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Internal Medicine > JH IM Board Review - Plasma Cell Dyscrasias > Flashcards

JH IM Board Review - Plasma Cell Dyscrasias Flashcards

1
Q

7 categories of plasma cell dyscrasias:

A
  1. MGUS.
  2. MM.
  3. Plasmacytoma.
  4. POEMS.
  5. AL amyloidosis.
  6. Waldenstrom.
  7. Cryoglobulinemia.
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2
Q

What else can lead to the production of monoclonal Igs, but is NOT malignant and is distinct from plasma cell dyscrasias?

A

Autoimmune disorders and chronic inflammatory conditions:

  1. CTDs.
  2. TB, endocarditis, hepatitis, HIV.
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3
Q

Monoclonal Igs are seen in approx. …% of adults.

A

1%.

==> Prevalence increases with age.

==> IgG > IgM > IgA.

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4
Q

MM - Poor prognostic factors:

A
  1. Renal insufficiency.
  2. Elevated beta-2 microglobulin.
  3. Genetic abnormalities including:

==> 1q amplification, t[4;14], t[14,16], t[14;20], p53 microdeletions, 13q del, complex karyotype.

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5
Q

What is requires to diagnose MM?

A

Presence of one or more of the CRAB criteria (hypercalcemia, renal insufficiency, anemia, bone disease).

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6
Q

Diagnostic criteria for MM - Smoldering myeloma:

A
  1. Clonal bone marrow plasma cells 10% of higher OR

2. Presence of serum and/or urinary monoclonal protein 3g/dL or higher.

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7
Q

Diagnostic criteria for MM - Active:

A

Above criteria with addition of end-organ damage attributable to the underlying plasma cell proliferative disorder, specifically the CRAB criteria.

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8
Q

CRAB criteria:

A
  1. Calcium >11.5 g/dL.
  2. Renal insufficiency (creatinine greater than 2mg/dL).
  3. Anemia (Hb <10).
  4. Bone disease (osteopenia or lytic lesions).
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9
Q

IV contrast in patients with known or suspected MM?

A

AVOID, UNLESS ABSOLUTELY NECESSARY.

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10
Q

Bone scan in patients with myeloma?

A

Typically normal ==> Shows only blastic lesions.

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11
Q

Evaluation of a monoclonal immunoglobulin - 4 steps - Step 1:

A

Define and quantitate abnormal immunoglobulin production.

  1. SPE + immunofixation.
  2. UPE + immunofixation.

==> If BOTH negative and high index of suspicion, check serum light chain assay.

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12
Q

Evaluation of a monoclonal immunoglobulin - 4 steps - Step 2:

A

Define and quantitate organ system dysfunction.

  1. CBC and differential ==> r/o anemia, pancytopenia.

2. r/o renal dysfunction with BUN/Cr; obtain serum calcium.

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13
Q

Evaluation of a monoclonal immunoglobulin - 4 steps - Step 3:

A

r/o MM ==>

  1. Bone marrow aspirate and bx.
  2. Skeletal x-ray series to r/o lytic or sclerotic bone lesions.
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14
Q

Evaluation of a monoclonal immunoglobulin - 4 steps - Step 4:

A

Define other characteristics of disorder:

  1. Serum viscosity if IgM gammopathy, or suggestive symptoms.
  2. r/o CTD or chronic inflammatory disorder if no plasma cell dyscrasias found.
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15
Q

Tx of smoldering myeloma:

A

Investigational.

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16
Q

MM Tx:

A

Chemo ==> not curative.

Should be instituted when pts are symptomatic or demonstrate end-organ dysfunction.

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17
Q

MM Tx - Initial Tx decision based on eligibility for stem cell transplantation ==> For TRANSPLANT INELIGIBLE:

A
  1. Melphalan, prednisone, and thalidomide.
  2. Melphalan, prednisone, and bortezomib.
  3. Lenaldiomide + dexamethasone.
  4. Bortezomib + dexa.
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18
Q

MM Tx - Initial Tx decision based on eligibility for stem cell transplantation ==> For TRANSPLANT ELIGIBLE:

A
  1. Lenaldiomide + dexa.
  2. Bortezomib + dexa.
  3. Lenaldiomide + borte + dexa.
  4. Borte + dexa + Cyclophosphamide.
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19
Q

MM Tx - BM transplantation:

A
  1. Autologous ==> incr. survival by 12-18 months. Eventually, relapse.
  2. Allogenic ==> potentially curative (25-30%) but has greatest tox.
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20
Q

MM - VZV prophylaxis if on …?

