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Hematology/Immunology Part 2 > MM and Plasma Cell Disorders > Flashcards

MM and Plasma Cell Disorders Flashcards

1
Q

What are the etiologies and risk factors of Multiple Myeloma?

A

Age, African American race, first-degree relative, exposure to radiation, pesticides, cleaners, military, autoimmune or inflammatory disorders, and possibly infections.

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2
Q

What is the underlying pathology of Multiple Myeloma?

A

Malignant proliferation of a single clone of plasma cells producing an abnormal amount of M Protein.

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3
Q

What are the presenting signs and symptoms of Multiple Myeloma?

A

Bone pain, vertebral compression fractures, weakness, fatigue, recurrent or serious infections.

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4
Q

What diagnostic studies are appropriate for Multiple Myeloma?

A

CBC with differential, peripheral smear, CMP, LDH, β2 microglobulin, serum protein electrophoresis, serum immunofixation electrophoresis, quantitative immunoglobulins, 24-hour urine for electrophoresis and immunofixation, serum free light chains.

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5
Q

What are the major criteria for diagnosing POEMS syndrome?

A

Polyneuropathy, Monoclonal Plasma cell disorder, Sclerotic bone lesions, Castleman disease, VEGF elevation

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6
Q

What is the purpose of serum PEP and IEP tests?

A

To detect the presence of an M-spike in the gamma region.

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7
Q

How is Multiple Myeloma staged and risk stratified?

A

Based on laboratory findings, imaging studies, and clinical presentation.

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8
Q

How many major criteria are required to diagnose POEMS syndrome?

A

Three

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9
Q

What are the treatment modalities for Multiple Myeloma?

A

Chemotherapy, stem cell transplant, radiation therapy, targeted therapy, immunotherapy.

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10
Q

What does IEP stand for?

A

Immunofixation electrophoresis.

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11
Q

What is a common feature of Castleman disease in POEMS syndrome?

A

Lymph node hyperplasia

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12
Q

What are the adverse effects of Multiple Myeloma treatments?

A

Nausea, fatigue, infections, bone marrow suppression, neuropathy.

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13
Q

Why is a 24-hour urine collection required for PEP and IEP?

A

To detect Bence-Jones Protein, an Ig or light chain found in the urine.

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14
Q

What does VEGF stand for in the context of POEMS syndrome?

A

Vascular endothelial growth factor

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15
Q

What is a higher rate of neutropenia associated with?

A

Lenalidomide and Dexamethasone (RD)

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16
Q

What are plasma cells?

A

B lymphocytes that mature and produce antibodies for humoral immunity.

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17
Q

What requires ASA prophylaxis?

A

Lenalidomide and Dexamethasone (RD)

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18
Q

Why are UPEP and UIEP tests done along with serum tests?

A

20% of patients only produce free light chains, which may not be detectable in serum.

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19
Q

What is one of the minor criteria for diagnosing POEMS syndrome?

A

Organomegaly, ECF volume overload, Endocrine disorder, skin changes, papilledema, thrombocytosis/polycythemia

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20
Q

What increases the sensitivity of detecting free light chains?

A

Serum free light chains.

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21
Q

What are the side effects of Bortezomib and Dexamethasone (VD)?

A

Peripheral neuropathy, HSV reactivation

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22
Q

What are immunoglobulins?

A

Antibodies produced by plasma cells, also known as gammaglobulins.

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23
Q

What are the heavy chain isotypes of immunoglobulins?

A

G, M, A, D, E.

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24
Q

What imaging study is used to evaluate for lytic lesions?

A

Skeletal survey with plain films.

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25
Q

What is the clinical course of POEMS syndrome often defined by?

A

Progressive polyneuropathy

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26
Q

What prophylaxis is required for Bortezomib and Dexamethasone (VD)?

A

HSV prophylaxis

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27
Q

Why is a bone scan not used for lytic lesions?

A

Radioisotope is not taken up by lytic lesions.

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28
Q

Why is the diagnosis of POEMS syndrome often delayed?

A

It is often confused with other neurologic diseases.

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29
Q

What are the light chain isotypes of immunoglobulins?

A

Kappa (κ) and lambda (λ).

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30
Q

What are the components of the CyBorD regimen?

A

Cyclophosphamide, Bortezomib, and Dexamethasone

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31
Q

What can MRI detect in multiple myeloma?

