Immunoproliferative disorders Qs

studied byStudied by 3 people
5.0(1)
learn
LearnA personalized and smart learning plan
exam
Practice TestTake a test on your terms and definitions
spaced repetition
Spaced RepetitionScientifically backed study method
heart puzzle
Matching GameHow quick can you match all your cards?
flashcards
FlashcardsStudy terms and definitions

1 / 166

flashcard set

Earn XP

Description and Tags

167 Terms

1
Which of the following is a type of B-cell tumor that arises from a germinal center **B cell** and does not express **antigen receptor**?

a. multiple myeloma

b. acute lymphoblastic leukemia (ALL)

c. Hodgkin’s lymphoma

d. follicular center cell lymphoma
c
New cards
2
You are a researcher interested in learning more about B-cell **anergy**. You would like to obtain a B-cell line from a tumor that has a single receptor specificity and resembles a **naive B cell**. Which tumor type would most likely serve your needs?

a. acute lymphoblastic leukemia (ALL)

b. mantle cell lymphoma

c. multiple myeloma

d. Hodgkin’s lymphoma
b
New cards
3
Which fit the following description non-germline B-cell receptor gene, with CD19, Igα/Igβ, and Pax-5 expression, and surface IgM molecules?

a. chronic lymphocytic leukemia

b. pre-B-cell leukemia

c. acute lymphoblastic lymphoma (ALL)

d. Burkitt’s lymphoma
a, d
New cards
4
An individual presents in the clinic with complaints of general achiness and fatigue. When her blood is checked for infection, it is found that her antibodies are overwhelmingly targeting a single antigen. What would you expect to find in her B-cell populations?

a. only cells that express IgM isotypes

b. a repertoire of normal depth and breadth

c. clonal population derived from a single ancestral cell

d. circulating pro-B cells
c
New cards
5
Which one of the following statements about the types of monoclonal M bands found by electrophoresis of serum indicates multiple myeloma?

a. An M band that moves in the beta position on protein electrophoresis

b. M band found in alpha position

c. Heavy deposit at the original suggesting large load of monoclonal protein

d. All of the above

e. None of the above
d
New cards
6
What is multiple myeloma?

a.      Cancer of the bone marrow

b.      Cancer of the plasma cells

c.       Cancer of the blood

d.      Cancer of the lymphatic system

e. Cancer of the bone
b
New cards
7
Bence Jones proteins are:

a.      Fragments of transferrin

b.      Polyclonal free light chains

c.       Monoclonal free light chains

d.      Beta 2-microglobulin

e.      Prostaglandins
c
New cards
8
Which one of the following statements about patients with monoclonal serum bands is true?

a.      All will develop a lymphoid malignancy within 3 years

b.      All should be followed up once a year, with quantitation of the band and examination of the urine

c.       All should have a skull X-ray

d.      Over 90% will have frank myeloma

e.      Only those with renal failure need urine examination for monoclonal free light chains
b
New cards
9
How is multiple myeloma diagnosed?

a.      Bone Marrow

b.      Serum Protein Electrophoresis

c.       Immunofixation

d.      X-rays

e.      All on the above
e
New cards
10
How is the prognosis determined for a patient with multiple myeloma?

