Plasma cell disorders Flashcards
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
What is a plasma cell clone?
a group of plasma cells that secrete identical immunoglobulin
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
List three laboratory tests that can be used to assess immunoglobulins
Protein electrophoresis (serum and urine) Immunofixation (serum and urine) Quantitative assays (serum)
Electrophoresis is particularly useful in detecting ________
monoclonal immunoglobulin
The “gamma peak” on SPEP or UPEP is made up of :
IgM and IgG and may contain IgA
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
What is Bence Jones protein?
Monoclonal light chains excreted in the urine- found only on UPEP, may result in SMALLER gamma peak in SPEP
What information can be gathered from immunofixation
immunoglobulin class type of light chain
Multiple myeloma is a malignancy of ______ cells
plasma
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
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
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
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
Production of monoclonal IgM is characteristic of ______ and is very rare in multiply myeloma
Waldenstrom macroglobulinemia
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
What radiologic findings are seen in multiple myeloma
osteoporosis
osteolytic lesions
fractures
compression of vertebral bodies
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
What therapies are used in multiple myeloma
chemotherapy + radiation
bisphosphonates to slow bone destruction
pain control
avoid nephrotoxic drugs
What is the prognosis for multiple myeloma?
poor- about 5 years
death due to renal failure, hypercalcemia, bone marrow failure and complications like infection
_______ often progress to MM. Development of MM in patients with __________ after radiation treatment is
uncommon.
osseous plasmacytomas
extraosseous plasmacytoma
___________ 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
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
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
