMML?

A

azacytidine or imatinib

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9
Q

What two cell lines are almost always seen during CML?

A

basophils

eosinophils

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10
Q

What two labs are commonly high during CML?

A

LDH

uric acid

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11
Q

How can one tell that CML is transitioning into AML?

A

splenomegaly

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12
Q

What two drugs can be used in CML patients that have become resistant to imatinib?

A

dasatanib

nilotinib

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13
Q

What is the major side effect of nilotinib?

A

prolong QT

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14
Q

What is the most common mechanism of resistance to imatinib?

A

ABL mutations

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15
Q

What is the Hb diagnostic level for PV in men?

A

greater than 18.5

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16
Q

What is the Hb diagnostic level for PV in women?

A

greater than 16.5

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17
Q

Would serum EPO be high or low in PV?

A

low

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18
Q

What three drugs are used to treat PV?

A

hydroxyurea

interferon alpha

Anagrelide

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19
Q

What age and sex is most commonly presenting with Essential Thrombocytosis?

A

60 yr old, female

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20
Q

What two myeloproliferative diseases have very similar presenting symptoms?

A

essential thrombocytosis

polycythemia vera

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21
Q

What three drugs are used to treat Essential Thrombocytosis?

A

Hydroxyurea

Anagrelide

Interferon-alpha

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22
Q

What are the two common hallmarks of Primary Myelofibrosis?

A

marrow fibrosis

extramedullary hematopoiesis

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23
Q

What modalities are used to prolong life during primary myelofibrosis?

A

none

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24
Q

Does multiple myeloma effect men or women more often?

A

men

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25
Q

Does multiple myeloma effect AAs or caucasians more often?

A

AAs

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26
Q

What is the average age of mutliple myeloma Dx?

A

65

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27
Q

What are the two most common immunoglobulins to be produced during Multiple Myeloma? Which is more common?

A

IgA and IgG

IgG

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28
Q

What is the goal for stem cell transplants during Multiple Myeloma?

A

prolong life

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29
Q

What three drugs are used to treat multiple myeloma?

A

thalidomide

lenalidomide

carfilzomib

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30
Q

What is a plasmacytoma?

A

isolated malignant plasma cell tumor growing within axial skeleton or soft tissue

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31
Q

What is the most common site for a plasmacytoma?

A

upper respiratory tract

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32
Q

What is the mainstay of treatment for a plasmacytoma?

A

radiation

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33
Q

What is the stain for amyloidosis? What color does amyloidosis look like under fluorescent microscopy ?

A

congo red

red-green

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34
Q

What organ has the worst prognosis for the development of amyloidosis?

A

heart

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35
Q

Other than immunoglobulin, what is the main difference between mutliple myeloma and Waldenstroms Macroglobulinemia?

A

WM has greater than 10% lymphocytic involvement

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36
Q

What is the most common form of cancer in children under 14?

A

ALL

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37
Q

What is the most common form of cancer in children between 14 and 18?

A

Hodgkins Lymphoma

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38
Q

How hot and for how long does a fever indicative of Leukemia have to last for a child?

A

greater than 101F

longer than seven days

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39
Q

What are the two most common presentations of a childhood CNS tumor? Why?

A

vomiting and headaches

compression of CSF = increase ICP

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40
Q

How large must a lymph node from the epitrochlear region be to considered large in the epitrochlear region?

A

greater then 5 mm

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41
Q

How large must a lymph node from the inguinal region be to considered large?

A

greater than 15 mm

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42
Q

Adenopahy in what three regions is almost always considered abnormal?

A

epitrochlear, cervical, posterior auricular

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43
Q

Is ALL more common in AA children or white children?

A

caucasian

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44
Q

Is ALL more common in males or female children?

A

male

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45
Q

What is the peak age for ALL?

A

2-3 years old

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46
Q

In what child population is ALL most common?

A

hispanic

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47
Q

What are the two most common risk factors for the development of a child with ALL?

A

pre-natal Xrays

post-natal radiation

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48
Q

What two characteristics are the standard risk for ALL?

A

1-10 years old

WBCs below 50K

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49
Q

What two characteristics are the high risk for ALL?

A

below one or greater than ten

WBCs above 50K

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50
Q

By day 28, what level of lymphoblasts is wanted?

A

below 5%

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51
Q

What is the most common childhood brain tumor?

