Quiz 2 Flashcards
1
Q
What is Gout?
A
The deposition of MSU crystals in tissues (joints especially) due to hyperuricemia
2
Q
What is the function of CD antigens?
A
They are the primay cell surface molecules used to determine cell lineage
3
Q
What is the immunophenotype expressed by lymphocytes?
A
The pattern of expressed CD antigens
4
Q
What is the T-cell lineage marker?
A
CD3
5
Q
What is the B-cell lineage marker?
A
CD19
6
Q
What is the mature B cell marker?
A
CD20
7
Q
What is the immaturity marker of a cell?
A
CD34
8
Q
What antigens are responsible for assessment of B-cell clonality?
A
Kappa and Lambda light chains
9
Q
What antigen acts as a myeloid lineage marker?
A
MPO
10
Q
What antigen acts as an immature lymphocyte marker?
A
TdT
11
Q
What type of lymphoma is most likely indicated by a t(14;18) IGH-BCL2 translocation?
A
a follicular lymphoma
12
Q
What type of lymphoma is most likely indicated by a t(11;14) CCND1-IGH translocation?
A
a mantle cell lymphoma
13
Q
What type of lymphoma is most likely indicated by a t(8;14) MYC-IGH translocation?
A
Burkitt lymphoma
14
Q
What test could be run for more information on the granulation status of neutrophils?
A
morphologic WBC analysis
15
Q
In RNA/DNA catabolism, loss of Adenosine Deaminase (ADA) leads to shutting down of what enzyme?
A
Ribonucleotide Reductase (RNR)
16
Q
Ribonucleotide Reductase (RNR) is required for synthesis of which DNA precursors?
A
All dNTP synthesis requires RNR
17
Q
DNA catalysis requires stable free radicals scavenged by which molecule?
A
hydroxyurea
18
Q
What is the role of an E1 ubiquitin ligase?
A
To activate multiple E2 ubiquitin ligases
19
Q
What is the role of E2 ubiquitin ligases?
A
To hold ubiquitin (bullet) for the target protein
20
Q
What is the role of E3 ubiquitin ligases?
A
To form a complex with E2 and identify the target protein for ubiquitination
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
22
Q
What are dohle bodies a sign of in PMNs?
A
activation in response to inflammation
23
Q
What are aquired causes of neutrophilia?
A
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
25
Would reactive neutrophilia or CML be more likely in a patient with an LAP score of over 115?
Reactive neutrophilia
26
Would a CBC revealing elevated eosinophils and basophils with absolute basophilia be more indicative of reactive neutrophilia or CML?
CML
27
Which translocation would be expected in diagnosis of CML?
t(9;22) BCR/ABL
28
What are the 5 main causes of lymphocytosis?
Infection (viral most common)
autoimmune disease
smoking (polyclonal B-cell lymphocytosis)
Chronic lymphocytic leukemia (CLL)
Other non-Hodgkin lymphomas
29
What treatment is preferred for CML?
tyrosine kinase inhibitors
30
What lab testing would be preferred for reactive lymphocytosis?
infectious serology
31
What lab testing would be preferred for mononucleosis?
EBV, monospot, serology, PCR
32
What signs in the peripheral blood smear might indicate infectious mononucleosis?
Downey cells, or variant lymphocytes
33
What clinical signs might suggest mononucleosis?
fever, pharyngitis, cervical lympadenopathy (LAD)
34
What is the most likely clinical presenation for a pt with undiagnosed CML or CLL?
asymptomatic
35
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?
CLL
36
What are the 5 most common causes of Eosinophilia?
Infection (parasitic or fungal most likely)
Allergic diseases
rheumatologic diseases
Drug rxns
neoplasm
37
Prolonged elevations of eosinophil counts above what level are usually injurious, and can lead to cardiac fibrosis?
5000u/L
38
Patients with hypereosinophilia of uncertain cause can be treated with what medication?
hydroxyurea
39
What are the most common causes of neutropenia?
Genetic: Kostmann syndrome, idiopathic
Acquired: Infection, antivirals or antipsychotics, autoimmune disorders, aplastic anemia, neoplasms
40
What are possible causes of neutrophil hyposegmentation?
Pelger-Huet anomaly, MDS, medications (tacrolimus)
41
What are possible causes of neutrophil hypersegmentation?
Vit B12/Folate deficiency, MDS, medications
42
What pathology would be indicated by a FISH finding of a t(9;22) BCR-ABL fusion?
Chronic myeloid leukemia
43
What is the pathology of Pelger-Huet anomaly?
