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MCC > Quiz 2 > Flashcards

Quiz 2 Flashcards

1
Q

What is Gout?

A

The deposition of MSU crystals in tissues (joints especially) due to hyperuricemia

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2
Q

What is the function of CD antigens?

A

They are the primay cell surface molecules used to determine cell lineage

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3
Q

What is the immunophenotype expressed by lymphocytes?

A

The pattern of expressed CD antigens

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4
Q

What is the T-cell lineage marker?

A

CD3

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5
Q

What is the B-cell lineage marker?

A

CD19

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6
Q

What is the mature B cell marker?

A

CD20

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7
Q

What is the immaturity marker of a cell?

A

CD34

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8
Q

What antigens are responsible for assessment of B-cell clonality?

A

Kappa and Lambda light chains

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9
Q

What antigen acts as a myeloid lineage marker?

A

MPO

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10
Q

What antigen acts as an immature lymphocyte marker?

A

TdT

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11
Q

What type of lymphoma is most likely indicated by a t(14;18) IGH-BCL2 translocation?

A

a follicular lymphoma

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12
Q

What type of lymphoma is most likely indicated by a t(11;14) CCND1-IGH translocation?

A

a mantle cell lymphoma

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13
Q

What type of lymphoma is most likely indicated by a t(8;14) MYC-IGH translocation?

A

Burkitt lymphoma

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14
Q

What test could be run for more information on the granulation status of neutrophils?

A

morphologic WBC analysis

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15
Q

In RNA/DNA catabolism, loss of Adenosine Deaminase (ADA) leads to shutting down of what enzyme?

A

Ribonucleotide Reductase (RNR)

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16
Q

Ribonucleotide Reductase (RNR) is required for synthesis of which DNA precursors?

A

All dNTP synthesis requires RNR

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17
Q

DNA catalysis requires stable free radicals scavenged by which molecule?

A

hydroxyurea

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18
Q

What is the role of an E1 ubiquitin ligase?

A

To activate multiple E2 ubiquitin ligases

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19
Q

What is the role of E2 ubiquitin ligases?

A

To hold ubiquitin (bullet) for the target protein

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20
Q

What is the role of E3 ubiquitin ligases?

A

To form a complex with E2 and identify the target protein for ubiquitination

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21
Q

What is the difference between monoubiquitylation and polyubiquitylation of a target protein?

A

M- changes target protein activity
P- leads to degradation through proteasome

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22
Q

What are dohle bodies a sign of in PMNs?

A

activation in response to inflammation

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23
Q

What are aquired causes of neutrophilia?

A
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24
Q

What are the 5 main causes of neutrophilia?

A

Infection
Medications (corticosteroids)
Stress/trauma w/ necrosis
Chron. inflam. or autoimmune (rh. arthritis)
neoplasms

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25
Q

Would reactive neutrophilia or CML be more likely in a patient with an LAP score of over 115?

A

Reactive neutrophilia

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26
Q

Would a CBC revealing elevated eosinophils and basophils with absolute basophilia be more indicative of reactive neutrophilia or CML?

A

CML

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27
Q

Which translocation would be expected in diagnosis of CML?

A

t(9;22) BCR/ABL

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28
Q

What are the 5 main causes of lymphocytosis?

A

Infection (viral most common)
autoimmune disease
smoking (polyclonal B-cell lymphocytosis)
Chronic lymphocytic leukemia (CLL)
Other non-Hodgkin lymphomas

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29
Q

What treatment is preferred for CML?

A

tyrosine kinase inhibitors

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30
Q

What lab testing would be preferred for reactive lymphocytosis?

A

infectious serology

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31
Q

What lab testing would be preferred for mononucleosis?

A

EBV, monospot, serology, PCR

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32
Q

What signs in the peripheral blood smear might indicate infectious mononucleosis?

A

Downey cells, or variant lymphocytes

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33
Q

What clinical signs might suggest mononucleosis?

A

fever, pharyngitis, cervical lympadenopathy (LAD)

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34
Q

What is the most likely clinical presenation for a pt with undiagnosed CML or CLL?

A

asymptomatic

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35
Q

An elderly patient with an elevated WBC count of 40,000 with monomorphous mature lymphocytes, atypical monoclonal B-cells, and + B-cell clonality is most likely to have reactive lymphocytosis or CLL?

