Immunoproliferative Disorders Flashcards
Monoclonal gammopathy
what causes it
Results from a single clone of plasma cell producing elevated levels of a single Ig type
What are monoclonal proteins also called
M protein, para protein
What are the two main kinds of monoclonal gammopathies
Multiple Myeloma or WM
What is a polyclonal gammopathy
A secondary disease
Elevated production of 2 or more Ig types bye several plasma cell clones
What are general characteristics of monoclonal gammopathies
not the symptoms the internal characteristics
Suppressed Igs
Dysfunctional T cell response
String peak/band in M protein region of electrophoresis
What are common characteristics of polyclonal gammopathies
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
What is the pathophysiology of MM
process leading to
Complex karyotype abnormalities leading to: dysfunctional T cell response, plasma cells incapable of apoptosis (IL6 inactive), over production of Bence Jones proteins
Skeletal abnormalities of MM
Bone pin caused by overactive osteoclasts forming lytic punch out regions and general skeletal destruction
Renal issues of MM
From BJ proteins blocking kidney filtration and direct tubular injury from bence jones proteins
What are the immunological manifestations of MM
Defect in humoral immunity, increased serum protein concentrations, PLT issues causing excess bleeding
How does MM effect T cells
Reduced T helper and increased cytotoxic Ts
What are bence jones proteins
Incomplete, single polypeptide light chains of IgM, IgG, or IgA of either kappa or lambda variations
Etiology of WM
what is the cause
B cell disorder where plasma cells infiltrate BM and over produce IgM
Signs and symptoms of WM
Intracellular buildup of high levels of IgM leading to: cold hypersensitivity, excess bleeding, increased blood viscosity and skin lesions
Describe MGUS
Presence of monoclonal proteins without symptoms of other monoclonal gammopathies
Most commonly proliferations of IgG or IgM
What are the characteristics of MGUS
Absence of bone pain
Anemia
Hypercalcemia
Renal insufficiency
Serum proteins <3 g/dL
What are light chain diseases
10-15% of monoclonal gammopathies
Only kappa or lambda light chains and BJ proteins produced
What are heavy chain diseases
Monoclonal proteins made of only HC portion
Alpha chain most common
Franklins disease =gamma chain
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
c. multiple myeloma
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
d. infectious disease
pt with MM have defects in
a. cellular immunity
b. humoral immunity
c. synthesis of normal Igs
d. both B and C
d. both b and C
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
d. all of the above
M proteins are associated with all of the following except
a. MM
b. plasmacytoma
c. malignant lymphoproliferative disease
d. lymphoma
d. lymphoma
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
d. all of the above
in light chain diseases only what two monoclonal light chains are synthesized by a single cell clone
kappa and lambda
most pt w/ MM manifest
a. bone pain
b. acute renal failure
c. no symptoms
d. hepatomegaly and splenomegaly
a. bone pain (in 90%)
renal failure can happen in 5-10%
hepatomegaly (20%) and splenomegaly (5%)
pt with WM exhibit abnormally large amoutns of
a. IgM
b. IgG
c. IgE
d. IgA
a. IgM
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
d. all of the above