A

astrocytoma

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52
Q

What are astrocytomas associated with?

A

NF1

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53
Q

What is the etiology of LiFraumeni Syndrome?

A

p53 mutations

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54
Q

What are the four associated diseases that accompany Wilms Tumors?

A

Hemihypertrophy

Aniridia

Denys-Drash

Beckwith-Wiedemann

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55
Q

What type of tissue does a neuroblastoma develop from?

A

any neural crest tissue

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56
Q

How could a neuroblastoma in the cervical region develop?

A

Horner Sydrome

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57
Q

What protein is mutated in a neuroblastoma?

A

N-myc

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58
Q

What is the most common mesenchymal tissue in children?

A

Rhabdomyosarcoma

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59
Q

Are Ewing’s Sarcomas more common in male or females? What ages?

A

males

11-15

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60
Q

What are TH2 reactions?

A

antibody mediated

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61
Q

What are TH1 reactions?

A

cell mediated

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62
Q

What is the sex and age for AML presentation?

A

65 year old male

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63
Q

Is CML more common in males or females?

A

males

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64
Q

What is the age for the average onset of AML?

A

65 years old

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65
Q

Which form of myelegnous leukemia is known for infiltrating the skin?

A

AMML

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66
Q

Is organomegaly common in AML?

A

no

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67
Q

What two forms of AML have Auer rods?

A

M2 and M3

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68
Q

Which AML cellular subtype is known for invading the gingiva?

A

monocytic

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69
Q

What sex is more affected by mantle cell lymphoma? What age?

A

male

50-60

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70
Q

What GI organ does mantle cell lymphoma commonly effect?

A

small BOWEL

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71
Q

Does Mantle Cell lymphoma possess CD23?

A

no

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72
Q

What is the median age of deveopment for the sporadic form of Burkitts Lymphoma?

A

30

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73
Q

In the endemic form of Burkitts lymphoma, what are three common locations other than the jaw for cancer to develop?

A

ovaries, breast, kidneys

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74
Q

Does HTLV1 infect CD4 or CD8 cells?

A

CD4

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75
Q

What is the new antibody for HTLV1? What CD molecule does it target?

A

Alemtuzumab

CD52

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76
Q

What are two characteristics of the nucleus of cells infected with HTLV1?

A

folded

cerebriform

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77
Q

What is the difference between Sezary Syndrome and Mycoises Fungoides?

A

Sezary syndrome involves leukemia as well

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78
Q

What are two common presentation sites for T-cell leukemia?

A

Mediastinal mass

CNS issues

79
Q

According to Fang, what are the three markers for T-cell leukemia?

A

CD34, TdT, CD1a

80
Q

What is ALCL?

A

anaplastic large cell lymphoma

81
Q

Which marker has a poor prognosis in ALCL? Good Prognosis?

A

good = ALK1 positive

bad = ALK1 negative

82
Q

What is the shape of cells during ALCL?

A

kidney shaped

83
Q

What CD molecule can ALCL stain for?

A

CD30

84
Q

What can produce pain in lymph nodes with a B-cell lymphoma?

A

alcohol

85
Q

Beta 2 microglobulin is normally apart of what molecule?

A

MHC one

86
Q

In what two conditions can Beta 2 microglobulin be elevated?

A

multiple myoloma

lymphoma

87
Q

According to Heddinger, what is stage one?

A

one nodal region

88
Q

According to Heddinger, what is stage two?

A

two nodal regions on the same side of the diaphragm

89
Q

According to Heddinger, what is stage three?

A

both sides of diaphragm

90
Q

According to Heddinger, what is stage four?

A

extra-nodal site

91
Q

What is the most common type of Hodgkins Lymphoma?

A

nodular sclerosing

92
Q

What is the adverse prognostic number for albumin regarding Hodgkins Disease?

A

less than four

93
Q

What is the adverse prognostic number for age regarding Hodgkins Disease?

A

greater than 45

94
Q

What is the adverse prognostic number for hemoglobin regarding Hodgkins Disease?

A

less than 10.5

95
Q

What is the adverse prognostic number for WBC regarding Hodgkins Disease?

A

greater than 15,000

96
Q

What is the most common treatment for Hodgkins?

A

radiation

97
Q

What is more aggressive, B-cell or T-cell?

A

T-cell

98
Q

Which BCL molecule is present is Small lymphocytic leukemia?

