Unit 3 Lectures Flashcards
1
Q
What does it mean for a lymphoma to be nodal or extra nodal?
A
nodal: presenting as enlarged lymph nodes
extranodal: presenting in skin, brain, GI tract, etc.
2
Q
A myeloid sarcoma presents as a __________ and is very (common/rare)
A
solid mass, rare
3
Q
T/F: Many hematopoietic neoplasms contain both a leukemic and lymphomatous component
A
true
4
Q
Acute leukemia often has ____ RBCs, ____ platelets, and ____ neutrophils
A
low, low, low
5
Q
Chronic leukmeia has ____ WBCs
A
increased
6
Q
The chromosomal abnormality that is most commonly seen in hematologic malignancies is _______________
A
balanced translocation
7
Q
_____________ is implicated in the development of Hodgkin lymphoma, Burkitt lymphoma, and other B-cell non-Hodgkin lymphomas
A
Epstein-Barr virus
8
Q
_____________ is implicated in the development of adult T-cell leukemia/lymphoma
A
Human T-cell Leukemia Virus-1 (HTLV-1)
9
Q
_____________ is implicated in primary effusion lymphoma
A
Kaposi Sarcoma Herpesvirus/Human Herpesvirus 8
10
Q
What are some criteria of the WHO for classifying hematologic malignancies?
A
- microscopic appearance of malignant cells
- histologic growth patterns
- relative amount of malignant cells
- presence/absence of cell surface markers
11
Q
What cells are neoplastic cells in Hodgkin lymphoma derived from?
A
B cells
12
Q
Non-Hodgkin lymphoma refers to all the lymphomas of mature-appearing ____________
A
lymphocytes (excluding classic Hodgkin lymphomas)
13
Q
T/F: Surgery is often the first therapy used for hematologic malignancies
A
False (almost never used!)
14
Q
DNA alkylating agents and topoisomerase II inhibitors are risk factors for developing _________________
A
acute leukemia
15
Q
75% of cases of ALL occur in ________________
A
children less than 6 years old
16
Q
What is the generic marker of lymphocyte immaturity?
A
CD34
17
Q
What is the common lymphoblast marker (not on mature cells)?
A
TdT
18
Q
What are the markers of B cell lineage?
A
CD19, CD22
19
Q
What are the markers of T cell lineage?
A
CD3, CD7
20
Q
Is B or T lymphoblastic leukemia more common?
A
B (80-85%)
21
Q
B-ALL with t(9:22) is more often seen in (children/adults) and has a (favorable/unfavorable) prognosis
A
adults, unfavorable
22
Q
B-ALL with t(11q23) has a (favorable/unfavorable) prognosis and B-ALL with t(12;21) has a (favorable/unfavorable) prognosis
A
unfavorable, favorable
23
Q
T-ALL presents with _____________ more frequently in ____________
A
mediastinal mass, teenagers/young adults
24
Q
What is the average age at diagnosis with AML?
A
65
25
What are the generic markers of immaturity seen with AML?
CD34
26
What are the common myeloid markers?
CD117 (C-Kit), myeloperoxidase
27
What is a classic histological finding in AML?
auer rods
28
What translocation is associated with "AML with maturation" (still some normally developing neutrophils)?
t(8;21), RUNX1/RUNX1T1
| RUNX1 needed for differentiation
29
Does AML with t(8;21) have a good prognosis?
yes
30
Does AML with inv(16) or t(16; 16) have a good prognosis?
yest
31
AML with inv(16) or t(16; 16) is associated with the presence of abnormal ____________ precursors.
eosinophilic ("baso-eos")
32
In AML with t(15;17), cells are blocked in the ____________ stage
promyelocytic (hence, Acute Promyelocytic Leukemia rather than just AML)
33
In APL, cells have a ____________ morphology
hypergranular
34
What type of acute leukemia can be treated with retinoic acid?
APL
35
AML with t(1;22) usually shows ____________ differentiation and is often seen in infants with _____________
megakaryoblastic, Down syndrome
36
What is the latency period from therapy to AML after using an alkylating agent or radiation?
2-8 years
37
What is the latency period from therapy to AML after using a topoisomerase inhibitor?