A

BORTEZOMIB.

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21
Q

Plasmacytomas:

A

Proliferations of monoclonal plasma cells forming a mass at extramedullary or osseous sites.

==> No evidence of multiple myeloma or end-organ dysfunction.

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22
Q

50% of osseous plasmacytomas and 15% of extraosseous …?

A

DEVELOP INTO OVERT MYELOMA.

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23
Q

Solitary plasmacytoma:

A

Single tumor of plasma cells resulting in localized complaints (bone pain, pathologic fracture).

==> No evidence of diffuse plasma cell overgrowth throughout remainder of bone marrow.

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24
Q

Extramedullary plasmacytoma:

A

Plasma cell tumor arising outside of the bone marrow.

==> most commonly found in URT (nasal passages, sinuses, nasopharynx, larynx).

==> Occasionally described in the lower extremity, GI, lung, lymph nodes.

==> Can be associated with HIV infection.

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25
Q

Plasmacytomas - Tx:

A
  1. Radiation therapy to the involved area.

2. 15-25% are disease-free at 10yrs.

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26
Q

Clinical manifestations of MM - Hyperviscosity:

A
  1. Retinal bleeds.
  2. Bruising.
  3. HF.
  4. Cerebral ischemia.
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27
Q

Clinical manifestations of MM - Amyloid:

A
  1. Panda eyes.
  2. Nephrotic syndrome.
  3. Carpal tunnel syndrome.
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28
Q

Clinical manifestations of MM - Bone:

A

Pain + Fx + lytic lesions.

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29
Q

Clinical manifestations of MM - Spinal cord compression:

A
  1. Bony collapse.

2. Extradural mass.

30
Q

Clinical manifestations of MM - Blood:

A
  1. Normochromic normo-/macro-cytic.
  2. Pancytopenia.
  3. Raised ESR.
31
Q

Clinical manifestations of MM - Renal failure:

A
  1. Paraprotein deposition.
  2. Hypercalcemia.
  3. Infection.
  4. NSAIDs.
  5. Amyloid.
  6. Dehydration.
32
Q

Hyperviscosity syndrome - Symptoms:

A
  1. Neurologic ==> Headache, dizziness, ataxia, paresthesias, somnolence, coma.
  2. Visual ==> Diplopia, blurred vision, loss of vision.
  3. Cardiac ==> CHF, myocardial ischemia.
  4. Hematologic ==> Spontaneous bleeding, particularly from nose and gums/ GI bleeding described.
33
Q

Hyperviscosity syndrome - Physical findings:

A
  1. Flame-shaped retinal hemorrhages.
  2. Papilledema.
  3. Boxcar formation of RBCs in retinal vessels.
34
Q

Hyperviscosity - Tx:

A
  1. Plasmapheresis until symptoms resolve (may be needed daily if symptoms persists).
  2. Therapy directed at underlying cause.
  3. Caution if RBC transfusion needed, because this will expand volume and worsen symptoms.
35
Q

MGUS is diagnosed entirely on what criteria?

A

Laboratory criteria.

36
Q

MGUS evaluation - When to do BM aspiration?

A

Recommended for all patients with:

  1. M protein greater than 1g/dL.
  2. Non-IgG paraprotein, regardless of quantification of M protein.
37
Q

MGUS diagnostic criteria:

A
  1. M protein less than 3 (SPEP).
  2. Less than 10% plasma cells in bone marrow.
  3. Normal Hb, creatinine, calcium.
  4. Absence of lytic bone disease.
  5. Asymptomatic.
38
Q

MGUS - Increased risk of progression to MM:

A
  1. High monoclonal protein.
  2. Non-IgG paraprotein.
  3. Abnormal serum κ/λ ratio.
39
Q

POEMS syndrome is also called?

A

Osteosclerotic myeloma.

40
Q

Waldenstrom is also known as …?

A

Lymphoplasmacytic lymphoma.

41
Q

Waldenstrom:

A

Bone marrow infiltration of clonal B cells with production of M protein (IgM).

42
Q

What percentage of pts with IgM monoclonal gammopathy have Waldenstrom?

A

Only 25%. Others have:

  1. MGUS.
  2. MM.
  3. CLL.
  4. NHL.
  5. Cold agglutinin disease.
43
Q

Waldenstrom diagnosis?

A

Based on the appearance of IgM + Evidence of B-cell lymphoma by pathology.

44
Q

Cryoglobulinemia:

A

Group of disorders (3 subtypes) characterized by the production of soluble proteins that precipitate on cooling, typically associated with symptoms.