A

CLL

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36
Q

What are the 5 most common causes of Eosinophilia?

A

Infection (parasitic or fungal most likely)
Allergic diseases
rheumatologic diseases
Drug rxns
neoplasm

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37
Q

Prolonged elevations of eosinophil counts above what level are usually injurious, and can lead to cardiac fibrosis?

A

5000u/L

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38
Q

Patients with hypereosinophilia of uncertain cause can be treated with what medication?

A

hydroxyurea

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39
Q

What are the most common causes of neutropenia?

A

Genetic: Kostmann syndrome, idiopathic
Acquired: Infection, antivirals or antipsychotics, autoimmune disorders, aplastic anemia, neoplasms

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40
Q

What are possible causes of neutrophil hyposegmentation?

A

Pelger-Huet anomaly, MDS, medications (tacrolimus)

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41
Q

What are possible causes of neutrophil hypersegmentation?

A

Vit B12/Folate deficiency, MDS, medications

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42
Q

What pathology would be indicated by a FISH finding of a t(9;22) BCR-ABL fusion?

A

Chronic myeloid leukemia

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43
Q

What is the pathology of Pelger-Huet anomaly?

A

It is a benign manifestation of hyposegmented neutrophils

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44
Q

T/F: A lymphoma can include immature lymphoid cells

A

False

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45
Q

T/F: A leukemia can be lymphoid or myeloid

A

True

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46
Q

What type of neoplasms are clonal disorders?

A

Lymphoid neoplasms

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47
Q

How are immunoglobulins expressed by B-cells measured?

A

flow cytometry or PCR

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48
Q

What are the most notable B-cell lymphomas?

A

CLL/SLL
Mantle cell lymphoma
Follicular lymphoma
DLBC lymphoma
Burkitt lymphoma
Hodgkin lymphoma

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49
Q

Which of the common B-cell lymphomas are considered high-grade, aggressive? What characterizes them?

A

Mantle cell lymphoma, DLBCL, Burkitt lymphoma

They affect adults, children, immunocompromised pts, usually present in stage Ib/IIb, grow rapidly and need immediate treatment, but generally curable

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50
Q

Which of the common B-cell lymphomas are considered low-grade, indolent? What characterizes them?

A

CLL/SLL, follicular lymphoma (can also be high-grade)

Pts commonly present in stage III/IV, usually in elderly (50-70 median age), generally incurable, but slow growth and long survival

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51
Q

Which B-cell lymphoma can have translocations involving MYC, BCL2, or BCL6

A

DLBCL

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52
Q

Which lymphoma is characterized by a “starry sky” appearance of lymph node histology?

A

Burkitt lymphoma

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53
Q

In cases of HIT, what medication may be indicated for a pt to switch to?

A

Argatroban is the IIa inhibitor indicated in cases of HIT

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54
Q

What are the symptoms of multiple myeloma?

A

CRAB symptoms:
hyper C alcemia
Renal failure (elevated creatinine)
Anemia
Bone pain

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55
Q

What is the Rouleaux formation as seen on a blood smear?

A

“Coin stacks” of RBCs

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56
Q

What do Auer rods indicate on a blood smear?

A

a cell of myeloid lineage

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57
Q

What pathology is associated with a t(15;17) PML-RARA translocation?

A

acute promyelocytic leukemia

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58
Q

Which leukemia is most associated with DIC and considered an acute hematological emergency?

A

AML t(15;17) PML-RARA (acute promyelocytic leukemia)

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59
Q

What treatments are used for acute promyelocytic leukemia?

A

ATRA (all-transretinoic acid) and arsenic trioxide

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60
Q

What is the significant finding on the bone marrow aspirate smear?

A

numerous promyelocytes

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61
Q

What 4 findings are most consistant with DIC?

A

elevated PT
elevated aPTT
decreased fibrinogen
elevated D- dimer

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62
Q

What is the biggest risk with DIC?

A

hemorrhage

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63
Q

What two types of AML are referred to as “CBF leukemias”?

A

AML t(8;21) RUNX1-RUNX1T1
AML t(16;16) CBFB-MYH11

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64
Q

What is a common presentation of acute B-lymphoblastic leukemia (B-ALL) with hyperdiploidy?

A

A high number of triploid or quadriploid chromsomes
Often presents with cytopenias and bone pain

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65
Q

What are the four Ts for ddx for a mediastinal mass?

