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GBA - Y4 > Harrison's 107 - Plasma cells Disorders > Flashcards

Harrison's 107 - Plasma cells Disorders Flashcards

1
Q

What diseases are called plasma cell disorders?

A
  1. Multiple myeloma (MM), SMM, MGUS
  2. Waldenstrom’s macroglobulinemia
  3. Heavy Chain diseases
  4. POEMS Syndrome
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2
Q

What diseases are the synonyms for Heavy Chain diseases?

A

Monoclonal Gammopathies Paraproteinemias
Plasma cell Dyscrasias
Dysproteinemias

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3
Q

What is the root common cause for plasma cell disorders?

A

B cell Transformation into adult defective plasma cell without sufficient antigenic stimulus.

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4
Q

What 3 factors determines the clinical manifestation & symptoms of a plasma cell disorder?

A

Amount of tumor cells
Type of Secretion Product
Immune cells response

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5
Q

What is the normal light chains excess produced by plasma cells and excreted renally?

A

10 mg/day max.

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6
Q

What cell stage is missing in this hematopoietic lineage?

Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
\_\_\_\_\_\_\_\_\_
Immature B cell
Mature B cell
Plasma cell
A 
Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
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7
Q

What cell stage is missing in this hematopoietic lineage?

Pluripotent Stem cell
Common Lymphoid Precursor cell
\_\_\_\_\_\_\_\_\_
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
A 
Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
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8
Q

What cell stage is missing in this hematopoietic lineage?

\_\_\_\_\_\_\_\_\_\_
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
A 
Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
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9
Q

What cell stage is missing in this hematopoietic lineage?

Pluripotent Stem cell
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
A 
Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
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10
Q

What cell stage is missing in this hematopoietic lineage?

Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
\_\_\_\_\_\_\_\_\_\_\_\_\_
Mature B cell
Plasma cell
A 
Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
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11
Q

What cell stage is missing in this hematopoietic lineage?

Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
\_\_\_\_\_\_\_\_\_\_\_
Plasma cell
A 
Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
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12
Q

What cell stage is missing in this hematopoietic lineage?

Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
\_\_\_\_\_\_\_\_\_\_
A 
Pluripotent Stem cell
Common Lymphoid Precursor cell
Pro-B cell
Pre- B cell
Immature B cell
Mature B cell
Plasma cell
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13
Q

What is the classic description of a serum electrophoresis in a state of a plasma cell disorder?

A

M-Component - Additional Peak of γ Globulins

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14
Q

Where are B cells formed? Matured?

A

Bone Marrow

Bone Marrow

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15
Q

BCR - which Igs?

A

IgM and IgD

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16
Q

Processes of the Development of B cells after Antigen meeting?
Ending in a Plasma cell

A

Activation
Affinity Maturation
Differentiation
Isotypes Switch

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17
Q

Negative Selection of B cells - Outcomes

A

1- Apoptosis of Auto-reactivity B cells
2 - Central tolerance:
Receptor Modification,New Light chain expression

Ultimately - No auto-reactivity

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18
Q

Classical B cells in Lymph Node Cortex Follicles - which response kind?

A

TD - Require T cells
For Protein antigens
Long Lived Plasma cells, Isotype switched

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19
Q

Lymph Node Marginal area B cells response - what kind?

A

TI - No T cell
Lipids and Polysaccharides antigens
Short lived plasma cells - IgM

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20
Q

What are the co-stimulatory molecules on TH2 and B cells?

TD, B cell Activation - 3 By order

A

BCR+Antigen - TCR
CD40 - CD40L
IL21R - IL21

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21
Q

Isotype (Class) Switch - Signals leading to which Antibody constant regions by B cell

A

IFN gamma - IgG
IL5 - IgA
IL4/IL13 - IgE

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22
Q

Serum electrophoresis in a state of a plasma cell disorder - Quantitative / Qualitative?

A

Quantitative

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23
Q

Immunoelectrophoresis in a state of a plasma cell disorder - Quantitative / Qualitative?

A

Qualitative

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24
Q

Immunoelectrophoresis

What does it show?

A

Check the Ig Type and if Monoclonal

Presents Heavy or Light Chain

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25
Q

In 20% of Multiple Myeloma Cases there is Light Chain Secretion that will turn out as ____ ______ Protein in Urine.

A

In 20% of Multiple Myeloma Cases there is Light Chain Secretion that will turn out as Bence Jones Protein in Urine.

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26
Q

In Extramedullary Plasmacytoma or Solitary Bone Plasmacytoma only third of Patient will present __________ on serum electrophoresis.

A

In Extramedullary Plasmacytoma or Solitary Bone Plasmacytoma only third of Patient will present M-Component on serum electrophoresis.

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27
Q

In what other Neoplasms will we see a M-Component on serum electrophoresis?

A

CML, CLL, Breast and Colon Cancer

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28
Q

In what Non-neoplastic states will we see a M-Component on serum electrophoresis?

A 
Cirrhosis
Sarcoidosis
Gaucher's Disease
Parasitic Infections
Pyoderma gangrenosum
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29
Q

In what Autoimmune Diseases will we see a M-Component on serum electrophoresis?

A

Rheumatoid Arthritis
Myasthenia Gravis
Cold Agglutinin Disease

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30
Q

Multiple Myeloma - Definition

A

Monoclonal Plasma cell Malignant Proliferation

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31
Q

Multiple Myeloma - Risk factors

A

Radiation, Pesticides, also Leather, Wood and Crude oil at Workplace

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32
Q

Multiple Myeloma - Typical Chromosomal Aberrations

A

Hyperdiploidy ,13q14 deletion, 17p13 deletion

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33
Q

Multiple Myeloma - Typical Chromosomal Translocations

A

t(11;14)(q13;q32), t(4;14)(p16;q32), t(14;16) , 1q amplification or 1p deletion

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34
Q

Activation
Affinity Maturation
Differentiation
Isotypes Switch

Which process was found to damaged in MM ?