a.      Stage of disease

b.      Age of patient

c.       Kidney function

d.      Cytogenetic markers

e.      All of the above
e
New cards
11
Yasuo Yamagata, 63 years old, experienced severe back pain for several weeks before visiting his family physician. He also complained of fatigue and looked pale. Blood analysis revealed a red blood cell count of 3.2 × 106/μl (normal 4.2–5.0 × 106/μl), a white blood cell count of 2800/μl (normal 5000/μl), neutropenia (low levels of neutrophils in the blood), a sedimentation rate of 30 mm/h (normal
b
New cards
12
Which two linages do B-cell tumours arise from?
B-1, B-2
New cards
13
What proof do B-cell tumours have to their derivation from the same ancestral gene?
as an identical immunoglobulin-gene rearrangement
New cards
14
Despite individual B-cell tumours being homogenous what do B-cell tumours have that reflects multiplicity of rearrangements found in the normal B cells of a healthy person?
different rearrangements
New cards
15
Where do tumour characteristics come from?
retain characteristics of the cell type from which they arose
New cards
16
Where do tumours derived from mature B cells grow?
follicles of lymph nodes
New cards
17
Where do plasma cell tumours grow?
bone marrow
New cards
18
What are plasma-cell tumours called?
myelomas
New cards
19
What are tumors derived from mature naive B cells grow in the follicles of lymph nodes called?
follicular centre cell lymphoma
New cards
20
What was one of the first tumours to be successfully treated by radiotherapy?
Hodgkin’s
New cards
21
Why do Hodgkins tumours no longer have antigen receptors?
somatic mutation
New cards
22
What morphology do Hodgkins tumours have?
Reed-Sternberg cells
New cards
23
What are the two forms of Hodgkins?
In some people the disease is dominated by the proliferation of nonmalignant T cells that respond to the tumor cells, whereas others have Reed–Sternberg cells as well as more normal-looking malignant B cells.
New cards
24
Where did the very first sequences of heavy and light chains were obtained from?
multiple myeloma
New cards
25
What tumours have rearranged **B-cell receptor** genes and resemble naive **B cells**?
Mantle cell lymphoma
New cards
26
What lymphoma arises from transformation of B-cells in germinal centre?
Hodgkin’s
New cards
27
Which lymphoma do not express immunoglobulin?
Hodgkin’s
New cards
28
Which tumours resemble lymphoid progenitor cells and would not have a non-germline **B-cell receptor** gene?
ALL
New cards
29
What tumours resemble activated or memory **B cells** with non-germline immunoglobulin genes and surface expression of the receptor and signaling complex?
CLL
New cards
30
What tumours resemble germinal center **B cells** with non-germline immunoglobulin genes and surface expression of the receptor and signaling complex?
Burkitts lymphoma
New cards
31
Why do B-cells immunoglobulin light chains would be in their **germline configuration** whereas their heavy-chain genes would be rearranged?
lack a functional B-cell receptor
New cards
32
Where are ALL and Pre-B cell leukaemia tumours found?
Bone marrow and blood
New cards
33
Where are CLL, mantle cell lymphoma, follicular center cell lymphoma, Hodgkin’s and Waldenstrom’s macroglobunemia tumours located?
Periphery
New cards
34
Where are multiple myeloma tumours located?
bone marrow
New cards
35
What leukaemia has various isotypes and originates in plasma cells?
Multiple myeloma
New cards
36
Which tumour is found in IgM-secreting B cells?
Waldenstrom’s macroglobunemia
New cards
37
Which tumour arises from mature memory B cells?
follicular centre cell lymphoma
New cards
38
Which tumour originates from Pre-B cells?
Pre-B cell leukaemia
New cards
39
What is the status of Ig V genes in MM?
Mutated, no variability within clone
New cards
40
What is the status of Ig V genes in Waldenstrom’s macroglobunemia?
Mutated, no variability within clone
New cards
41
What is the status of Ig V genes in Hodgkin’s?
Mutated +/- intraclonal variability
New cards
42
What is the status of Ig V genes in follicular centre cell lymphoma and Burkitts?
Mutated intraclonal variability
New cards
43
What is the status of Ig V genes in CLL?
Usually unmutated
New cards
44
What is the status of Ig V genes in mantle cell?
Unmutated
New cards
45
What is the status of Ig V genes in Pre-B cell?
Unmutated
New cards
46
What is the status of Ig V genes in ALL?
Unmutated
New cards
47
Gammopathy
a disorder characterised by abnormality of gamma globulins
New cards
48
Hypergammaglobulinaemias
the overproduction of more than one class of immunoglobulins by plasma cells
New cards
49
Monoclonal gammopathy arises from?
single clone of plasma cells producing high levels of a single class and type of antibody (monoclonal protein/M protein/paraprotein)
New cards
50
Monoclonal gammopathy examples?
MM, Waldenstrom primary macroglobulinaemia
New cards
51
What do XS light chains κ (kappa) free light chains secreted as?
monomers
New cards
52
What do XS light chains λ (lambda) free light chains secreted as?
dimers or polymers
New cards
53
Where do XS lights chains enter?
kidney
New cards
54
How do XS light chains enter the kidney through the glomerulus and PCT?
due to low molecular weight
New cards
55
Where are XS free light chains reabsorbed and degraded into smaller peptides?
PCT
New cards
56
What is the normal amount of light chains dialy reaching distal tubule and in urine?
1-10 mg
New cards
57
When are much higher levels of light chains is seen in the urine detected?
Monoclonal gammopathies
New cards
58
Why are much higher levels of light chains is seen in the urine detected in monoclonal gammopathies?
capacity of PCT are overwhelmed
New cards
59
What allows plasma cells to overcome growth restraints?
chromosome abnormalities
New cards
60
Why alongside the extensive growth replication is a discrete band by electrophoriesis seen?
the cell secretes its programmed immunogloubulin isotype (class) in such large amounts
New cards
61
What is secreted by growth of abnormal plasma clones?
monoclonal antibody, paraprotein or M protein
New cards
62
How is monoclonal gammopathy defined by?
monoclonal immunoglobulin in the serum and/or urine
New cards
63
Examples of monoclonal gammopathy


—Monoclonal gammopathy of undetermined significance (MGUS)



—Myeloma/Multiple Myeloma



—Waldenstrom primary macroglobulinaemia



—Light-chain disease



—Heavy-chain disease

New cards
64
Why do patients with MGUS not have symptoms?
due to the monoclonal gammopathy
New cards
65
What monoclonal band is seen in MGUS?
less than 30g/L
New cards
66
What is the % of plasma cells in BM?
Less than 10%
New cards
67
How does the incidence of MGUS change with age?
Increases from 1% in 50y to 10% in 80y
New cards
68
Who has the highest incidence of MGUS?
African-Caribbean patients
New cards
69
How many patients with MGUS transform into MM?
1%
New cards
70
How often should patients with MGUS be seen and monitored?
Annually
New cards
71
What are the characteristics of MM?