It is a benign manifestation of hyposegmented neutrophils
44
T/F: A lymphoma can include immature lymphoid cells
False
45
T/F: A leukemia can be lymphoid or myeloid
True
46
What type of neoplasms are clonal disorders?
Lymphoid neoplasms
47
How are immunoglobulins expressed by B-cells measured?
flow cytometry or PCR
48
What are the most notable B-cell lymphomas?
CLL/SLL
Mantle cell lymphoma
Follicular lymphoma
DLBC lymphoma
Burkitt lymphoma
Hodgkin lymphoma
49
Which of the common B-cell lymphomas are considered high-grade, aggressive? What characterizes them?
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
50
Which of the common B-cell lymphomas are considered low-grade, indolent? What characterizes them?
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
51
Which B-cell lymphoma can have translocations involving MYC, BCL2, or BCL6
DLBCL
52
Which lymphoma is characterized by a "starry sky" appearance of lymph node histology?
Burkitt lymphoma
53
In cases of HIT, what medication may be indicated for a pt to switch to?
Argatroban is the IIa inhibitor indicated in cases of HIT
54
What are the symptoms of multiple myeloma?
CRAB symptoms:
hyper C alcemia
Renal failure (elevated creatinine)
Anemia
Bone pain
55
What is the Rouleaux formation as seen on a blood smear?
"Coin stacks" of RBCs
56
What do Auer rods indicate on a blood smear?
a cell of myeloid lineage
57
What pathology is associated with a t(15;17) PML-RARA translocation?
acute promyelocytic leukemia
58
Which leukemia is most associated with DIC and considered an acute hematological emergency?
AML t(15;17) PML-RARA (acute promyelocytic leukemia)
59
What treatments are used for acute promyelocytic leukemia?
ATRA (all-transretinoic acid) and arsenic trioxide
60
What is the significant finding on the bone marrow aspirate smear?
numerous promyelocytes
61
What 4 findings are most consistant with DIC?
elevated PT
elevated aPTT
decreased fibrinogen
elevated D- dimer
62
What is the biggest risk with DIC?
hemorrhage
63
What two types of AML are referred to as "CBF leukemias"?
AML t(8;21) RUNX1-RUNX1T1
AML t(16;16) CBFB-MYH11
64
What is a common presentation of acute B-lymphoblastic leukemia (B-ALL) with hyperdiploidy?
A high number of triploid or quadriploid chromsomes
Often presents with cytopenias and bone pain
65
What are the four Ts for ddx for a mediastinal mass?
"Terrible" lymphoma or leukemia
Thymic tumor
"Teratoma" or GC tumor
Thyroid tumor
66
Which type of cancer often presents as a mediastinal mass with/without BM involvement, more commonly in adolescents?
T-ALL (leukemia/ or lymphoma)
67
Which neoplasms fall into the group of acute myeloid neoplasms?
68
Which neoplasms fall into the group of acute lymphoid neoplasms
69
Which neoplasms fall int othe group of MPN-MDS?
70
Which neoplasms fall in to the group of mature lymphoid neoplasms?
71
What does a blast quantity of over 20% in peripheral blood indicate?
an acute leukemia
72
How can gene fusion be detected?
Chromosome analysis (karyotype), FISH, or molecular testing
73
What is considered a major molecular response in treatment of CML?
1,000 fold or a 3 log reduction in BCR-ABL1 transcripts
74
What chiefly characterizes Polycythemia vera?
-Exclusion of secondary causes of increased RBC mass
-Low serum EPO
-usually a JAK2V617F mutation
-usually hyperceullar BM with panmyelosis
75
What are three principal treatments of PV?
Phlebotomy, JAK inhibitors, hydroxyurea
76
Which conditions have a bimodal distribution in average age at dx?
Hodgkin's lymphoma, ET,...
77
What characterizes essential thrombocythemia?
-Platelets >450K/uL
-atypical megakaryocyte hyperplasia in BM
-JAK2, CALR or MPL mutation
-must exclude secondary thrombocytosis
78
What is notable about this blood smear?
thrombocytosis
79
What are characteristics of myelofibrosis?
-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
What are treatments for primary myelofibrosis?
JAK inhibitors, hydroxyurea, spenectomy (if massive), and SC transplant if progressed
81
Of PV, ET, and PMF, which has the poorest prognosis?
PMF
82
What are major similarities between PV, ET, and PMF?
-Peripheral blood thrombocytes
-Gene mutations in JAK2, MPL, or CALR
- BM abnormalities
83
What characertizes MDS?