A

“Terrible” lymphoma or leukemia
Thymic tumor
“Teratoma” or GC tumor
Thyroid tumor

66
Q

Which type of cancer often presents as a mediastinal mass with/without BM involvement, more commonly in adolescents?

A

T-ALL (leukemia/ or lymphoma)

67
Q

Which neoplasms fall into the group of acute myeloid neoplasms?

A
68
Q

Which neoplasms fall into the group of acute lymphoid neoplasms

A
69
Q

Which neoplasms fall int othe group of MPN-MDS?

A
70
Q

Which neoplasms fall in to the group of mature lymphoid neoplasms?

A
71
Q

What does a blast quantity of over 20% in peripheral blood indicate?

A

an acute leukemia

72
Q

How can gene fusion be detected?

A

Chromosome analysis (karyotype), FISH, or molecular testing

73
Q

What is considered a major molecular response in treatment of CML?

A

1,000 fold or a 3 log reduction in BCR-ABL1 transcripts

74
Q

What chiefly characterizes Polycythemia vera?

A

-Exclusion of secondary causes of increased RBC mass
-Low serum EPO
-usually a JAK2V617F mutation
-usually hyperceullar BM with panmyelosis

75
Q

What are three principal treatments of PV?

A

Phlebotomy, JAK inhibitors, hydroxyurea

76
Q

Which conditions have a bimodal distribution in average age at dx?

A

Hodgkin’s lymphoma, ET,…

77
Q

What characterizes essential thrombocythemia?

A

-Platelets >450K/uL
-atypical megakaryocyte hyperplasia in BM
-JAK2, CALR or MPL mutation
-must exclude secondary thrombocytosis

78
Q

What is notable about this blood smear?

A

thrombocytosis

79
Q

What are characteristics of myelofibrosis?

A

-Increased megakaryocytes and granulocytes in BM
-Fibrous CT in BM
-Leukoerythroblastic smear- teardrop cells, nucleated RBCs, left shift
-JAK2, CALR, MPL mutation
-extramedullary hematopoeisis

80
Q

What are treatments for primary myelofibrosis?

A

JAK inhibitors, hydroxyurea, spenectomy (if massive), and SC transplant if progressed

81
Q

Of PV, ET, and PMF, which has the poorest prognosis?

A

PMF

82
Q

What are major similarities between PV, ET, and PMF?

A

-Peripheral blood thrombocytes
-Gene mutations in JAK2, MPL, or CALR
- BM abnormalities

83
Q

What characertizes MDS?

A

-Ineffective hematopoeisis
-High risk of developing AML
-Abnormal cells remain in BM, causing peripheral cytopenias

84
Q

What three features are required for a dx of MDS?

A
  1. Cytopenia
  2. Morphologic dysplasia (under microscope)
  3. Abnormal cytogenetic/chromsome studies
85
Q

What treatments are used for MDS?

A

Limited options; but in younger patients, allogeneic SC transplant, maybe DNA methyltransferaseinhibitors (Azacitidine)

86
Q

In what category do MGUS, smoldering myeloma and multiple myeloma fall?

A

Plasma cell neoplasms

87
Q

Which anticoagulants are contraindicated in pregnancy?

A

Warfarin and DOACs

88
Q

Where is DVT most likely to occur?

A

in the legs, usually the calves

89
Q

What are the current treatments for DVT?

A

Direct Xa inhibitor
LMWH
Thrombin inhibitor (LMWH bridging)
Warfarin (LMWH briding)

90
Q

How long is DVT usually treated?

A

3 months, but could be more depending on situation

91
Q

What is a good treatment for DVT if bleeding is a problem? Why?

A

Unfractionated heparin; it is quickly/completely reversible with protamine

92
Q

How is PE treated differently than DVR?

A

It is currently treated the same

93
Q

Which is the pathology of heparin-induced thrombocytopenia?

A

Antibodies in the pt. bind to the heparin-PF4 complex

94
Q

In the event of HIT after heparin administration, what would be the best course of action?

A

STOP heparin treatment, and administer a thrombin inhibitor (argatroban is preferred for HIT)

95
Q

Which anticoagulants have been used most consistantly in cancer patients? Are there any other types that are becoming more commonly used?

