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IMMUNOLOGY AND SEROLOGY > STEVENS CHAPTER 18: IMMUNOPROLIFERATIVE DISEASES > Flashcards

STEVENS CHAPTER 18: IMMUNOPROLIFERATIVE DISEASES Flashcards

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1
Q

It refers to a diverse group of hematologic malignancies that arise from hematopoietic cells of a lymphoid or myeloid lineage.

A

immunoproliferative diseases

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2
Q

Leukemias are malignancies that originate from what cell in the bone marrow, which migrate to the peripheral blood?

A

hematopoietic cells

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3
Q

These are solid tumors whose cells usually arise in the lymph nodes and other lymphoid tissues, such as the tonsils and spleen, and proliferate in those sites.

A

lymphomas

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4
Q

T/F: The sites affected by leukemias and lymphomas don’t really overlap, as leukemic cells cannot be found in the lymphoid tissues and other organs in the body, and lymphoma cells are also not present in the peripheral blood.

A

F

(they can overlap)

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5
Q

Differentiate leukemia and lymphoma base on their origin.

A

Leukemias originate from hematopoietic cells and migrate to peripheral blood

Lymphomas usually arise in the lymph node and other lymphoid tissues

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6
Q

Group of disorders that include malignancies of the plasma cells as well as associated premalignant conditions.

A

plasma-cell dyscrasias

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7
Q

Sites commonly involve in plasma-cell dyscrasias

A
  • bone marrow
  • lymphoid organs
  • other nonlymphoid sites
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8
Q

plasma-cell dyscrasias, also known as? (2 other names)

A
  • plasma-cell disorders
  • monoclonal gammopathies
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9
Q

T/F: Plasma-cell dyscrasia are considered biologically distinct from the leukemias and lymphomas.

A

T

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10
Q

Hematologic malignancies are characterized by

A

excessive accumulation of cells in the blood, bone marrow, or other lymphoid organs

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11
Q

Excessive accumulation of cells in hematologic malignancies may occur due to?

A
  • Rapid proliferation and excess production of the cells
  • Failure of the cells to undergo apoptosis
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12
Q

In hematologic malignancies, aside from failure of growth regulation, mutations can also result in

A

arrested maturation of a cell

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13
Q

Can malignant and premalignant proliferation of cells occur at any stage in the differentiation of the lymphoid lineages?

A

yes

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14
Q

Malignant lymphoid cells generally retain some or all of the morphological and functional characteristics of their normal counterpart. These characteristics
are often used to?

A

classify lymphoid malignancies

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15
Q

Normal immune responses are polyclonal/monoclonal
(?)

A

polyclonal

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16
Q

What does polyclonal immune response mean?

A

Cells with different features, such as antigen specificity, all proliferate in response to an immune stimulus.

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17
Q

Hematologic malignancies are polyclonal/monoclonal
(?)

A

monoclonal

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18
Q

Suspicion of a hematologic malignancy is raised when there are?

A

elevated numbers of a specific population of lymphocytic cells in the bloodstream, bone marrow, or lymphoid tissues

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19
Q

The key genes involved in hematologic malignancies are

A

proto-oncogenes and tumor suppressor genes

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20
Q

This gene promote cell growth and division

A

proto-oncogenes

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21
Q

This gene control cell division by regulating the progression of cells through the cell cycle and maintain the genetic stability of the cells by repairing damaged DNA.

A

tumor suppressor genes

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22
Q

Changes in proto-oncogenes and tumor suppressor genes can result in uncontrolled what?

A

uncontrolled cell proliferation

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23
Q

The genetic alterations in malignant cells of hematopoietic origin include

A
  • Point mutations
  • Duplications/deletions of specific genes
  • Chromosome translocations
  • Additions or deletions of specific chromosomes
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24
Q

Additions or deletions of specific chromosomes which result in abnormal numbers of chromosomes is referred to as