A

BCL-3

99
Q

Which BCL molecule is present in diffuse large b-cell lymphoma?

A

Bcl-6

100
Q

Which BCL molecule is present during Mantle Cell Pymphoma?

A

bcl-1

101
Q

What are the three prognostic factors for DLCL?

A

stage III/IV

over 60

elevated LDH

102
Q

What is Richters Transformation?

A

Follicular lymphoma transforming into DLCL

103
Q

What two viruses are known to cause lymphoma in HIV patients?

A

HHV8

EBV

104
Q

Which leukemia present most like a lymphoma?

A

CLL

105
Q

What are the two most common signs of CLL on physical exam?

A

lymphoma

splenomegaly

106
Q

Which three immunoglobulins are known to be low during the late stages of CLL?

A

IgA/IgG/IgM

107
Q

CLL will be positive for which three CD molecules?

A

CD5/CD20/CD23

108
Q

Is CLL curable?

A

no

109
Q

What neutrophil anamoly can present during MDS?

A

pseudo-pelger huet

110
Q

What is Sweet’s Syndrome?

A

febrile neutrophilic dermatosis

111
Q

What type of patient most often presents with a 5q syndrome?

A

elderly female

112
Q

Other than RBCs, what two cell lines also increase in their numbers during PV?

A

WBCs and platelets

113
Q

What amino acids are mutated during a JAK2 mutation?

A

V617F

114
Q

What disease does a plasmacytoma often progress to?

A

multiple myeloma

115
Q

What organ is most commonly effected by amyloidosis?

A

kidney

116
Q

What organ gives the worst prognosis for amyloidosis?

A

heart

117
Q

What percent of lymphocytes have to be monoclonal to Dx Waldenstroms?

A

greater than 10%

118
Q

What type of bone pain is indicative of cancer in kids?

A

wakes them from sleep

119
Q

What is the most common solid tumor of childhood?

A

brain tumor

120
Q

What type of molecules do neuroblastomas secrete?

A

catecholamines

121
Q

Do osteosarcomas effect long bones or all bones?

A

long bones

122
Q

Does Ewing’s Sarcoma effect long bones or all bones?

A

all bones

123
Q

When are antibiotics used during AML therapy?

A

ablating bone marrow

124
Q

Which form of AML is most likely to produce DIC?

A

M3

125
Q

One will almost always find a large spleen during what malignancy?

A

CML

126
Q

How does a T-cell leukemia spread in the CNS?

A

via meninges

127
Q

What is considered higher risk, B-ALL or T-ALL?

A

T-ALL

128
Q

What two types of malignancies is EBV associated with?

A

Burkitts

Nasopharyngeal carcinoma

129
Q

What two proteins undergo fibrosis in the bone marrow during MDS?

A

collagen and fibrillin

130
Q

What drug can cause an MDS?

A

ethanol

131
Q

What is the MOA of anagrelide?

A

inhibits platelet production

132
Q

Does T-cell ALL effect adolescents or children more often?

A

adolescent

133
Q

Does T-cell ALL effect males or females more often?

A

males

134
Q

Is an astrocytoma a low-grade or high-grade tumor? What type of tumor?

A

low grade

glioma

135
Q

What type of brain tumor does not respond to radiation?

A

choroid plexus tumors

136
Q

Which ethnicity has a higher prevalence of Wilms Tumor?

A

AAs

137
Q

Which ethnicity has a lower prevalence of Wilms Tumor?

A

Asians

138
Q

What is the most common extra-cranial solid tumor in children?

A

neuroblastoma

139
Q

Are Ewings sarcomas amenable to radiation?

A

no

140
Q

Over the age of what is a poor prognosis for ALL?

A

35

141
Q

Longer than how many weeks for remission of ALL is a poor prognostic factor?

A

4 weeks

142
Q

Which lymphoma is known to involve the liver?

A

mantle cell

143
Q

According to Fang, where is the most common site for a sporadic Burkitt lymphoma to arise?

A

ileo-cecal region

144
Q

Where is the plaque phase of Sezary/Mycosis Fungoides located?

A

epidermis

145
Q

Where is the tumor phase of Sezary/Mycosis Fungoides located?

A

dermis

146
Q

Which leukemia or lymphoma displays a bimodal distribution of the ages it effects?