A

More lesions and assess for spinal cord compression.

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32
Q

What is hypogammaglobulinemia?

A

Low levels of antibodies.

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33
Q

What are the components of the VRD regimen?

A

Bortezomib, Lenalidomide, and Dexamethasone

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34
Q

What is monoclonal gammopathy?

A

Disease involving a single antibody clone.

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35
Q

What is used for pain control in plasmacytomas or lytic lesions?

A

Radiation

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36
Q

What are plasma cell disorders?

A

Monoclonal neoplasms arising from uncontrolled maturation and proliferation of antibody-secreting B-lymphocytes.

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37
Q

Why is PET often preferred in combination with a skeletal survey?

A

Increased uptake with lytic lesions.

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38
Q

What can neuropathy in POEMS syndrome progress to?

A

Respiratory compromise

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39
Q

What is the treatment for POEMS syndrome aimed at?

A

Underlying plasma cell disorder

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40
Q

What treatment has shown significant or complete resolution of symptoms in POEMS syndrome?

A

Autologous SCT

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41
Q

What is used to treat hypercalcemia?

A

Bisphosphonates

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42
Q

What is the role of CT in multiple myeloma?

A

Characterization of soft tissue masses/plasmacytomas.

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43
Q

What resource is known as a comprehensive guide for internal medicine and is useful for POEMS syndrome?

A

Harrison’s Principles of Internal Medicine

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44
Q

What is required for the diagnosis of lytic lesions?

A

Bone marrow biopsy.

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45
Q

What are some examples of plasma cell disorders?

A

Multiple Myeloma, MGUS, Waldenstrom’s macroglobulinemia, primary amyloidosis.

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46
Q

What was the 5-year survival rate for all ages and sexes in 1975 according to SEER data?

A

26.6%

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47
Q

What does a bone marrow biopsy quantify and characterize?

A

Plasma cells.

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48
Q

What is Multiple Myeloma?

A

A malignant proliferation of a single clone of plasma cells producing an abnormal amount of M Protein.

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49
Q

What is the purpose of flow cytometry in multiple myeloma?

A

Immunophenotyping to differentiate normal from malignant plasma cells.

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50
Q

Which resource provides current medical diagnosis and treatment information relevant to POEMS syndrome?

A

Current Medical Diagnosis & Treatment

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51
Q

What online resource is mentioned for up-to-date information on POEMS syndrome?

A

Up to Date

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52
Q

What do cytogenetics and karyotyping help determine in multiple myeloma?

A

Risk and prognosis.

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53
Q

What was the 5-year survival rate for all ages and sexes from 2006-2012 according to SEER data?

A

48.1%

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54
Q

What is M protein in Multiple Myeloma?

A

Monoclonal protein that may be intact immunoglobulin or fragments of heavy or light chains.

55
Q

What was the 5-year survival rate for all ages and sexes from 2012-2018 according to SEER data?

A

57%

56
Q

What are the three criteria required for diagnosing symptomatic multiple myeloma?

A

Any level of M-protein in serum/urine, bone marrow biopsy with >10% plasma cells or biopsy-proven plasmacytoma, and evidence of organ/tissue involvement (CRAB criteria).

57
Q

What does SEER stand for?

A

Surveillance, Epidemiology, and End Results Program

58
Q

What does CRAB stand for in the context of multiple myeloma?

A

Hypercalcemia, renal disease, anemia, bone disease.

59
Q

What does ‘multiple’ in Multiple Myeloma refer to?

A

Bony lesions in multiple sites at the time of diagnosis.

60
Q

What is a plasmacytoma?

A

Proliferation of plasma cells outside the bone marrow.

61
Q

What happens in Plasma Cell Leukemia?

A

Plasma cells exit BM and enter peripheral bloodstream

62
Q

What is the International Staging System (ISS) used for?

A

Staging multiple myeloma based on albumin and B2-macroglobulin levels.

63
Q

What percentage of patients are affected by Plasma Cell Leukemia?

A

~1%

64
Q

What are the most common subtypes of Multiple Myeloma?

A

IgG (60%) and IgA (20%).

65
Q

What are the albumin and B2-macroglobulin levels for Stage I multiple myeloma?

A

B2M ≤ 3.5 mg/L, albumin ≥ 3.5 g/dL.

66
Q

What is the median survival for Stage I multiple myeloma?

A

62 months.