A

aneuploidy

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25
A translocation involving the c-MYC gene on chromosome 8 and the immunoglobulin µ gene on chromosome 14, denoted as [t(8;14)], is believed to be involved in the pathogenesis of
Burkitt lymphoma
26
Rearrangement, such as c-MYC is placed under the control of a different gene promoter sequence could result to?
Overexpression of the c-MYC gene
27
Most cases of follicular lymphoma have what translocation?
t(14;18)
28
Chromosome that contains the immunoglobulin [Ig] heavy-chain genes
chromosome 14
29
Chromosome that contains an anti-apoptotic gene called BCL-2
chromosome 18
30
The BCL-2 gene induces the production of
an inner mitochondrial membrane protein
31
The inner mitochondrial protein induced by BCL-2 blocks what process
apoptosis
32
T/F: cells affected by t(14;18) translocation die normally
F
33
In follicular lymphoma, why is there an excessive number of cell proliferation even though the altered cells do not proliferate at an increased rate?
because their survival is enhanced compared with normal cells
34
Chronic myelogenous leukemia is characterized by a translocation between what genes?
Breakage Cluster Region (BCR) and c-ABL proto-oncogene
35
c-ABL proto-oncogene is located in what chromosome?
chromosome 22
36
BCR (breakage cluster region) is located in what chromosome?
chromosome 9
37
Translocation between the BCR and the c-ABL proto-oncogene results in what protein?
BCR/ABL fusion protein
38
BCR/ABL fusion protein codes for?
a continuously activated TYROSINE KINASE enzyme
39
a continuously activated TYROSINE KINASE enzyme causes what?
unregulated cell division
40
An anticancer drug that specifically targets the BCR/ABL fusion protein
Gleevec
41
Several schemes have been used over the years to classify the hematologic malignancies. What are these schemes?
- French-American-British (FAB) - Cooperative Group consensus criteria for leukemias and myelodysplastic syndromes - Revised European American Lymphoma (REAL)
42
This scheme was adopted by WHO in 2001, updated in 2008, and became the basis for the classification scheme for all types of hematologic malignancies.
REAL
43
Leukemias can be broadly divided into what two groups? This is based on the cell type from which they originated.
myeloid and lymphoid
44
The myeloid leukemias are derived from
common myeloid precursor
45
Myeloid leukemias are derived from common myeloid precursor, that encompasses what cells?
granulocytic, monocytic, megakaryocytic, and erythrocytic leukemias
46
The lymphoid and myeloid leukemias can be further divided into what types?
acute and chronic
47
Identify if chronic or acute: Involve immature/precursor cells called lymphoblasts (or blasts) that multiply rapidly in the bone marrow and are present in the peripheral blood.
ACUTE lymphoid leukema
48
Identify if acute or chronic leukemia: This generally involve mature cells. They usually present with variable nonspecific symptoms and are more slowly progressive.
chronic leukemia
49
Which has high response to therapy? Acute or chronic?
acute
50
This leukemia is characterized by the presence of poorly differentiated precursor cells (blast cells) in the bone marrow and peripheral blood and is usually seen in children.
Acute lymphoblastic leukemia (ALL)
51
Acute lymphoblastic leukemia (ALL) is also known as?
acute lymphocytic leukemia
52
ALL is usually seen in children between what age?
2-5 years old
53
T/F: ALL has a poor prognosis with a low rate of remission
F (it's a treatable disease with a high-rate remission)
54
Cure rate of ALL in percentage
90%
55
IS remission and cure rates higher in adults with ALL?
NO (they're lower)
56
Main type of treatment in ALL
chemotherapy
57
The majority of ALL cases are of what origin?
B-cell origin
58
B-cell ALL can be further classified based on their stage of maturation. Enumerate the stages.
- Early-stage (pre-pro-B or pro B) B-cell ALL - Intermediate-stage (or common) B-cell ALL - Pre–B cell ALL
59
The blasts are typically positive for? (the CD markers and such)
- terminal deoxynucleotidyl transferase (TdT) - HLA-DR - CD79a - CD19 - CD22 - CD34 (variable)
60
The CD10 marker is originally named as
Common Acute Lymphoblastic Leukemia Antigen (CALLA)
61
T/F: The CD10 is found in early stages of B-cell differentiation
T
62
Slightly more mature B cells, at the pre–B-cell stage, express what?
cytoplasmic µ immunoglobulin heavy chain
63
T/F: Slightly more mature B cells, at the pre–B-cell stage, is also positive for surface immunoglobulin
F (they're negative)
64
Myeloid-associated antigens
- CD13 - CD15 - CD33
65
About how many percent of ALL cases are of precursor T-cell origin?
10-20%
66
TALL occur most commonly in what population?
teenaged males
67
ALL cases of precursor T-cell origin occur most commonly in teenaged males as?
lymphoma or as leukemia/lymphoma combined
68
T-cell precursor ALL is characterized by presence of
- TdT - CD7 - CD3 - CD4 - CD8 - CD2 (variable) - CD5 (variable)
69
What do you call it when malignant cells contain more than 46 chromosomes?
hyperdiploidy
70
Identify which of the 2 is associated with good prognosis and poorer prognosis: hyperdiploidy hypodiploidy
hyperdiploidy - good prognosis hypodiploidy - poorer prognosis
71
The most common translocation in ALL of B-cell origin
t(12:21)(p13;q22)
72
t(12:21)(p13;q22) is known as
ETV6/RUNX1 (or TEL-AML-1)
73
ETV6/RUNX1 (or TEL-AML-1) is associated with what prognosis in children?
excellent prognosis
74
The most common leukemia in adults living in Western countries of the world.
Chronic Lymphocytic Leukemia (CLL)
75
T/F: The WHO considers CLL and SLL a single disease with different clinical presentations.
T
76
CLL primary occurs in patients over what age?
over 50 yrs old
77
male to female predominance of CLL
2-to-1 male-to-female predominance
78
79
The malignant clone in CLL/SLL is derived from what cells?
small mature B lymphocytes
80
T/F: The cells in CLL appear to be cytologically normal but are dysfunctional.
T
81
This is prominent early in the CLL disease
Lymph node enlargement
82
Anemia and thrombocytopenia are usually present/absent(?) at the time of CLL diagnosis
absent
83
In CLL, as the malignant lymphocytes continue to increase in number, replacement of normal elements in the bone marrow leads to what?
anemia and thrombocytopenia
84
Treatment of CLL includes monoclonal antibodies directed against what antigen?
CD20 antigen
85
T/F: CLL is compatible with a long survival, as treatments can help control or reduce symptoms and they are curative.
F (long survival due to treatments that control symptoms but not curative)
86
The malignant cells in CLL/SLL express the B-cell markers
- CD19 - CD20 (weaker expression) - CD23 - CD200 - CD5 - surface immunoglobulin
87
TP53 tumor suppressor gene is located in what chromosome?
chromosome 17
88
Patients whose malignant B cells have undergone somatic hypermutations in the variable immunoglobulin heavy-chain genes have worse/better(?) prognosis than those whose cells have not undergone such mutations.
better
89
A rare, slowly progressive disease characterized by infiltration of the bone marrow and spleen by leukemic cells without the involvement of lymph nodes.
Hairy-cell leukemia
90
Hairy cell leukemia is predominant in what sex?
male
91
hairy cell leukemia is most often seen in individuals over what age?
over 50 years of age
92
Hairy cell leukemia patients usually present with these diseases because of bone marrow infiltration.
Bone marrow disease and Pancytopenia
93

IMMUNOLOGY AND SEROLOGY (11 decks)

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