Malignancies Flashcards
(91 cards)
1
Q
What is TdT a marker of?
A
Lymphoblasts, specifically
2
Q
The Philadelphia Chromosome is seen in what percentage of cases of adult B-ALL?
A
25%
3
Q
The Philadelphia Chromosome is seen in what percentage of cases of childhood B-ALL?
A
2%
4
Q
What is the Philadelphia chromosome?
A
t(9;22); BCR-ABL1
5
Q
Is the presence of the Philadelphia chromosome a marker of good or poor prognosis?
A
Poor
6
Q
Is B-ALL with t(12;21); ETV6-RUNX1 a good or poor prognosis?
A
Very favorable prognosis
7
Q
B-ALL with t(12;21); ETV6-RUNX1 is seen in what percentage of childhood B-ALL?
A
25%
8
Q
What age group does T-ALL favor?
A
Adolescents and young adults
9
Q
How does T-ALL often present?
A
A mediastinal mass
10
Q
What is the average age of onset of AML?
A
65 years old
11
Q
How is AML diagnosed?
A
Presence of >20% myeloblasts in marrow and/or peripheral blood
12
Q
Which virus contributes to Hodgkin lymphoma, Burkitt lymphoma, and other non-Hodgkin lymphomas?
A
EBV
13
Q
EBV contributes to which neoplasms?
A
Hodgkin lymphoma, Burkitt lymphoma, and other non-Hodgkin lymphomas
14
Q
Which virus contributes to adult T cell leukemia/lymphoma?
A
HTLV-1
15
Q
HTLV-1 contributes to which neoplasms?
A
Adult T cell leukemia/lymphoma
16
Q
KSHV/HHV-8 contributes to which neoplasms?
A
Primary effusion lymphoma
17
Q
Which virus contributes to primary effusion lymphoma?
A
KSHV/HHV-8
18
Q
Which is more indolent or curable: Leukemia or Lymphoma?
A
Leukemia
19
Q
Leukemias account for roughly what percentage of all childhood cancers?
A
37%
20
Q
ACUTE leukemias are most often accumulation of what?
A
Blasts
21
Q
CHRONIC leukemias are most often accumulation of what?
A
Mature cells
22
Q
Most ALL is diagnosed in what age group?
A
< 6 years old
23
Q
What are two categories of ALL?
A
B-ALL & T-ALL
24
Q
What do B lymphoblasts express?
A
CD19, CD22, and/or CD79a
25
What are markers of mature B cells?
CD20 & surface Ig
26
B-ALL accounts for what percentage of all ALL?
80-85%
27
Is the 11q23;MLL cytogenetic finding of B-ALL a good or poor prognosis?
Poor
28
T 11q23;MLL cytogenetic finding of B-ALL is usually found in what age group?
Neonates & young infants
29
What gender does T-ALL favor?
Males
30
Which ALL is more likely to have a more elevated WBC count?
T-ALL
31
What is associated with a better ALL prognosis?
1-10 years old, B hyperdiploidy
32
What is associated with a worse ALL prognosis?
10yrsold, very high WBC, T hypodiploidy, slow response to Rx
33
What is about the incidence of both AML and ALL?
~3 cases per 100,000 per year
34
What is a general marker of immaturity on myeloblasts and lymphoblasts?
CD34
35
What are two markers of myeloid cells specifically?
CD117(C-Kit) & Myeloperoxidase
36
Where are Auer rods?
Only on abnormal Myeloblasts
37
What is the age group and prognosis of AML with t(8;21);RUNX1-RUNX1T1?
Younger patients; relatively good prognosis
38
What does RUNX1 encode for?
Alpha subunit of core binding factor (CBF) which is necessary for hematopoiesis
39
What is the age group and prognosis of AML with inv(16) or t(16/16);CBFB-MYH11?
Younger patients; relatively good prognosis
40
What is AML with t(15/17);PML-RARA also known as?
Acute Promyelocytic Leukemia (APL)
41
Acute Promyelocytic Leukemia (APL) is aka?
AML with t(15/17);PML-RARA
42
What type of cell predominates in APL?
Abnormal promyelocytes (instead of blasts)
43
What are two reasons it's important to recognize that an AML is specifically the APL subtype?
1. APL does not require traditional chemo but instead it can be treated with all-trans retinoic acid (ATRA) in combination with arsenic salts which has much less morbidity than chemotherapy
2. APL can give rise to DIC, so it's important to be on the lookout for this complication
44
AML with t(1;22)(p13;q13);RBM15-MKL1 is most often seen in who?
Infants with DS
45
What kind of prognosis does AML with t(1;22)(p13;q13);RBM15-MKL1 have?
Relatively good prognosis
46
What kind of prognosis does AML with abnormalities of 11q23;MLL have?
Poor
47
What is CD34 a marker of?
Immature lymphoblasts & myeloblasts
48
Therapy-related AML (t-AML) accounts for what percentage of all cases of AML?
10-20%
49
What are the two different classes of t-AML?
Secondary to alkylating agents or radiation; Secondary to topoisomerase-II inhibitors
50
What percentage of AML cases have no recurrent cytogenetic findings and normal karyotype? (AML-NOS)
50%
51
What are the three molecular markers currently used to determine prognosis of AML-NOS?
