Plasma Cell Neoplasms Flashcards

1
Q

All Plasma Cell Neoplasms are most common in what age group?

A

seen in older adults, not children

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2
Q

What is the most common plasma cell neoplasm?

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

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3
Q

What is the Serum M-protein level in a patient with MGUS?

A

<3g/dL

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4
Q

What is the clinical presentation of a patient with MGUS?

A

asymptomatic with no bony lesions or renal disease

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5
Q

What is Multiple Myeloma? What is its clinical triad?

A

malignant plasma cell neoplasm

  • Clinical Triad
    • Monoclonal protein
      • Serum M-protein >3 g/dL
      • Urine M-protein > 500mg/24 hr
    • Monoclonal plasmacytosis (in BM)
      • plasma cells = 10-60% BM cells
    • Disease-related organ damage (CRAB)
      • Hypercalcemia
      • Renal insufficiency
      • Anemia
      • Bone lesions
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6
Q

Multiple Myeloma is commonly preceded by what condition?

A

MGUS

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7
Q

What risk factors are associated with Multiple Myeloma?

A
  • Chronic antigenic stimulation (autoimmune, infectious, chronic inflammatory conditions)
  • Radiation / toxins
    • herbicides, pesticides, asbestos
    • rubber, plastic, wood, or petroleum
  • Declining immune function
  • Genetic predisposition
    • black:white = 2:1
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8
Q

What is clinical presentation of a patient with Multiple Myeloma?

A
  • middle-aged to older African American
  • asymptomatic to aggressive
    • bone pain
    • fatigue
    • weight loss
    • pallor
    • infection
    • bleeding
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9
Q

What type of end organ damage from Multiple Myeloma is shown in the provided image?

A

Kidney- myeloma cast neuropathy

  • Fragmented pieces tubular cast (open arrow)
  • Gian cell reaction (solid arrow)
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10
Q

The provided images are characteristic of what condition?

A

Multiple Myeloma

punched out lesion on skull & lytic lesions on X-Ray

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11
Q

What are the names of the cells indicated by the arrows in the provided images? They are seen in what condition?

A

Multiple Myeloma

  • Left
    • Dutcher bodies- myeloma cells with nuclear pseudoinclusions (cytoplasmic, but look like they are invaginating the nucleus)
  • Right
    • White arrow
      • Russel Bodies- cytoplasmic immunoglobulins accumulations appearing as uniform, round, colorless globules
    • Black arrow
      • Mott cells- myeloma cells with multiple Russell bodies
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12
Q

Multiple Myeloma has what type of BM involvement?

A

patchy

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13
Q

What RBC pathology is commonly seen in Multiple Myeloma?

A

Rouleaux

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14
Q

What is the usual cause of death for a patient with Multiple Myeloma?

A

bleeding or infection

the malignant plasma cells outgrow & suppress the production of normal cells, which leads to impaired immunity and blood cytopenias

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15
Q

Multiple Myeloma may develop into what complicating conditions?

A

amyloidosis of AL types

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16
Q

What is the prognosis of a patient with Multiple Myeloma?

A

varies - few months to >10 yrs

17
Q

What is Smoldering Myleloma? What percent progress to Multiple Myeloma?

A

asymptomatic myeloma with myeloma level plasmacytosis or M-protein

NO end-organ damage or lytic bone lesion (CREST)

75% progress over 15 yrs

18
Q

What is primary amyloidosis?

A

extracellular deposition of fibrillar proteins with beta-pleated sheet tertiary structure

19
Q

What is AL amyloid composed of?

A

immunoglobulin light chains

20
Q

What is the clinical picture of a patient with Primary Amyloidosis?

A
  • Older adult
  • Symptoms
    • organomegaly, purpura, bone pain, peripheral neuropathy, hemorrhage, CHF, neuropathic & malabsorption syndromes
21
Q

What condition is depicted by the provided images?

A

Primary Amyloidosis

involved tissue may have waxy gross appearance

  • Congo Red staining, & will have apple-green birefringence on polarized light microscopy (all forms amyloid)
22
Q

What is a solitary plasmacytoma?

A

Solitary plasma cell neoplasm of monotypic plasma cells

23
Q

What are the 2 forms of solitary plasmacytoma?

A

Intraosseous (intramedullary)

Extraosseous (extramedullary)

24
Q

Solitary plasmacytomas most commonly affect what age group?

How are they differentiated from other myelomas?

A

middle-aged

  • NO clinical feature of myeloma
    • polyclonal immunoglobulins are normal
    • NO anemia, hypercalcemia, or renal failure
25
Q

How is solitary plasmacytoma treated?

A

Radiotherapy

26
Q

What are the most common sites of Extraosseous Plasmacytoma?

Prognosis?

A

Head/neck region

good prognosis- may recur but infrequently progress to myeloma

27
Q

What imaging results & lab values would you expect to see in a patient with Intraosseous Plasmacytoma?

Prognosis?

A

Single site of bone involvement (pain at site/fractures) - no other bony lesions on skeletal survey

M-protein low levels (70%)

develop into myeloma or additional plasmacytomas