A

LMWHs (dalteparin, enoxaparin, tinzaparin); DOACs (rivaroxaban, apixaban, edoxaban, dibigatran)

96
Q

Of the following reversal protocols for Warfarin administration, which has the longest time of action?: PT complex concentrate, FFP, Vit K

A

Vit K

97
Q

What are the most common B-cell neoplasms in adults?

A

Follicular lymphoma and DLBCL

98
Q

What is the most common leukemia in adults in the Western hemisphere?

A

SLL/CLL

99
Q

What is the main difference between CLL and SLL?

A

CLL: leukemic form
SLL: in node form

100
Q

What are the approximate ratios of B-cell NHL, Hodgkin lymphoma, and T-cell NHL in the population?

A

B-cell NHL: 80%
Hodgkin lymphoma: 11%
T-cell NHL: 9%

101
Q

What are the main differences between low grade/indolent and high grade/aggressive lymphomas?

A

LG/I: older pt, not curable, high stage
HG/A: younger pt, curable, low stage, B symptoms

102
Q

What type of lymphoma is indicated by this biopsy? Why?

A

DLBCL; the lymphocytes are atypical and large (1.5-2x normal lymphocyte)

103
Q

What type of lymphoma is indicated by this biopsy? why?

A

Hodgkin lymphoma; the classic “owl eye” cells are Reed Sternberg cells

104
Q

What type of lymphoma is indicated by this biopsy? Why?

A

Burkitt lymphoma; the classic “starrry sky” appearance of the lymph node (increase in tingible body macrophages)

105
Q

What type of lymphoma is indicated by this biopsy? Why?

A

Follicular lymphoma; low power view of back-to-back follicles

106
Q

What translocation is associated with Burkitt lymphoma? What are other notable features?

A

t(8;14)

107
Q

What translocation is associated with CML (required for CML dx, but also possible in ALL or AML)? What are other notable features

A

t(9;22)

108
Q

What translocation is associated with Follicular lymphoma? What are other notable features?

A

t(14;18)

109
Q

What translocation is associated with Acute promyelocytic leukemia? What are other notable features?

A

t(15;17)

110
Q

What featuers are likely in CML, but NOT in reactive neutrophilia?

A

Absent toxic changes in neuotrphils, absolute basophilia, splenomegaly, low LAP score, BCR-ABL1 fusion t(9;22)

111
Q

What is the difference between average age at dx for AML and ALL?

A

AML: mostly adults
ALL: mostly kids

112
Q

What is the liklihood of splenomegaly in leukemia? In MDS? In aplastic anemia?

A

Leukemia: Common
MDS: less common
aplastic anemia: not common

113
Q

What is a major differentiator between MDS and aplastic anemia?

A

MDS will have a hyper-cellular marrow

114
Q

What is the criteria for monoclonal gammopathy of undetermined significance (MGUS)?

A
115
Q

What is the criteria for multiple myeloma?

A
116
Q

What is the criteria for smoldering myeloma?

A
117
Q

A small, round, blue cell tumor is representative of what specific kind?

A

a pediatric tumor

118
Q

What is the clinical use of Rituximab?

A

Immunotherapy; a monoclonal antibody used to treat CLL

119
Q

What does the American Society for Hematology recommend in work-up for a plasma cell neoplasm?

A

1- CBC w/ diff, Ca, Cr, UA
2- SPEP for M-spike
3- Immunofixation to confirm Ig
4- Imaging and BM biopsy

120
Q

What does an M-protein or M-spike look like on SPEP compared to normal?

A
121
Q

What is another name for multiple myeloma?

A

plasma cell myeloma

122
Q

In what type of of plasma cell myelomas is rouleax seen?

A

neoplasms with monoclonal proteins

123
Q

In what age range would you expect to see ALL and AML respectively?

A

ALL: mostly in children and young adults
AML: ages 15-39

124
Q

In what age range would you expect to see CML and CLL respectively?

A

CML: between 30-60 yo
CLL: over 50 yo

125
Q

What type of test is a monospot test?

A

A serologic test for mononucleosis

126
Q

What is the clinical indication of imatinib?

A

It is a popular tyrosine kinase inhibitor

127
Q

What are two advantages of using FISH over PCR?

A

FISH is quicker
Allows detection in an intact cycle

128
Q

Lack of HGPRTase is responsible for what disorder?

A

Lesch-Nyhan syndrome

129
Q

What is the action of HGPRTase?

