Immunoproliferative Disorders Flashcards

1
Q

Monoclonal gammopathy

what causes it

A

Results from a single clone of plasma cell producing elevated levels of a single Ig type

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2
Q

What are monoclonal proteins also called

A

M protein, para protein

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3
Q

What are the two main kinds of monoclonal gammopathies

A

Multiple Myeloma or WM

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4
Q

What is a polyclonal gammopathy

A

A secondary disease
Elevated production of 2 or more Ig types bye several plasma cell clones

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5
Q

What are general characteristics of monoclonal gammopathies

not the symptoms the internal characteristics

A

Suppressed Igs
Dysfunctional T cell response
String peak/band in M protein region of electrophoresis

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6
Q

What are common characteristics of polyclonal gammopathies

A

Common protein abnormality
Increase of 2+ Igs from multiple plasma clones
Normal electrophoresis staining more intensely
Consists of 1+ Hc classes and both Lc classes

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7
Q

What is the pathophysiology of MM

process leading to

A

Complex karyotype abnormalities leading to: dysfunctional T cell response, plasma cells incapable of apoptosis (IL6 inactive), over production of Bence Jones proteins

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8
Q

Skeletal abnormalities of MM

A

Bone pin caused by overactive osteoclasts forming lytic punch out regions and general skeletal destruction

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9
Q

Renal issues of MM

A

From BJ proteins blocking kidney filtration and direct tubular injury from bence jones proteins

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10
Q

What are the immunological manifestations of MM

A

Defect in humoral immunity, increased serum protein concentrations, PLT issues causing excess bleeding

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11
Q

How does MM effect T cells

A

Reduced T helper and increased cytotoxic Ts

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12
Q

What are bence jones proteins

A

Incomplete, single polypeptide light chains of IgM, IgG, or IgA of either kappa or lambda variations

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13
Q

Etiology of WM

what is the cause

A

B cell disorder where plasma cells infiltrate BM and over produce IgM

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14
Q

Signs and symptoms of WM

A

Intracellular buildup of high levels of IgM leading to: cold hypersensitivity, excess bleeding, increased blood viscosity and skin lesions

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15
Q

Describe MGUS

A

Presence of monoclonal proteins without symptoms of other monoclonal gammopathies
Most commonly proliferations of IgG or IgM

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16
Q

What are the characteristics of MGUS

A

Absence of bone pain
Anemia
Hypercalcemia
Renal insufficiency
Serum proteins <3 g/dL

17
Q

What are light chain diseases

A

10-15% of monoclonal gammopathies
Only kappa or lambda light chains and BJ proteins produced

18
Q

What are heavy chain diseases

A

Monoclonal proteins made of only HC portion
Alpha chain most common
Franklins disease =gamma chain

19
Q

polyclonal gammopathies can be exhibited as a secondary manifestation of all the following except
a. chronic infection
b. chronic liver disease
c. multiple myeloma
d. rheumatoid connective disease

A

c. multiple myeloma

20
Q

What is the most frequent cause of death in a pt with multiple myeloma
a. skeletal destruction
b. chronic renal failure
c. neurologic disorders
d. infectious disease

A

d. infectious disease

21
Q

pt with MM have defects in
a. cellular immunity
b. humoral immunity
c. synthesis of normal Igs
d. both B and C

A

d. both b and C

22
Q

what is the most consistent immunologic feature of MM
a. synthesis of dysfunctional single monoclonal proteins
b. synthesis of Ig chains or fragments
c. presence of M protein in serum/urine
d. all of the above

A

d. all of the above

23
Q

M proteins are associated with all of the following except
a. MM
b. plasmacytoma
c. malignant lymphoproliferative disease
d. lymphoma

A

d. lymphoma

24
Q

monoclonal gammopathies involve elevated lvls of a single class and type of Ig referred to as
a. monoclonal protein
b. M protein
c. paraprotein
d. all of the above

A

d. all of the above

25
Q

in light chain diseases only what two monoclonal light chains are synthesized by a single cell clone

A

kappa and lambda

26
Q

most pt w/ MM manifest
a. bone pain
b. acute renal failure
c. no symptoms
d. hepatomegaly and splenomegaly

A

a. bone pain (in 90%)

renal failure can happen in 5-10%
hepatomegaly (20%) and splenomegaly (5%)

27
Q

pt with WM exhibit abnormally large amoutns of
a. IgM
b. IgG
c. IgE
d. IgA

A

a. IgM

28
Q

in MGUS represents a
a. monoclonal protein in pt with no features of MM or related
b. a disorder that can evolve into a malignant monoclonal gammopathy
c. serum monoclonal protein concentration less then 3 g/dL
d. all of the above

A

d. all of the above