B3.043 Plasma Cell Myeloma Flashcards

1
Q

what are plasma cell neoplasms?

A

neoplasms of terminally differentiated, Ig secreting plasma cells
plasma cell proliferation and monoclonal Ig (M-component)

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2
Q

who primarily develops plasma cell neoplasms?

A

middle aged and older adults

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3
Q

what are 3 types of plasma cell neoplasms

A

plasma cell myeloma (multiple myeloma)
monoclonal gammopathy of undetermined significance (MGUS)
primary amyloidosis
-lamba Ig chain folds and causes organ dysfunction

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4
Q

define plasma cell myeloma

A

multifocal destructive infiltration of plasma cells in bone marrow

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5
Q

define MGUS

A

M-component without features of myeloma

1% per year transformation into plasma cell myeloma

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6
Q

what are some clinical features of plasma cell myeloma`

A
CRAB criteria
-bone pain, fractures, hypercalcemia
-Renal disease
-Anemia, thrombocytopenia
-punched out Bone lesions
infections
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7
Q

primary cause of death in people with plasma cell myeloma

A

infection or renal disease

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8
Q

what are some features of plasma cell myeloma in the blood

A

monoclonal immunoglobulin or light chain
-spike on serum protein electrophoresis
serum free light chains
Rouleaux formation (red cells line up in columns bc protein content is high)

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9
Q

describe the appearance of electrophoresis in clonal vs of variable specimens

A

clonal will separate into a well defined band
-10-20% of chains need to be clonal to make a defined band/spike on a graph
variable will appear to be a smear

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10
Q

plasma cell myeloma findings in urine

A

Bence Jones protein (monoclonal free light chain)

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11
Q

what is included in gamma on a serum protein electrophoresis

A

Igs, fribrinogen, c-reactive protein

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12
Q

what would an increased gamma signify if it was polyclonal

A

non-specific
chronic inflammation
liver disease

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13
Q

what would a decreased gamma signify

A

hypogammaglobulinemia

consider further evaluation for a paraprotein

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14
Q

what would a monoclonal band signify

A

M-protein (paraprotein)

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15
Q

what is immunofixation electrophoresis?

A

detection of M-protein
-higher sensitivity
determination of Ig class
-G,A,M,K,L, tested (doesn’t detect E or D paraproteins)

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16
Q

how are serum free light chains measured

A

measurement by nephelometry using an antibody to a region of the light chain normally not accessible in intact Ig molecules
high sens

17
Q

why is the K/L ratio useful

A

can confirm monoclonality

determines light chain class

18
Q

why are K and L levels useful

A

disease progression and monitoring

19
Q

what is the function of 24 h urine studies

A
detection of monoclonal free light chains (MFLC) (Bence Jones protein)
total protein
urine protein electrophoresis
-low sense
urine immunofixation
-high sense
20
Q

what 3 things are required for plasma cell disorder diagnosis

A
  1. M-protein
    - direct measurement from SPE
    - serum free light chains
    - urinary MFLC/BJP
  2. bone marrow clonal plasma cells
    - flow cytometry
    - K/L immunohistochemistry
  3. related organ or tissue impairment
    - hypercalcemia
    - renal insufficiency
    - anemia
    - bone lesions
21
Q

describe plasma cell myeloma findings in the bone marrow

A

> 10% monoclonal plasma cells
nodules or diffuse sheets of plasma cells
CD38+
CD138+

22
Q

clinical features of plasma cell myeloma: bone related

A
bone pain
fractures
hypercalcemia
punched out bone lesions
osteolytic factors from plasma cells - increased osteoclastic activity, decreased osteoblastic activity
23
Q

clinical features of plasma cell myeloma: renal

A
cast nephropathy- light chain aggregates form tubular casts
direct tubular toxicity
amyloidosis (AL type)
light chain deposition in interstitium
hypercalcemia, hyperuricemia
24
Q

clinical features of plasma cell myeloma: anemia, thrombocytopenia

A

bone marrow replacement by plasma cells

25
Q

clinical features of plasma cell myeloma: infections

A

impaired humoral immunity

26
Q

what is the best way to treat plasma cell myeloma

A

induction followed by hematopoietic stem cell transplant, if possible

27
Q

drugs/therapies for multiple myeloma

A
immunomodulatory agents
proteasome inhibitors
steroids
cytotoxic drugs (cyclophosphamide)
monoclonal Ab (against CD 38)
HDAC inhibitors
28
Q

therapies for bone disease

A

bisphosphonates

radiation

29
Q

therapies for hypercalcemia

A

hydration

bisphosphonates

30
Q

therapies for hyperviscosity

A

plasmapheresis

31
Q

therapies for anemia

A

transfusion

EPO

32
Q

therapies for infection

A

vaccines

antimicrobial prophylaxis