CC May 2017 Monoclonal Gammopathy of Undetermined Significance (MGUS)

echoecho's version from 2017-05-15 14:34


Question Answer
Define monoclonal gammopathy of undetermined significance (MGUS)?is an asymptomatic, premalignant plasma cell or lymphoplasmacytic disorder
*** True or false, MGUS is very common?true
*** How many cases per 100,000 men at age 50? At age 90?1) 120 cases 2) 530 cases
*** What is the prevalence if > 50 y.o? What is the prevalence if > 85 y.o.?3.2%; 7.5%
*** Women are less commonly affected, with about ____% the number of cases seen in men?65%
*** Blacks have a ___ to ___ fold increase in the diagnosis of MGUS and multiple myeloma compared with whites?2; 3
True or false? MGUS is typically an incidental finding during evaluation for other disorders?true
MGUS is ____ times more common than active multiple myeloma?80
The average age of diagnosis for MGUS is?70 yo
It is felt that all patients with active multiple myeloma have evolved through the sequence of _____ and smoldering myeloma before overt multiple myeloma is diagnosed?MGUS
MGUS begins ___ to ___ years before the diagnosis of active multiple myeloma is made?8; 10
The definition of MGUS is serum monoclonal protein (IgG, IgA, IgD or IgM) < ____ g/dL?3
The definition of MGUS is bone marrow with less than ___% monoclonal plasma cells or lymphoplasmacytic cells?10%
The definition of MGUS is no evidence of what 5 conditions?1) hypercalcemia 2) anemia 3) lyric bone lesions 4) renal insufficiency 5) hyperviscosity
List the work-up to be done for MGUS?1) CBC 2) metabolic panel 3) ESR 4) serum and urine electrophoresis 5) skeletal survey to r/o lyric lesions 6) a bone marrow biopsy
What is the treatment for MGUS?1) no treatment 2) must have careful follow-up for early detection of disease progression
Is frequency of surveillance correlated with risk?yes
List the risk factors for transformation of MGUS?1) monoclonal protein > 1.5 g/dL 2) a non-IgG monoclonal protein 3) abnormal free light chain ratio (kappa/lambda) 4) percentage of plasma cells in bone marrow (<5% vs 6-10%) 5) advanced age 6) suppression of uninvolved immunoglobulins
A simplified risk stratification using only what three parameters will accurately predict disease progression over a 120 year period?1) monoclonal protein level > 1.5 g/dL 2) non-IgG MGUS 3) abnormal free light chain ratio
*** With no risk factors, only ___% of patients have progression? Evaluation is neede only every ___ years?1) 5% 2) 5 years
*** With one risk factor, the progression risk is ___%? Progression risk rises to ___% with 2 risk factors?21%; 37%
*** Patients with 1 or 2 risk factors should be evaluated every ___ months?12
*** A patient with all 3 risk factors has a ___% chance of progression and should be followed every _________ to _________ months?58%; 6; 9
Overall, the rate of transformation of MGUS patients to active myeloma is about ___% per year?1%
IgM MGUS represents ____% of MGUS?15%
IgM MGUS is commonly associated with what condition?lymphoplasmacytic lymphoma (Waldenstrom's Macroglobulinemia)
Is IgM MGUS a IgM myeloma?no; IgM myeloma is rare and accounts for < 1% of IgM MGUS
Light-chain-only MGUS accounts for about ___% to ___% of MGUS patients and has a lower incidence of progression to more significant disease?10-15%
How is the free light chain ratio calculated?at intervals according to risk
How are Bence Jones monoclonal light chains measured?in the urine
If Bence Jones proteins are increasing, therapy is indicated to protect what organs from damage?kidneys
What is the POEMS syndrome?1) Polyneuropathy 2) Organomegaly 3) Endocrinopathy 4) Monoclonal protein 5) Skin changes
Describe what POEMS syndrome is?it is a rare plasma cell dysplasia with multiple systemic symptoms and is not likely found in an asymptomatic patient
What are the causes of MGUS?unknown
Is there strong data that first-degree relatives of patients with MGUS or MM are at increased risk of developing a plasma cell disorder?yes
Survivors of what exposure as well as those in what occupational contact are also at greater risk to develop a plasma cell dysplasia?1) survivors of nuclear radiation exposure 2) petroleum products and pesticides (Agent Orange)
*** Most patients with MGUS have no symptoms or problems related to the low level of circulating monoclonal proteins, true or false?true
*** Is there an association of MGUS with proliferative glomerulonephritis that can respond to myeloma-type treatment?yes
*** Is there a modestly increased risk of neuropathy, especially with IgM MGUS?yes
*** MGUS patients are also more likely to have what types of diseases?1) venous thromboembolism 2) bacterial infections (pneumonia, cellulitis, viral infections like influenza)
What antibodies can be found in the serum of 10-15% of patients with neuropathy?serum anti-myelin associated glycoproteins antibodies (MAG)
What is smoldering myeloma?it is another stage in the development of myeloma thought to be next in a sequence between MGUS and active multiple myeloma (MM)
Smoldering myeloma describes a group of patients who have either OR both > ____ g/dL of monoclonal protein and > _________% plasma cells in the bone marrow?3; 10
*** Smoldering myeloma patients are asymptomatic, what does this mean?no bone pain, hypercalcemia, anemia or renal dysfunction
*** Smoldering myeloma patients evolve to active multiple myeloma (MM) much faster than patients with MGUS, with a risk for developing MM of ___% yearly for the first 5 years, ___% yearly for the next 5 years, and ___% yearly after that?10%; 3%; 1%
Fifteen years after a smoldering myeloma diagnosis, what % of patients will still be stable and not require treatment?20-30%
In a recent change in treatment, patients with > ____% myeloma cells in the bone marrow, a free light chain ratio > ____ or more than one asymptomatic bone lesion on PET or MRI imaging are restratified as active myeloma and treatment is started?60%; 100
Although most cases of MGUS are sporadic, those with multiple first-degree relatives with the syndrome may have certain cytogenetic abnormalities including what?1) t(4:14) 2) gain of 1q21 3) deletion of 17p 4) hypodiploidy
The above patient group should be reevaluated more frequently ( every ___ to ___ months) so treatment for active myeloma can be started in a timely manner?2; 4
Has a survival benefit been proven and a standard of care proposed in trials looking at myeloma treatment in patients with smoldering myeloma?no
*** SUMMARY = Define MGUS?a common, idiopathic, asymptomatic plasma cell dyscrasia usually found incidentally during investigation of other problems
*** SUMMARY = MGUS appears to be part of a spectrum that many infrequently lead to ____ _____ and must be distinguished from that invariably fatal disease?multiple myeloma
*** SUMMARY = Frequence of monitoring patients with MGUS depends on what?variety of risk factors seein in their hematologic profile