-Ineffective hematopoeisis
-High risk of developing AML
-Abnormal cells remain in BM, causing peripheral cytopenias
84
What three features are required for a dx of MDS?
1. Cytopenia
2. Morphologic dysplasia (under microscope)
3. Abnormal cytogenetic/chromsome studies
85
What treatments are used for MDS?
Limited options; but in younger patients, allogeneic SC transplant, maybe DNA methyltransferaseinhibitors (Azacitidine)
86
In what category do MGUS, smoldering myeloma and multiple myeloma fall?
Plasma cell neoplasms
87
Which anticoagulants are contraindicated in pregnancy?
Warfarin and DOACs
88
Where is DVT most likely to occur?
in the legs, usually the calves
89
What are the current treatments for DVT?
Direct Xa inhibitor
LMWH
Thrombin inhibitor (LMWH bridging)
Warfarin (LMWH briding)
90
How long is DVT usually treated?
3 months, but could be more depending on situation
91
What is a good treatment for DVT if bleeding is a problem? Why?
Unfractionated heparin; it is quickly/completely reversible with protamine
92
How is PE treated differently than DVR?
It is currently treated the same
93
Which is the pathology of heparin-induced thrombocytopenia?
Antibodies in the pt. bind to the heparin-PF4 complex
94
In the event of HIT after heparin administration, what would be the best course of action?
STOP heparin treatment, and administer a thrombin inhibitor (argatroban is preferred for HIT)
95
Which anticoagulants have been used most consistantly in cancer patients? Are there any other types that are becoming more commonly used?
LMWHs (dalteparin, enoxaparin, tinzaparin); DOACs (rivaroxaban, apixaban, edoxaban, dibigatran)
96
Of the following reversal protocols for Warfarin administration, which has the longest time of action?: PT complex concentrate, FFP, Vit K
Vit K
97
What are the most common B-cell neoplasms in adults?
Follicular lymphoma and DLBCL
98
What is the most common leukemia in adults in the Western hemisphere?
SLL/CLL
99
What is the main difference between CLL and SLL?
CLL: leukemic form
SLL: in node form
100
What are the approximate ratios of B-cell NHL, Hodgkin lymphoma, and T-cell NHL in the population?
B-cell NHL: 80%
Hodgkin lymphoma: 11%
T-cell NHL: 9%
101
What are the main differences between low grade/indolent and high grade/aggressive lymphomas?
LG/I: older pt, not curable, high stage
HG/A: younger pt, curable, low stage, B symptoms
102
What type of lymphoma is indicated by this biopsy? Why?
DLBCL; the lymphocytes are atypical and large (1.5-2x normal lymphocyte)
103
What type of lymphoma is indicated by this biopsy? why?
Hodgkin lymphoma; the classic "owl eye" cells are Reed Sternberg cells
104
What type of lymphoma is indicated by this biopsy? Why?
Burkitt lymphoma; the classic "starrry sky" appearance of the lymph node (increase in tingible body macrophages)
105
What type of lymphoma is indicated by this biopsy? Why?
Follicular lymphoma; low power view of back-to-back follicles
106
What translocation is associated with Burkitt lymphoma? What are other notable features?
t(8;14)
107
What translocation is associated with CML (required for CML dx, but also possible in ALL or AML)? What are other notable features
t(9;22)
108
What translocation is associated with Follicular lymphoma? What are other notable features?
t(14;18)
109
What translocation is associated with Acute promyelocytic leukemia? What are other notable features?
t(15;17)
110
What featuers are likely in CML, but NOT in reactive neutrophilia?
Absent toxic changes in neuotrphils, absolute basophilia, splenomegaly, low LAP score, BCR-ABL1 fusion t(9;22)
111
What is the difference between average age at dx for AML and ALL?
AML: mostly adults
ALL: mostly kids
112
What is the liklihood of splenomegaly in leukemia? In MDS? In aplastic anemia?
Leukemia: Common
MDS: less common
aplastic anemia: not common
113
What is a major differentiator between MDS and aplastic anemia?
MDS will have a hyper-cellular marrow
114
What is the criteria for monoclonal gammopathy of undetermined significance (MGUS)?
115
What is the criteria for multiple myeloma?
116
What is the criteria for smoldering myeloma?
117
A small, round, blue cell tumor is representative of what specific kind?
a pediatric tumor
118
What is the clinical use of Rituximab?
Immunotherapy; a monoclonal antibody used to treat CLL
119
What does the American Society for Hematology recommend in work-up for a plasma cell neoplasm?