Malignant monoclonal proliferation of bone marrow marrow plasma cells, and accumulation of plasma cells in the bone marrow



Lytic bone lesions

New cards
72
How is MM detected?


Monoclonal immunoglobulin in serum and/or urine

New cards
73
Why does MM have symptoms?
Due to

Malignant monoclonal proliferation of bone marrow marrow plasma cells, and accumulation of plasma cells in the bone marrow

Lytic bone lesions

Monoclonal immunoglobulin in serum and/or urine
New cards
74
Clinical features of MM
  • bone pain,

  • anaemia,

  • infections (defects in humoral not cell immunity),

  • renal failure,

  • proteinuria in 50% of patients excreting abnormal amounts of Bence Jones proteins (light chains),

  • hyperviscosity syndrome

New cards
75
Which monoclonal gammopathy has lytic bone lesions?
MM
New cards
76
Lab features of MM
  • —electrophoresis of serum or urine shows a monoclonal protein in 90% of patients,

  • increased serum calcium,

  • low haemoglobin,

  • raised mean cell volume (MCV) and

  • increased erythrocyte sedimentation rate

New cards
77
Overall incidence of MM


Incidence is \~5-7 per 100,000; very rare
New cards
78
Paraprotein
\
this is the type of antibody that is characteristic of and produced by the malignant cells in multiple myeloma. Because this paraprotein is produced by a clone of cells it has a single light chain type, kappa or lambda.
New cards
79
What is the possible mechanism of MM?
IL-6
New cards
80
What happens to untreated patients with MM?
Rapidly progressive and usually die in a year
New cards
81
MM Ig


—IgG (60%)



—IgA (>20%)



—IgD 1%



—IgE very rare



—IgM extremely rare

10% are κ or λ light chain only     
New cards
82
non-producing myeloma
are myeloma subtype with more than 10% of monoclonal plasma cells in the bone marrow
New cards
83
What is the most common Ig secreted by MM?
IgG then IgA
New cards
84
What myeloma does not secrete Ig?
non-secreting
New cards
85
What myeloma cannot produce Ig/Ig fragments?
non-producing
New cards
86
When does proteinuria occur?


if levels of free light chains are too high
New cards
87
What forms discrete bands on electrophoresis?
light chains
New cards
88
What property of light chains leads to the formation of discrete bands on electrophoresis?
monoclonal
New cards
89
What determines whether κ or λ?
immunofixation
New cards
90
Why is renal function deteriorates the presenting symptom?
The renal tubules suffer increasing damage
New cards
91
What is found in patients with renal failure in MM?
Bence jones proteins
New cards
92
What do all secretory myelomas produce?
XS free light chains
New cards
93
What do light chain myelomas often have?
immune paresis
New cards
94
What is immune paresis?
suppression of normal immunoglobulin production by a malignant plasma clone
New cards
95
What may be the only sign of a small serum free light chain or an IgD monoclone ?
Low immunoglobulin levels
New cards
96
What is the reason for doing immunofixation on samples with low immunoglobulin even if no abnormal electrophoretic band seen in serum electrophoresis?
Low immunoglobulin levels may be the only sign of a small serum free light chain or an IgD monoclone
New cards
97
What is heavy-chain disease?
Characterized by monoclonal proteins composed of the heavy chain part of the immunoglobulin molecule
New cards
98
What is γ heavy chain disease?
Franklin disease
New cards
99
What is the commonest type of heavy chain gammopathy, and often seen in men of Mediterranean descent?
α heavy chain disease
New cards
100
What heavy-chain disease is rare?
M heavy chain
New cards

Explore top notes

note Note
studied byStudied by 8 people
1039 days ago
5.0(1)
note Note
studied byStudied by 9 people
798 days ago
5.0(1)
note Note
studied byStudied by 1 person
712 days ago
5.0(1)
note Note
studied byStudied by 36 people
1003 days ago
5.0(1)
note Note
studied byStudied by 67 people
775 days ago
5.0(1)
note Note
studied byStudied by 106 people
324 days ago
4.8(5)
note Note
studied byStudied by 12 people
858 days ago
4.0(1)
note Note
studied byStudied by 10625 people
708 days ago
4.7(60)

Explore top flashcards

flashcards Flashcard (197)
studied byStudied by 1 person
175 days ago
5.0(1)
flashcards Flashcard (55)
studied byStudied by 1 person
68 days ago
5.0(1)
flashcards Flashcard (76)
studied byStudied by 3 people
100 days ago
5.0(1)
flashcards Flashcard (53)
studied byStudied by 10 people
507 days ago
5.0(1)
flashcards Flashcard (26)
studied byStudied by 10 people
794 days ago
5.0(2)
flashcards Flashcard (50)
studied byStudied by 27 people
262 days ago
5.0(2)
flashcards Flashcard (55)
studied byStudied by 3 people
865 days ago
5.0(1)
flashcards Flashcard (20)
studied byStudied by 87 people
239 days ago
5.0(1)
robot