67
Q

How is Plasma Cell Leukemia reported?

A

On CBC with Differential as % Plasma cells

68
Q

What defines Stage II multiple myeloma?

A

Neither Stage I nor III criteria are met.

69
Q

What is the epidemiology of Multiple Myeloma?

A

More common in males, median age 69, African American > Caucasian 2:1.

70
Q

What does the presence of plasma cells on a peripheral smear indicate?

A

Poorer prognosis

71
Q

What is the median survival for Stage II multiple myeloma?

A

44 months.

72
Q

What are the risk factors for Multiple Myeloma?

A

Age, African American race, first-degree relative, exposure to radiation, pesticides, cleaners, military, autoimmune or inflammatory disorders, possibly infections.

73
Q

What are the B2-macroglobulin levels for Stage III multiple myeloma?

A

B2M ≥ 5.5 mg/L.

74
Q

What are other plasma cell disorders mentioned?

A

Waldenstrom Macroglobulinemia, Amyloidosis, POEMS syndrome

75
Q

What is the median survival for Stage III multiple myeloma?

A

29 months.

76
Q

What are common symptoms of Multiple Myeloma?

A

Bone pain, vertebral compression fractures, weakness, fatigue, recurrent or serious infections.

77
Q

What is another name for Waldenstrom Macroglobulinemia?

A

Lymphoplasmacytic Lymphoma

78
Q

What type of cells are involved in Waldenstrom Macroglobulinemia?

A

Mature B lymphocytes undergoing plasmacytic differentiation

79
Q

What is smoldering multiple myeloma?

A

Asymptomatic multiple myeloma with M protein ≥ 3g/dL or BM plasma cells ≥ 10%, no CRAB criteria.

80
Q

What are common physical exam findings in Multiple Myeloma?

A

Often normal, anemia (pallor), extramedullary plasmacytomas, palpable masses, point tenderness at fracture sites.

81
Q

What is the expected progression time for smoldering multiple myeloma to symptomatic multiple myeloma?

A

4-5 years.

82
Q

What do the cells in Waldenstrom Macroglobulinemia produce?

A

Monoclonal IgM

83
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance, a pre-malignant condition.

84
Q

What are the criteria for MGUS?

A

M protein < 3g/dL, bone marrow plasma cells <10%, no CRAB criteria.

85
Q

Is treatment indicated for smoldering multiple myeloma or MGUS?

A

No.

86
Q

What laboratory evaluations are used for Multiple Myeloma?

A

CBC with differential, peripheral smear, CMP, LDH, β2 microglobulin, serum protein electrophoresis, serum immunofixation electrophoresis, quantitative immunoglobulins, 24-hour urine for electrophoresis and immunofixation, serum free light chains.

87
Q

What are the common presentations of Waldenstrom Macroglobulinemia?

A

Anemia, Lymphadenopathy, splenomegaly, hyperviscosity

88
Q

What is the expected survival without treatment for symptomatic multiple myeloma?

A

~6 months.

89
Q

What is usually absent in Waldenstrom Macroglobulinemia?

A

Lytic lesions

90
Q

How much can chemotherapy or transplant increase survival in symptomatic multiple myeloma?

A

2-5+ years.

91
Q

What are common laboratory findings in Multiple Myeloma?

A

Anemia, renal dysfunction, hypercalcemia, hyperproteinemia, hypogammaglobulinemia, Rouleaux formation on peripheral smear.

92
Q

What are the symptoms of hyperviscosity in Waldenstrom Macroglobulinemia?

A

Vision changes, HA, dizziness, cardiopulmonary symptoms, bleeding

93
Q

How is the response to treatment monitored in multiple myeloma?

A

By reduction of M protein in serum/urine and size of plasmacytomas.

94
Q

What is Rouleaux formation?

A

Stacking of RBCs related to increased serum protein levels.

95
Q

Is there a cure for multiple myeloma?

A

No, treatment is for disease control, palliation, and prolonging survival.

96
Q

How is hyperviscosity treated in Waldenstrom Macroglobulinemia?

A

Plasmapheresis

97
Q

What is the work-up for Waldenstrom Macroglobulinemia similar to?

A

Multiple Myeloma

98
Q

What is the frontline treatment for multiple myeloma?

A

Autologous stem cell transplantation with high-dose melphalan chemotherapy.

99
Q

What is the significance of serum protein electrophoresis (SPEP) in Multiple Myeloma?