1-2 years
38
What are the typical cytogenetic abnormalities in AML after using an alkylating agent or radiation?
whole or partial deletion of chr5, 7
39
What are the typical cytogenetic abnormalities in AML after using a topoisomerase inhibitor?
rearrangement of 11q23; MLL
40
Does cancer progress to AML through an MDS stage after using an alkylating agent or radiation?
usually yes
41
Does cancer progress to AML through an MDS stage after using a topoisomerase inhibitor?
usually no
42
What is the prognosis if the following molecular abnormalities are found?
- FLT3 internal tandem duplication
- nucleophosmin-1 (NPM1) mutation
- CEBPA mutation
poor
good
good
43
SCID is a syndrome in which you lack _____________
T and B cells
44
The block for SCID occur between _____________ and _____________
hematopoietic stem cells, early lymphoid precursors
45
inheritance pattern for SCID
x-linked recessive
46
A block in the transformation from pre-B cell to immature B cell results in _____________
Bruton's x-linked hypoimmunoglobulinemia
47
In Bruton's, you can recover from ______ infections because you have functioning ____ cells even though you lack ____ cells
viral, T, lack B cells
48
Hyper IgM occurs due to a block in...
Tfh cells (CD40L) being able to activate B cells (CD40)
49
Transient hypoglobulinemia occurs due to too little ____
IgG
50
IVIg can be used to treat ____________ immunodeficiency
secondary
51
For people with the adenosine deaminase version of SCID, what treatment is available?
blood transfusion (but you have to irradiate the blood to be sure you're killing any lingering T cells)
52
Two main characteristics of myelodysplastic syndrome
- ineffective hematopoiesis
| - increased risk of transforming to AML
53
In myelodysplastic syndrome, __________ is replaced by a __________
marrow, malignant clone
54
MDS is diagnosed in the setting of persistent peripheral ____________
cytopenia
55
Dyshematopoiesis occurs when more than ___% of cells in one lineage appear dysplastic
10%
56
Small, hypolobated or non-lobated nuclei are morphologic evidence of _______________
dysmegakaryopoiesis
57
MDS can be diagnosed by presence of...
- whole or partial deletion of chr5, 7
- isolated deletion 5q
- trisomy 8
- others
58
What are some other possible differential diagnoses for patients with suspected MDS?
- vitamin deficiency (B12, folate)
- toxin exposure
- exposure to certain drugs
- viral infections
59
In low grade MDS, blasts are
60
Refractory cytopenia with unilineage dysplasia and refractory cytosine with multilineage dysplasia are examples of ______ grade MDS
low
61
Refractory anemia with excess blasts-1 (5-9%) and refractory anemia with excess blasts-2 (10-19%) are examples of ______ grade MDS
high
62
Myeloproliferative neoplasms are characterized by an increase in _________, with a corresponding increase in __________
1+ blood cell types, marrow cellularity
63
______ is associated with persistent neutrophilic leukocytosis
CML
64
CML has ________ WBCs, platelets, and basophils
increased
65
In CML, the marrow is _________ and the nuclei are ___________
hypercellular, non-lobated
66
If left untreated, CML progresses to a ________ phase
blast
67
In (CML/Ph+-ALL), the BCR breakpoint is usually in the minor breakpoint cluster region
Ph+-ALL (190kd vs. 210kd in CML)
68
Gleevac is a _______________ used to treat ____
protein tyrosine kinase inhibitor, CML
69
Polycythemia vera is a _____ characterized by an increase in _________
myeloproliferative neoplasm, RBC mass (erythrocytosis)
70
Polycythemia vera contains a _______ mutation
JAK2 (V617F)
71
If a patient seems to have polycythemia vera but lacks a JAK2 mutation, consider...
secondary erythrocytosis (seen in smokers, chronic hypoxia, Hb disorders)
72
PV transitions from a ________ phase (________ blood cell count) to a _______ phase (_________ blood cell count)
polycythemic, increased
| spent, decreased
73
Thrombosis of the mesenteric vein, portal vein, or splenic vein should always raise concerns for ________
polycythemia vera
74
The main treatment for polycythemia vera is _________
phlebotomy
75
Primary myelofibrosis is characterized by __________ and __________ but no _____________
granulocytic, megakaryocytic hyperplasia; no erythrocytosis
76
What mutations are found in primary myelofibrosis?