A

Hodgkins lymphoma

147
Q

What is the typical lymphocyte count of CLL?

A

greater than 5K

148
Q

What is seen during Stage 1 CLL?

A

lymphadenopathy

149
Q

What is seen during Stage 2 CLL?

A

splenomegaly

150
Q

What is seen during Stage 3 CLL?

A

Hb under 11

151
Q

What is seen during Stage 4 CLL?

A

platelets under 100K

152
Q

Which fungus can cause SVC syndrome?

A

Histoplasma capsulatum

153
Q

A total neutrophil count of less than what raises suspicion for neutropenic fever?

A

500

154
Q

What is the most common source of bacteria during neutropenic fever?

A

endogenous gut flora

155
Q

What type of organism is rarely the primary causative agent of neutropenic fever?

A

fungus

156
Q

Which two fungi are the most common to cause complications from neutropenic fever?

A

candida and aspergillus

157
Q

What organism is the most common cause of venous catheter infection?

A

Candida

158
Q

What is Lhermitte’s Sign?

A

pain down spine during neck flexion

159
Q

What are the three metabolic abnormalities seen during TLS?

A

hyperkalemia

hyperphosphatemia

hypocalcemia

160
Q

Would TLS produce metabolic acidosis or metabolic alkalosis?

A

acidosis

161
Q

Deposition of what electrolyte can cause arrhythmia problems?

A

phosphate

162
Q

Which two metabolites precipitate causing renal stones during TLS?

A

uric acid

calcium phosphate

163
Q

What are the three treatments for TLS?

A

hydration

allopurinol

Rasburicase

164
Q

What drug is used before the onset of TLS?

A

allopurinol

165
Q

What drug is used after the onset of TLS?

A

Rasburicase

166
Q

What rxn does Rasburicase catalyze?

A

uric acid to allantoin

167
Q

What is the best test for a PE?

A

CT Pulmonary Angiography

168
Q

What is the Tx for a hemodynamically stable patient having a PE?

A

warfarin

169
Q

What is the Tx for a hemodynamically unstable patient having a PE?

A

fibrinolytic

170
Q

What is the treatment of Waldenstroms Macroglobulinemia?

A

plasmapharesis

171
Q

What two types of drugs are known to cause a myelodysplastic syndrome?

A

alkylating agents

topoisomerase inhibitors

172
Q

According to Hughes, how do alkylating agents progress through a MDS?

A

MDS and then leukemia

173
Q

According to Hughes, how do topoisomerase inhibitors produce an MDS?

A

present as leukemia (AML)

174
Q

How are hypocellular MDS treated?

A

immunosuppressives

175
Q

What is the only chance at cure for an MDS?

A

bone marrow transplant

176
Q

In which Myeloproliferative Disorder is it common to see leukocytosis (> 100,000)?

A

CML

177
Q

When is anagrelide contraindicated ?

A

pregnancy

178
Q

In patients with a JAK2 mutated Essential Thrombocytosis, what molecule and cell type are increased?

A

Hb and neutrophils

179
Q

Which leukemia can present with arthralgia?

A

AML

180
Q

Does Burkitts respond will to therapy?

A

yes

181
Q

What are the three complications of radiation during Hodgkins Lymphoma?

A

lung cancer

breast cancer

heart disease

182
Q

Is NHL more common in men or women?

A

men

183
Q

What is the most causative agent of gram positive infections during neutropenic fever?

A

S. epidermidis

184
Q

Which gram-negaives are most likely to cause severe neutropenic fever?

A

E. coli and pseudomonas

185
Q

What virus is most likely to arise during neutropenia?

A

herpes

186
Q

Is the prognosis for 5q syndrome good or bad?

A

good

187
Q

Whick leukemia presents like a lymphoma?

A

CML

188
Q

What are the two associated viruses of Hodgkins Lympoma ?

A

EBV and HIV

189
Q

Can DLCL be cured? What therapy?

A

yes

CHOP

190
Q

A lymphocyte count greater than 5K should have a patient worked up for what?

A

CLL

191
Q

Which viral infection can produce an MDS?

A

HIV

192
Q

Which has a shorter latency period, topoisomerase inhibitor or alkylating agent?

A

topoisomerase inhibitor

193
Q

Does 5q Syndrome have a good or bad prognosis?

A

good

194
Q

A hypocellular MDS resembles what other disease?

A

aplastic anemia