1. FLT3 (poor prognosis)
2. NPM1 (good)
3. CEBPA Mutation (good)
52
What are the two main features that characterize MDS?
1. Ineffective hematopoiesis
| 2. Increased risk of transformation to AML
53
What are the two clinical scenarios of MDS?
1. Primary/Idiopathic
a. People over 50 yrs old
b. Insidious onset
c. 3-5 cases per 100,000 per year
2. Secondary/Therapy-related (t-MDS)
a. Occurs like t-AML
b. Usually 2-8 years after alkylating agents of radiation
Usually contains whole or partial deletions of chromosomes 5 and/or 7
54
What are some types of tests that can be used to diagnose MDS?
Morphologic evidence of dysplasia; presence of clonal cytogenetic abnormalities
55
What are four possible causes of secondary myelodysplasia that might mimic MDS?
Drugs like chemi, toxicity (esp heavy metals), B12 folate etc deficiencies, viral infection
56
What are the differences in diagnostic criteria and prognosis between low grade and high grade MDS?
• Low grade MDS: Myeloblasts account for 5% of marrow cells and >2% of peripheral blood cells. Dismal prognosis.
57
What cytogenetic abnormalities can lead to MDS?
Monosomy 5 or 7, 5q or 7q deletions, trisomy 8
58
List 2 reasons for the frequent occurrence of splenomegaly and hepatomegaly in patients with MPNs
* Too many cells, get sequestered
| * Extramedullary hematopoiesis
59
What are three possible negative end points for MPNs?
development of AML, MDS, or excessive marrow fibrosis resulting in marrow failure
60
What are four types of MPNs?
1. Chronic Myelogenous Leukemia (CML)
2. Polycythemia Vera (PV)
3. Primary Myelofibrosis (PMF)
4. Essential Thrombocythemia (ET)
61
What cell type is elevated in CML?
Neutrophils
62
What are the marrow findings of CML?
Small megakaryocytes with round non-lobulated nuclei; no dysplasia
63
What is the common cytogenetic/molecular abnormality of CML?
Philadelphia chromosome
64
What cell type is elevated in PV?
Erythrocytes (accompanied by neutrophils and platelets)
65
What are the marrow findings of PV?
Clusters of large bizarre megakaryocytes
66
What is the common cytogenetic/molecular abnormality of PV?
Mutation of the JAK2 gene encoding the JAK2 cell signaling protein
67
What are the major complications of PV?
Venous & Arterial Thrombosis
68
Thrombosis is what vessels make you suspect PV?
Mesenteric, portal, and splenic veins
69
What cell type is elevated in PMF?
Proliferation of granulocytic & megakaryocytic lineages
70
What cell type is elevated in ET?
Thrombocytes
71
What are the marrow findings of PMF?
Large bizarre clustered megakaryocytes
72
What are the marrow findings of ET?
Large bizarre clustered megakaryocytes (even larger and more bizarre than PMF)
73
What is the common cytogenetic/molecular abnormality of PMF?
JAK2 mutation in 50% of cases
74
What is the common cytogenetic/molecular abnormality of ET?
JAK2 mutation in 50% of cases
75
What is the most common treatment for PV?
Serial phlebotomy (blood letting) and aspiring therapy
76
What are CD10 and BCL6 markers of?
Germinal B-cells and derived lymphomas
77
What molecular markers do Reed-Sternberg (RS) cells express?
CD30 and CD15
78
What is the main difference between CLL and SLL?
In SLL, extramedullary sites predominate
79
What is the median age of SLL and which gender predominates?
65, M:F 2:1
80
What is the most common genetic abnormality of SLL?
deletion of 13q14
81
What specific cells are associated with Follicular Lymphoma?
Germinal B cells
82
What is the median age of Follicular Lymphoma, and what gender does it prefer?
60 years, M:F 1:1
83
What is the common genetic abnormality of Follicular Lymphoma?
Positive for BCL2, which suppresses apoptosis
84
What is the median age of Mantle Cell Lymphoma, and what gender does it prefer?
60 years, M:F 2:1
85
What is the common genetic abnormality of Mantle Cell Lymphoma?
Positive for Cyclin D1 (BCL1)
86
What are the characteristics of the cells affected by Burkitt's Lymphoma?
Medium sized B cells with basophilic cytoplasm and an increased mitotic rate
87
What are the two subcategories of Burkitt's lymphoma and where in the body do they predominate?
Endemic BL: found in equitoria Africa, 95% EBV+, jaw & facial bones, also distal ileum, cecum, and omentum
Sporadic BL: found mostly in children/young adults, mostly ileocecal area
88
What kind of cell is necessary for diagnosis of Classical Hodgkin's Lymphoma?
Reed-Sternberg (RS) cells
89
What are the two types of Hodgin's Lymphomas? And what is one of them further classified into?
Nodular Lymphocyte-Predominant (NLPHL)
```
Classical Hodgkin's Lymphoma (CHLs)
Nodular Sclerosis
Lymphocyte-Rich
Mixed Cellularity
Lymphocyte-depleted
```
90
Where does Nodular Sclerosis HL usually occur?
Above the diaphragm
91
Where does Lymphocyte-Rich HL usually occur?
Below or on both sides of diaphragm