A

Used to confirm the type of hyperproteinemia and detect M-spike in the gamma region.

100
Q

What is the treatment approach for asymptomatic Waldenstrom Macroglobulinemia?

A

Observation

101
Q

What is immunofixation electrophoresis (IEP)?

A

A technique to identify specific types of immunoglobulins in the serum.

102
Q

What is the benefit of autologous stem cell transplantation over chemotherapy alone?

A

Significant survival benefit.

103
Q

What precedes autologous stem cell transplantation?

A

2-4 cycles of chemotherapy to reduce disease burden.

104
Q

What are the treatment indications for Waldenstrom Macroglobulinemia?

A

Hyperviscosity, Hgb <10 g/dL, Platelet <100K, Bulky adenopathy or symptomatic organomegaly, Neuropathy

105
Q

What factors determine eligibility for autologous stem cell transplantation?

A

Co-morbidities and age (Performance Status).

106
Q

What is the treatment for Waldenstrom Macroglobulinemia?

A

Rituximab +/- combination chemotherapy, Autologous SCT after disease control if eligible

107
Q

What is tandem transplantation?

A

Two transplants 3-6 months apart.

108
Q

When might an allogeneic transplant be required?

A

Depending on risk and response to autologous transplant.

109
Q

What is another name for Amyloidosis?

A

Systemic Light-Chain Amyloidosis

110
Q

What are the components of the RD treatment regimen?

A

Lenalidomide and dexamethasone.

111
Q

What are the side effects of the RD regimen?

A

Higher rate of neutropenia and VTE, requires ASA prophylaxis.

112
Q

What is deposited in organs in Amyloidosis?

A

Residues of light-chains (amyloid fibrils)

113
Q

What are the components of the VD treatment regimen?

A

Bortezomib and dexamethasone.

114
Q

What are the side effects of the VD regimen?

A

Peripheral neuropathy and HSV reactivation, requires HSV prophylaxis.

115
Q

What are the components of the CyBorD treatment regimen?

A

Cyclophosphamide, bortezomib, and dexamethasone.

116
Q

What are the components of the VRD treatment regimen?

A

Bortezomib, lenalidomide, and dexamethasone.

117
Q

What percentage of Amyloidosis cases involve lambda light chains?

A

75%

118
Q

What percentage of patients with Multiple Myeloma have Amyloidosis?

A

10-15%

119
Q

What does the presentation of Amyloidosis depend on?

A

Organ involvement

120
Q

What are the renal symptoms of Amyloidosis?

A

Renal insufficiency, nephrotic syndrome

121
Q

What are the cardiac symptoms of Amyloidosis?

A

Cardiomyopathy, arrhythmia

122
Q

What are the nervous system symptoms of Amyloidosis?

A

Peripheral neuropathy, weakness, orthostatic hypotension, ED, anhidrosis

123
Q

What are the gastrointestinal symptoms of Amyloidosis?

A

Hepatomegaly, GI bleeding, diarrhea, malabsorption

124
Q

How is Amyloidosis diagnosed?

A

Biopsy of abdominal fat pad or organ involved, Congo Red stain, mass spectrometry analysis

125
Q

What does Congo Red stain show in Amyloidosis?

A

Positive apple-green birefringence on polarized light exam

126
Q

What is the treatment goal for Amyloidosis?

A

Preventing further deposition

127
Q

What is the treatment for Amyloidosis?

A

High-dose chemotherapy + Autologous Stem Cell Transplant

128
Q

What is the prognosis for Amyloidosis with organ involvement?

A

Poor, particularly with cardiac involvement

129
Q

What is POEMS syndrome associated with?

A

Plasma cell disorder (typically Myeloma)

130
Q

What does POEMS stand for?

A

Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, Skin Changes

131
Q

How many major criteria are required for POEMS syndrome diagnosis?

A

3

132
Q

What are the major criteria for POEMS syndrome?

A

Polyneuropathy, Monoclonal Plasma cell disorder, Sclerotic bone lesions, Castleman disease, VEGF elevation

133
Q

How many minor criteria are required for POEMS syndrome diagnosis?

A

One

134
Q

What are the minor criteria for POEMS syndrome?

A

Organomegaly, ECF volume overload, Endocrine disorder, skin changes, papilledema, thrombocytosis/polycythemia

Hematology/Immunology Part 2 (9 decks)

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