1- CBC w/ diff, Ca, Cr, UA
2- SPEP for M-spike
3- Immunofixation to confirm Ig
4- Imaging and BM biopsy
120
What does an M-protein or M-spike look like on SPEP compared to normal?
121
What is another name for multiple myeloma?
plasma cell myeloma
122
In what type of of plasma cell myelomas is rouleax seen?
neoplasms with monoclonal proteins
123
In what age range would you expect to see ALL and AML respectively?
ALL: mostly in children and young adults
AML: ages 15-39
124
In what age range would you expect to see CML and CLL respectively?
CML: between 30-60 yo
CLL: over 50 yo
125
What type of test is a monospot test?
A serologic test for mononucleosis
126
What is the clinical indication of imatinib?
It is a popular tyrosine kinase inhibitor
127
What are two advantages of using FISH over PCR?
FISH is quicker
Allows detection in an intact cycle
128
Lack of HGPRTase is responsible for what disorder?
Lesch-Nyhan syndrome
129
What is the action of HGPRTase?
To salvage hypoxanthine, or guanine by attaching it to PRPP
130
What is a key symptom of Lesch Nyhan syndrome?
Uncontrollable lip/tongue biting, finger biting, or head banging
131
What is inhibited by allopurinol? What is the target molecule's function?
Xanthine oxidase; it is a purine oxidizing enzyme that makes urate
132
On what kinds of cells is Ki-67 expressed?
cells actively cycling (NOT in G0)
133
Is PRPP used in the de novo or salvage pathways for purine biosynthesis?
It is required for both
134
ATM is a cell cycle "checkpoint" protein that is specific to what stage?
It is S-phase specific (blocks entry to S-phase
135
The improvements from around a 90% mortality rate to less than 10% of ALL in children is mostly due to what?
Optimal combinations and timing of chemotherapy
136
How would a Hodgkin lymphoma in stage Ia or IIa be treated?
chemotherapy plus radiation therapy
137
What is the therapeutic INR for most patients on warfarin?
2.0-3.0
138
What test was done to produce these results?
a gene expression microarray
139
What useful information about cancer can be provided by a gene expression microarray?
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
What is the first step after finding lymphocytosis in a CBC?
Determining if the lymphocytes are derived from a single clone (leukemia/lymphoma) or have many variants (leukemoid reaction)
141
What is the result of a t(14;18) BCL-2 translocation?
Follicular lymphoma, more specifically, a mutation causing overexpression of BCL-2, an oncogene that inhibits apoptosis
142
Why is a protein electrophoresis of blood and urine an appropriate test to dx multiple myeloma?
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
What type of leukemia is princibly treated with a tyrosine kinase inhibitor (iminitab)?
CML
144
When should a bone marrow biopsy be performed?
When the etiology of an abnormal peripheral blood count is uncertain or if a hematologic malignancy is expected
145
What is indicated by a high or low INR, respectively?
High- slow forming clots
Low- fast forming clots
146
What is a clinical sign that warrants inclusion of Follicular lymphoma on the ddx?
Painless lympoh nodes that wax and wane (usually in adults)
147
What are bone pain, proteinurea, and Rouleaux formation all signs of?
Multiple Myeloma (plasma cell myeloma)
148
What is the first step in treating a patient with an embolism causing an acute MI?
Dissolve the clot
149
What are major signs of TTP?
Pupura in extremities, schistocytes, low platelets, and elevated fibrinogen. Testing will reveal levels <10 of ADAMTS13
150
How does a COX1/2 inhibitor like aspirin impair platelet aggregation?
Inhibition of COX 1 and 2 results in decreased TxA2 production, causing platelets to not express GPIIb/IIa and fail to aggregate
151
What is the primary characterization of MDS vs PMF?
MDS: ineffective hematiopoiesis -> pancytopenia
PMF: deposition of megakaryocytes and granulocytes in BM -> marrow fibrosis
152
When is adjuvant chemotherapy given?
After surgery to reduce local and distant recurrence
153
When is neoadjuvant chemotherapy given?
Before surgery to improve outcomes
154
When is inducion chemotherapy given?
to achieve remission of cancer
155
When is consolidation chemotherapy given?
After induction to treat microscopic disease
156
When is maintenance chemotherapy given?
Over long-term to maintain remission
157
When is salvage chemotherapy given?
After relapse or refractory to previous therapy
158
What is the most common B-cell neoplasm in adults?
follicular lymphoma
t(14;18) IGH/BCL-2
159
What is the most common leukemia in adults in the Western hemisphere?
SLL/CLL
160
What is a likely cause of severe combined immunodeficiency (SCID)?
lack of Adenosine Deaminase (ADA)