==> Often evidence of an underlying clonal disorder.

45
Q

Cryoglobulinemia type I - Ig?

A

Presence of isolated monoclonal Ig (usually IgG or IgM, less commonly IgA or free light chains).

46
Q

Cryoglobulinemia type I - Disease association:

A
  1. Waldenstrom.

2. MM.

47
Q

Cryoglobulinemia type I - Features:

A

Many are ASYMPTOMATIC.

Classic symptoms related to hyperviscosity.

==> Digital ischemia, Raynaud, purpura, livedo reticularis.

48
Q

Cryoglobulinemia type II - Ig:

A

“Essentially mixed CG” ==> A mixture of polyclonal Ig with a monoclonal Ig (usually IgM or IgA).

==> Can also have RF activity.

49
Q

Cryoglobulinemia type II - Disease association:

A

Persistent viral infections.

==> Most often HCV/HIV.

50
Q

Cryoglobulinemia type II - Features:

A
  1. MPGN.
  2. Mononeuritis multiplex.
  3. Vasculitis.
  4. Classic MELTZER TRIAD ==> Purpura + Arthralgias + Weakness.
51
Q

Cryoglobulinemia type III - Ig:

A

Polyclonal Ig.

52
Q

Cryoglobulinemia type III - Disease association:

A

Most often secondary to CTDs or chronic inflammatory conditions.

53
Q

Cryoglobulinemia type III - Features:

A

Tx directed at primary disorder.

54
Q

Amyloidosis - Types:

A
  1. AL.
  2. AA.
  3. Familial (transthyretin).
55
Q

AL amyloidosis - Dominant protein:

A

Immunoglobulin light chains (λ>κ).

56
Q

AL amyloidosis - Organ deposits:

A
  1. Heart.
  2. Kidney.
  3. Tongue.
  4. GI tract.
  5. CNS.
57
Q

AL amyloidosis - Symptoms:

A
  1. Low-voltage ECG.
  2. RHF with volume-overload.
  3. Echocardiogram shows classic myocardium speckled pattern.
  4. Orthostatic hypotension.
  5. Nephrotic-range proteinuria.
  6. Macroglossia.
  7. Infiltration of gut with malabsorptive process.
  8. Peripheral neuropathy, carpal tunnel syndrome.
58
Q

AA amyloidosis - Dominant protein:

A

Serum amyloid protein A.

59
Q

AA amyloidosis - Organ deposits:

A
  1. Kidney.
  2. Liver.
  3. Spleen.
60
Q

AA amyloidosis - Symptoms:

A

MC involved with nephrotic-range proteinuria.

==> Organomegaly.

61
Q

Familial amyloidosis - Transthyretin - Organ deposits:

A
  1. CNS.

2. Heart.

62
Q

Familial amyloidosis - Symptoms:

A
  1. Neuropathy with sensory or autonomic insuffiency.

2. Conduction abnormalities (also symptoms of cardiac deposits of AL amyloidosis).

63
Q

AL amyloidosis is basically a …?

A

Plasma cell dyscrasia related to myeloma.

64
Q

AA amyloidosis - Serum amyloid protein A is related to which Ig?

A

TO NO KNOWN Ig.

65
Q

AA amyloidosis is associated with chronic infections and inflammatory disorders:

A
  1. TB, osteomyelitis, bronchiectasis.

2. RA, IBD, FMF.

66
Q

Familial amyloidosis:

A

AD - Mutant protein is commonly transthyretin ==> Forms amyloid fibrils.

67
Q

Systemic senile amyloidosis:

A

SPORADIC disorder characterized by wild-type transthyretin protein deposition in the myocardium leading primarily to late-onset cardiomyopathy + potentially carpal tunnel syndrome.

==> Survival is significantly longer than other causes of amyloid cardiomyopathy.

68
Q

Amyloidosis Dx - How to identify amyloid subtype?

A

Mass spectroscopic analysis.

69
Q

AL amyloidosis - Tx:

A

Chemo shown to prolong survival ==> MELPHALAN + PREDNISONE.

==> Small studies suggest autologous BM transplantation may improve survival in selected patients.

70
Q

AL amyloidosis - Prognosis:

A

Median survival is 1-2 years.

==> If cardiac involvement ==> 6 months.

==> If renal involvement ==> 21 months.

71
Q

FAMILIAL amyloidosis - Tx:

A

LIVER TRANSPLANTATION IS CURATIVE. Often does not reverse the damage.

Internal Medicine (106 decks)

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