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Flashcards in Plasma cell disorders Deck (27):
1

Plasma cells are normally found in _______ and are not normally found in the ______

Plasma cells are lymph nodes, bone marrow, mucosa, spleen, and other tissues with lymphoid cells

not normally found in the blood

2

What is a plasma cell clone?

a group of plasma cells that secrete identical immunoglobulin

3

The Fc portion of the immunoglobulin molecule is formed from the _______ and determines the properties of each class of antibody, including ability to bind complement and attach to effector cells

two heavy chains

4

List three laboratory tests that can be used to assess immunoglobulins

Protein electrophoresis (serum and urine)
Immunofixation (serum and urine)
Quantitative assays (serum)

5

Electrophoresis is particularly useful in detecting ________

monoclonal immunoglobulin

6

The "gamma peak" on SPEP or UPEP is made up of :

IgM and IgG and may contain IgA

7

What is the significance of a narrow gamma peak on SPEP or UPEP

indicates the presence of an abnormally large amount of
immunoglobulin that is homogeneous in terms of amino acid composition and structure; this in turn implies the presence of an abnormally large clone of plasma cells

Referred to as M protein, M spike, or paraprotein

8

What is Bence Jones protein?

Monoclonal light chains excreted in the urine- found only on UPEP, may result in SMALLER gamma peak in SPEP

9

What information can be gathered from immunofixation

immunoglobulin class
type of light chain

10

Multiple myeloma is a malignancy of ______ cells

plasma

11

List four causes that underly the clinical problems seen in multiple myeloma

Bone marrow infiltration by the malignant plasma cells
Production of large quantities of functionless monoclonal immunoglobulin and/or monoclonal free light chain.
Decreased production of normal immunoglobulins
Bone destruction by cytokines produced by the malignant plasma cells

12

Describe the two hit hypothesis for multiple myeloma

Antigenic stimulation causes expansion of multiple benign clones
Mutagenic events in a dividing cell cause malignant transformation.

The primary
mutagenic event is thought to be a translocation involving an immunglobulin gene and an oncogene, similar to abmutations found in some B-cell lymphomas

13

List clinical signs and symptoms of multiple myeloma

anemia, neutorpenia, thrombocytopenia due to replacement of normal marrow with malignant plasma cells
extramedullary extension of plasma cells form tumors in soft tissue (plasmacytomas, often compress spinal cord)
abnormal immunoglobulin accumulates in serum- hyperviscosity of blood
light chain is filtered into the urine- causes renal damage
amyloid formation
bone pain, lytic bone lesions
renal insufficiency
mental status change
hypercalcemia
immunodeficiency

14

Describe lab findings seen in multiple myeloma

anemia or pancytopenia
Roleaux formation on smear
elevated total serum protein
hypercalcemia
ESR very high
hyperviscosity of blood
SPEP- low levels of normal immunoglobulin
UPEP- monoclonal light chains

15

Production of monoclonal IgM is characteristic of ______ and is very rare in multiply myeloma

Waldenstrom macroglobulinemia

16

What will a bone marrow biopsy and aspirate show in multiple myeloma?

Increased plasma cells with abnormal morphology
Kappa or lambda restriction in light chains
Translocations involving H chain on 14q23
possible amyloid deposition around vessels

17

What radiologic findings are seen in multiple myeloma

osteoporosis
osteolytic lesions
fractures
compression of vertebral bodies

18

List diagnostic criteria of multiple myeloma

M protein in urine or serum
bone marrow >10% plasma cells or extramedullary plasmacytoma
CRAB

smoldering myeloma= lab findings but no symptoms

19

What therapies are used in multiple myeloma

chemotherapy + radiation
bisphosphonates to slow bone destruction
pain control
avoid nephrotoxic drugs

20

What is the prognosis for multiple myeloma?

poor- about 5 years
death due to renal failure, hypercalcemia, bone marrow failure and complications like infection

21

_______ often progress to MM. Development of MM in patients with __________ after radiation treatment is
uncommon.

osseous plasmacytomas
extraosseous plasmacytoma

22

___________ is a low grade malignancy of lymphoplasmacytic cells, which characteristically secretes monoclonal IgM

Waldenstrom’s macroglobulinemia

** WM is a low grade NHL, MM is a more aggressive malignancy of plasma cells

23

List some clinical complications associated with Waldenstrom's macroglobulinemia

- can infiltrate lymph nodes and spleen (unlike MM)
- hyperviscosity due to high IgM
- sometimes associated with cold agglutinins or cryoglobulinemia
- anemia, bleeding

NO bone lesions or renal disease

24

Describe lab findings in Waldenstrom's macroglobulinemia

Anemia or pancytopenia
Rouleaux formation
agglutination if IgM is acting as a cold agglutinin
Monoclonal IgM on immunofixation

25

What therapies are used for Waldenstrom's macroglobulinemia

chemotherapy
plasmapharesis

26

____ light chain is associated with AL amyloid

Lambda

27

What is MGUS?

a patient has monoclonal gammopathy but MM,
lymphoma, and amyloidosis have been ruled out.

sometimes progresses to multiple myeloma

does not require treatment