JAK2 (50%)
| MPL, CALR
77
Primary myelofibrosis progresses from a pre-fibrotic to a __________ stage, which is characterized by _________________ and enlargement of organs (due to _______________________)
fibrotic
leukoerythroblastosis
extramedullary hematopoiesis
78
Entrapped bizarre megakaryocytes on H&E suggest an ______________
MPN (myeloproliferative neoplasm)
79
What mutations are found in essential thrombocythemia?
JAK2, MPL, CALR
80
T/F: splenomegaly is common in essential thrombocythemia
False
81
In myasthenia gravis, antibody is made against _____ receptors
Ach
82
What is the treatment for myasthenia gravis?
IVIg
83
What is goodpasture syndrome?
autoantibody reacts with basement membrane of capillaries in kidney and lung
84
What is the primary antigen in goodpasture syndrome?
collagen epitope
85
In Graves, antibody is stimulatory to the _____ receptor on the surface
TSH
86
Hashimoto is an autoimmune _________ disease
thyroid
87
The mantle zone of a lymph node is comprised entirely of ____ cells
B cells
88
The dark zone of a lymph node germinal center is comprised of ________ cells and the light zone is ___________ cells
dark: B cells
light: B cells, T cells, macrophages, etc.
89
CD19 and CD22 are ___ cell markers
B cell
90
CD3, CD4, CD5, and CD8 are ___ cell markers
T cell
91
What are two markers specifically expressed in normal germinal B cells?
CD10, BCL6
92
Germinal center B-cell lymphomas include...(3)
follicular, Hodgkin, Burkitt
93
In CLL cells, the nucleus has (round, condensed/loose) chromatin and the cells are (small/large)
round, condensed; small
94
Is there effacement of lymph node architecture in CLL/SLL?
yes
95
CLL and SLL are strongly positive for... (3)
CD5, CD19, CD23
96
Describe the histology of follicular lymphoma
homogenous tumor cells with no dark zone, light zone, or tangible body macrophages
97
BCL2's function is to ___________ and is (unregulated/downregulated in normal germinal centers)
suppress apoptosis, downregulated
98
Follicular lymphoma is strongly positive for...
B-cell markers: CD19, CD20
Germinal center B-cell marker: CD10, BCL6
BCL2
99
Mantle cell lymphoma differs from other B-cell lymphomas in that it (is/is not) particularly aggressive
is aggressive
100
Mantle cell lymphoma is positive for...
B-cell markers: CD19, CD20
CD5 (also positive in CLL/SLL)
cyclin D1 (BCL1) ***
101
Cyclin D1 (BCL1) is important because its function is to...
activate genes required for cell cycle progression (G1/S transition)
102
In mantle cell lymphoma, there is a _____ translocation that renders cyclin D1 continually on
t(11;14)
103
Burkitt lymphoma: Describe the typical patient, location of lymphoma, and % EBV involvement:
- endemic BL
- sporadic BL
- immunodeficiency-associated BL
- endemic BL: children, jaw or abdomen, 95%
- sporadic BL: children/young adults, iliocecal area, 30%
- immunodeficiency-associated BL: HIV patients, 25-40%
104
Burkitt's lymphoma presents with deeply _________ cytoplasm with lipid vacuoles and H&E stain has a ___________ pattern
basophilic, "starry sky" pattern
105
Burkitt's lymphoma is positive for...
B-cell markers: CD19, CD20
germinal center B-cell markers: CD10, BCL6
MYC ***
106
T/F: Diffuse large B-cell lymphoma is the most common of the non-Hodgkin lymphomas
true
107
List five factors that affect prognosis of ALL
- age
- WBC count
- response to therapy
- number of chromosomes
- B cell vs. T cell
108
What is the most common immunodeficiency disease?
selective IgA deficiency
109
In low grade myelodysplastic syndrome, myeloblasts (are/are not) increased in frequency
are not
110
Name 3 sites where DIC could occur with polycythemia vera
- mesenteric vein
- portal vein
- splenic vein
111
A type II immune reaction involves what immunoglobins?
IgG, IgA, IgM
112
What is the immunofluorescent pattern in Goodpasture syndrome?
linear
113
What is the only immunopathology that does not require B cells or antibodies?
type IV
114
_____ and _____ down regulate Tregs and up regulate Th1, Th2, and Th17
TGFbeta, IL-6
115
_____ favors Treg development
IL-10