A

To salvage hypoxanthine, or guanine by attaching it to PRPP

130
Q

What is a key symptom of Lesch Nyhan syndrome?

A

Uncontrollable lip/tongue biting, finger biting, or head banging

131
Q

What is inhibited by allopurinol? What is the target molecule’s function?

A

Xanthine oxidase; it is a purine oxidizing enzyme that makes urate

132
Q

On what kinds of cells is Ki-67 expressed?

A

cells actively cycling (NOT in G0)

133
Q

Is PRPP used in the de novo or salvage pathways for purine biosynthesis?

A

It is required for both

134
Q

ATM is a cell cycle “checkpoint” protein that is specific to what stage?

A

It is S-phase specific (blocks entry to S-phase

135
Q

The improvements from around a 90% mortality rate to less than 10% of ALL in children is mostly due to what?

A

Optimal combinations and timing of chemotherapy

136
Q

How would a Hodgkin lymphoma in stage Ia or IIa be treated?

A

chemotherapy plus radiation therapy

137
Q

What is the therapeutic INR for most patients on warfarin?

A

2.0-3.0

138
Q

What test was done to produce these results?

A

a gene expression microarray

139
Q

What useful information about cancer can be provided by a gene expression microarray?

A

A GE microarray reveals abundance of RNA transcripts in cells tested; this can be correllated with expression/activity of proteins, and can provide information on the class or subtype of cancer present

140
Q

What is the first step after finding lymphocytosis in a CBC?

A

Determining if the lymphocytes are derived from a single clone (leukemia/lymphoma) or have many variants (leukemoid reaction)

141
Q

What is the result of a t(14;18) BCL-2 translocation?

A

Follicular lymphoma, more specifically, a mutation causing overexpression of BCL-2, an oncogene that inhibits apoptosis

142
Q

Why is a protein electrophoresis of blood and urine an appropriate test to dx multiple myeloma?

A

In MM, an expanded clonal population of plasma cells secretes large amounts of intact (found in blood) Ig, or Ig light chains (Bence-Jones proteins) found in the urine

143
Q

What type of leukemia is princibly treated with a tyrosine kinase inhibitor (iminitab)?

A

CML

144
Q

When should a bone marrow biopsy be performed?

A

When the etiology of an abnormal peripheral blood count is uncertain or if a hematologic malignancy is expected

145
Q

What is indicated by a high or low INR, respectively?

A

High- slow forming clots
Low- fast forming clots

146
Q

What is a clinical sign that warrants inclusion of Follicular lymphoma on the ddx?

A

Painless lympoh nodes that wax and wane (usually in adults)

147
Q

What are bone pain, proteinurea, and Rouleaux formation all signs of?

A

Multiple Myeloma (plasma cell myeloma)

148
Q

What is the first step in treating a patient with an embolism causing an acute MI?

A

Dissolve the clot

149
Q

What are major signs of TTP?

A

Pupura in extremities, schistocytes, low platelets, and elevated fibrinogen. Testing will reveal levels <10 of ADAMTS13

150
Q

How does a COX1/2 inhibitor like aspirin impair platelet aggregation?

A

Inhibition of COX 1 and 2 results in decreased TxA2 production, causing platelets to not express GPIIb/IIa and fail to aggregate

151
Q

What is the primary characterization of MDS vs PMF?

A

MDS: ineffective hematiopoiesis -> pancytopenia
PMF: deposition of megakaryocytes and granulocytes in BM -> marrow fibrosis

152
Q

When is adjuvant chemotherapy given?

A

After surgery to reduce local and distant recurrence

153
Q

When is neoadjuvant chemotherapy given?

A

Before surgery to improve outcomes

154
Q

When is inducion chemotherapy given?

A

to achieve remission of cancer

155
Q

When is consolidation chemotherapy given?

A

After induction to treat microscopic disease

156
Q

When is maintenance chemotherapy given?

A

Over long-term to maintain remission

157
Q

When is salvage chemotherapy given?

A

After relapse or refractory to previous therapy

158
Q

What is the most common B-cell neoplasm in adults?

A

follicular lymphoma
t(14;18) IGH/BCL-2

159
Q

What is the most common leukemia in adults in the Western hemisphere?

A

SLL/CLL

160
Q

What is a likely cause of severe combined immunodeficiency (SCID)?

A

lack of Adenosine Deaminase (ADA)

MCC (10 decks)

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