Plasma Cell Neoplasm Pathology Flashcards

1
Q

Multiple Myeloma is a clonal neoplastic proliferation of ______.

A

mature plasma cells

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2
Q
A
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3
Q

Each clone of MM produces a single Ig, which are typically

A
  1. IgG
  2. IgA
  3. Bence-Jones proteins (free light chains)
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4
Q

_____ (cytokines) → increases plasma cell proliferation

A

IL-6

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5
Q

How does renal insufficiency develop in MM?

A

Bence-jones proteins are renally excreted → proteinuria/light chain toxicity

(some light chains also predispose to amyloidosis)

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6
Q

Russell bodies (cytoplasmic) or Dutcher bodies (nuclear)

(PAS+)

… are found in which 2 neoplasms?

A
  1. MM
  2. Waldenstrom
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7
Q

Mott cell

A

cell w/many Russel Bodies

(found in MM; note clock-faced nucleus)

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8
Q

Flame cells are due to _____ at the periphery

A

Ig

(found in MM)

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9
Q

MM flow cytometry markers (3)

A

CD38+, CD138+, CD19+

(contrast w/B cell CD20+, CD19+)

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10
Q

MM is due to a _____ (2) deletion or ____ duplication or _____ translocation.

A
  • 13q or 17p → LOF p53
  • 1q
  • t(4;14)(p16;q32)
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11
Q

Genetic pathway affected by MM

A

MAPK → RAS → c-myc activation (the event that transforms it into MM)

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12
Q

MM clinical presentation (6)

A
  1. anemia (normocytic, normochromic)
  2. bone pain + fatigue
  3. Elevated Creatinine
  4. hematologic malignancy sx: fever, wt. loss, fatigue
  5. peripheral neuropathy
  6. renal insufficiency
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13
Q

_____ (lab finding) that confirms MM.

A

M-spike: dense band on electrophoresis → shine a light through it and it gives you this graph

(albumin is the band to the left; Igs are in the gamma band)

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14
Q

Once you find the M-spike, what is the next step?

A

immunofixation to detect the isotype

(IgG & lambda present in the example)

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15
Q

Diagnostic criteria for MM includes > 10% clonal plasma, CRAB criteria. What is the CRAB mn?

(the opposite of MGUS which has <10% & NO CRAB sx)

A

C: increased calcium

R: renal insufficiency

A: Anemia

B: bone pain (osteoporotic)

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16
Q

Most sensitive imaging for “punched out lesions” of MM

A

MRI

17
Q

Staging of MM is based on _____(4).

A
  1. kidney function
  2. beta-2 microglobulin
  3. LDH
  4. cytogenetics
18
Q

MC cause of death in MM

A
  1. infection
  2. kidney failure
19
Q

prognosis of MM

A

50% at 5 years

20
Q

Clinical course of MM w/non-BMT tx

A
21
Q

Monoclonal gammopathy of Undetermined Significance (MGUS) is a production of low-levels of _____.

A

Igs

(clonal plasma cells in BM; plasma cell dyscrasia)

22
Q

MGUS affects 1 in _____ people of 50 years old

A

100! (mostly asymptomatic)

(african americans and white males)

23
Q
A

Right: could be due to inflammation

24
Q

________ has >10% BM cells and NO light chain restriction.

A

Reactive Plasmacytosis (aka polyclonal plasmacytosis)

25
Q

25% of ______ transforms into MM

A

Reactive Plasmacytosis (aka polyclonal plasmacytosis)

(tx: observation only)

26
Q

Smoldering myeloma is the middle ground between ______ (2 diseases)

A

MGUS & MM

(no CRAB manifestations)

27
Q

elevated plasma cells, elevated IgM, and an asymptomatic presentation is likely ______

A

smoldering myeloma

(tx: observation only)

28
Q

Monoclonal IgM (M-protein), malignant lymphoplasmacytic cell infiltration of BM, hepatosplenomegaly and lymphadenopathy, hyperviscosity indicate ________ (dx)

A

Waldenstrom Macroglobulinemia (aka lymphoplasmacytic lymphoma)

29
Q

Waldenstrom Macroglobulinemia (aka Lymphoplasmacytic Lymphoma) is a _____ - grade lymphoma.

A

low

30
Q

Waldenstrom Macroglobulinemia (aka lymphoplasmacytic lymphoma) risk factors (3)

A
  1. Hep C (viral)
  2. Autoimmune (i.e. Sjogren’s)
  3. White Elderly Caucasian
31
Q

MD88 activation on gene at 3p22.2 loci, CD20+, CD38+, CD138(-) → ______

A

Waldenstrom Macroglobulinemia (aka lymphoplasmacytic lymphoma)

(translocations rare)

32
Q

______ is a distinguishing laboratory finding of Waldenstrom and likely the reason it remains asymptomatic

A

elevated serum Igs

(hypergammaglobulinemia)

33
Q

Sequelae of Waldenstrom Macroglobulinemia (4)

A
  1. DLBCL (Richter)
  2. amyloidosis
  3. stroke
  4. cold agglutinin diz
  5. CHF
34
Q

Tx for waldenstrome

A
  1. observe
  2. chemo
  3. BMT
35
Q

Amyloidosis

A

accumulation of EC tissue deposition of fibrils (low molecular weight proteins (Ig light chains) in tissue

36
Q

Amyloidosis may lead to ______ (4).

A
  1. nephrotic syndrome
  2. heart failure
  3. hepatomegaly
  4. neuropathy

(<10% of MM is a/w amyloidosis)