Plasma Cell disorders Flashcards

1
Q

What is the originating cell line that all cells are derived from?

What are the two lineages that deviate from this? –and what cells come from those?

A

pluripotent stem cells

Myelod: RBC, platelets, monocyte, neutrophils, eosinophils, basophils

Lymphoid: T cell, B cell, Plasma cell

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2
Q

Where do plasma cells develop?

A

in bone marrow and lymph nodes

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3
Q

What is multiple myeloma? (living room language)

What is it characterized by? (medical language)

A

bone marrow makes cancerous blood cells.

-neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin (M protein)

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4
Q

Who gets Multiple Myeloma?

A
  • typically a disease of older adults, median age of dx 66
  • only 10% of pts are younger than 50yeras
  • occurs in all races
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5
Q

Multiple Myeloma Presentation (signs/symptoms)

A

-signs and symptoms related to infiltration of plasma cells into the bone or other organs or to kidney damage from excess light chains.

  • lytic bone lesions
  • infection
  • fractures
  • renal failure
  • proteinuria
  • purpura
  • raynauds
  • anemia
  • Bence Jone protein
  • bone pain
  • elevated creatinine (byproduct of muscle metab, indicator of kidney function)
  • fatigue/general weakness
  • hypercalcemia
  • weight loss
  • anemia (normocytic normochromic) ** related to:
  • -bone marrow replacement
  • -kidney damage
  • -dilution in case of a large M-protein (aby)
  • -B12 deficiency
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6
Q

80% of MM patients have abnormal x-rays d/t?

A

osteopenia (reduced bone mass

osteoporosis (brittle/fragile)

focal lytic bone

pathologic fx

vertebral body compression fx

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7
Q

Why might MM pts have renal failure?

A

d/t:

  • light chain cast nephropathy (called myeloma kidney)
  • hypercalcemia
  • concurrent light chain amyloidosis
  • drug induced renal damage

Amyloidosis= disorder marked by deposition of amyloid (abnormal protein in the bone marrow that gets deposited in an organ or soft tissue)

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8
Q

What are some rare signs and symptoms of MM?

A
  • parasthesias/neurologic disease
  • hepatomegaly
  • splenomegaly
  • lymphadenopathy
  • infections
  • CNS involvement
  • pleural effusion and pulmonary involvement
  • extramedullary plasmacytomas (build up of monoclonal plasma cells outside of the bone marrow)
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9
Q

Complete hx and physical exam, what are some specific complaints to pay attention to?

A
  • bone pain
  • constitutional sx (I dont feel good)
  • neurological sx
  • recurrent nagging infections
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10
Q

MM labs?

A
  • CBC w/ diff, peripheral blood smear (wil lsee rouleaux, thrombocytopenia, leukopenia)
  • CMP (kidney function and Ca)
  • LDH (released during tissue damage)
  • beta-2 microglobulin
  • c-reactive protein
  • serum protein electrophoresis + IF (immuno fixation)
  • Urinalysis and 24hr urine collection for electrophoresis + IF
  • serum free monoclonal light chain
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11
Q

WHat would you expect to see in SPEP in an MM pt?

A

Two spikes, one at albumin and another at gamma (Y)

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12
Q

What tests would be ordered if suspected MM?

A
  • serum viscosity if M protein conc is High
  • bone marrow aspiration/biopsy w/ immunophenotyping
  • conventional cytogenetics
  • fluorescence in situ hybridization (FISH)
  • Metastatic bone survey–plain radiographs of humeri and femoral bones
  • MRI, CT, or PET/CT
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13
Q

What percentage of Bone marrow biopsy would contain clonal plasma cells?

A

10%

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14
Q

When would you order a pet scan?

A
  • bone pain w/o abnormality along with a normal looking radiograph
  • sudden onset of neurologic sx (this is an emergency, compression fx may be pushin on spinal cord in the instance of sudden numb leg)
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15
Q

What three criteria must be met to dx MM?

A
  • presence of serum or urinary monoclonal protein
  • presence of clonal plasma cells in bone marrow or a plasmacytoma
  • presence of end organ damage felt related to plasma dyscrasia (malfunction, abnormal) Such as:
  • increased Ca conc.
  • lytic bone lesions
  • anemia
  • renal failure
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16
Q

What does M-CRAB stand for?

A

Monoclonal protein

Calcium

Renal failure

Anemia

Bone marrow + bone pain lytic lesions

17
Q

Monoclonal Gammopathy of Undetermined Significance (MGUS), what three criteria must be met for this dx?

A

-serum monoclonal protein

18
Q

MGUS sx and tx

A

asymptomatic, dont require tx.

“sit on your hands”

19
Q

Smoldering Multiple Myeloma (SMM), what criteria must be met for dx?

A
  • serum monoclonal protein are >/= 3g/dL and/or bone marrow plasma cells >/= 10%
  • no end organ damage related to plasma cell dyscrasia
  • this is one step further thant MGUS in the progression to MM.
20
Q

Indications for tx in MM

A
  • anemia
  • hypercalcemia
  • renal insuficiency
  • lytic bone lesions/severe osteopenia
  • extramedullary plasmacytoma
21
Q

MM Tx:

A

chemotherapy: survival =3yrs
transplantation: survival 4-5years

Novel agents targeting stromal interactions and associated signaling pathways: survival beyond 5 years
Novel agents: immunomodulating drugs and proteasome inhibitors

22
Q

What is Waldenstrom macroglobulinemia?

A

-lymphoplasmacytic lymphoma in the bone marrow with an IgM monoclonal gammopathy in the blood.

cancer affecting B cells, aby IgM, indolent, uncontrolled increase in B cells prolif of B ell interfere with RBC production resulting in anemia.

unique characteristic: B cells produce excess amounts of IgM thickening the blood

23
Q

Presentation of WM?

A

most common: weakness, fatigue, weight loss, chronic oozing of blood from nose or gums.

  • asymptomatic 25%
  • consititutional sx
  • bleeding
  • neurologic sx
  • hyperviscosity (HA, vertigo, diplopia)
  • lymphadenopathy
  • hepatomegaly
  • splenomegaly
  • fundoscopic abnormalities
24
Q

Symptoms of WM?

A

Cause: tumor infiltration
Sx: anemia, fever, night sweats, weight loss, lymphadenopathy, organomegaly

Causee: Monoclonal IgM
Sx: hyperviscosity, cryoglobulinemia, cold agglutinin, neuropathy, amyloidosis

25
Q

WM evaluation, what tests will you order?

A
  • CBC
  • Platelet function and blood coag.
  • Bone marrow examination
  • serum protein electrophoresis
  • serum viscosity
  • serum free light chain
  • assay

-CT of abd and pelvis to detect organomegaly

26
Q

WM Diagnostic Criteria

A
  • IgM monoclonal gammopathy in serum

- 10% or > of bone marrow biopsy demonstrates infiltration by small lymphocytes that exhibit plasma cell differentiation

27
Q

Tx of WM

A
  • asymptomatic: observe, no Tx
  • Symptomatic: rituximab
  • High tumor burden (must also be a candidate): hematopoietic cell transplantation
  • Hyperviscosity: therapeutic plasmapheresis