A

Isotypes Switch

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35
Q

What are some Mutations common in MM (not determined as causative) ?

A

K-ras
N-ras
B-raf

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36
Q

What Interleukin is suspected to have a role in malignant proliferation in MM ?

A

IL6

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37
Q

What hematologic malignancies lab test is usually useless in Multiple myeloma? Why?

A

Peripheral Blood Smear

Monoclonality is Undetectable

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38
Q

Multiple Myeloma incidence rises with -

A

older age, rare to encounter under 40

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39
Q

Multiple Myeloma Median diagnostic age is -

A

70

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40
Q

Multiple Myeloma is more common in Men/Women ?

A

Men

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41
Q

Multiple Myeloma is twice as common in African-Americans/Caucasians?

A

African-Americans > x2 Caucasians

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42
Q

What is the common mediterranean subtype of Multiple Myeloma?

A

Immunoproliferative small intestinal disease

IPSD

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43
Q

What are the clinical manifestations of Multiple Myeloma? “CRAB”

A

Calcemia (Hyper) - CNS & Clotting disorders
Renal Injury
Anemia & Infections
Bone Lytic lesions

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44
Q

What is the most common symptom in MM ? What does in arise from ?

A 
Bone Pain (70%)
Osteoclast Activating Factors formed by Tumor cells and Subsequent Pathological Fractures
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45
Q

What are the consequences of the lytic bone lesions in MM ?

A

Hypercalcemia with complications such as spinal cord compression, weakness, retinopathy, radicular pain.

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46
Q

Infections is the second most common symptom (after bone pain) in MM. What does in arise from ?

A

Hypogammaglobulinemia if M-Component is excluded T17 rise and Terg Malfunction
Granulocyte Lysosomal depletion with slow migration
Complement Malfunctions
Immunosuppressive treatments

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47
Q

What are the most common infections locations in MM?

A

Pneumonia and Pyelonephritis

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48
Q

What are the most common pathogens in MM ?

A

Strep. Pneumo, Staph. Aureus, Klebsiella Pneumo.

E. Coli in Pyelonephritis

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49
Q

AKI will appear in __% of patients and ESRD will occur in __% of patients of MM.

A

Renal Injury will appear in 50% of patients and ESRD will occur in 25% of patients of MM.

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50
Q

What are the causes for AKI in patients with MM ? (8)

A 
Hypercalcemia
NSAIDs
CIN
Hyperuricemia
Reccurent Infections
Amyloid Deposits
Bisphosphonates
Myeloma Tumor cells Infiltration
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51
Q

Tubular damage will almost always arise in MM due to -

A

Light Chain Deposition

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52
Q

What are the Normal Physiological processes that light chains go through in the kidney and Later on?

A

Filtration
Reabsorption
Catabolism

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53
Q

What is the indirect effect of Light Chain Deposition that enhances the toxic direct tubular damage?

A

Intracellular Lysosomal Enzymes Releases

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54
Q

What is the earliest manifestation of AKI as result of MM ? What is the most common cause of AKI here?

A

Adult Fanconi Syndrome - RTA type 2, Glucose and AA loss.

Hypercalcemia!

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55
Q

What is unique about the proteinuria in MM ?

A

Without Hypertension. Mostly Light Chains - “Bence Jones Protein”.

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56
Q

What is the glomerular effect of MM ?

A

Usually no effect - Correct Albumin levels

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57
Q

Patients with MM may have a _______ anion gap because M-Component is ______- leading to Chloride Retention.

A

Patients with MM may have a decreased anion gap because M-Component is Cationic - leading to Chloride Retention.

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58
Q

What does the high Antibodies concentration leads to on blood test presentation?

A

Pseudohyponatremia

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59
Q

What is the cause of ESRD in MM ?

A

Amyloid Nephropathy - AL amyloid from Light Chains

60
Q

What can exacerbate AKI in MM patients?

A

Dehydration

Medications such as Bisphosphonates or NAIDS

61
Q

What neurological symptoms does hypercalcemia cause in MM ?

A

Lethargy, Weakness, Depression

62
Q

What neurological symptoms does hyperviscosity syndrome cause in MM ?

A

Headaches, Vertigo, Blurred Vision and Retinopathy

Coma

63
Q

What neurological symptoms does lytic bone lesions cause in MM ?

A

Spinal Cord Compression, Radicular Pain, Incontinence

64
Q

What neurological symptoms does amyloid deposition cause in MM ?

A

Carpal Tunnel Syndrome with Sensory Polyneuropathy

65
Q

Sensory Polyneuropathy in MM - what can cause that, apart from Amyloid ?

A

Treatment: Bortezomib or Lenalidomide

66
Q

What are the emergencies that can arise from MM ?

A 
Spinal Cord Compression
Pathological Fracture
Hyperviscosity Syndrome
Sepsis
Severe Hypercalcemia
67
Q

Diagnosis of Multiple Myeloma

  1. __%< CD138+ Plasma cell level with Kappa/Lambda Monoclonality.
  2. M Component in Serum or Urine
  3. Myeloma Defining Event
A

Diagnosis of Multiple Myeloma

  1. 10%< CD138+ Plasma cell level with Kappa/Lambda Monoclonality.(30 g/L)
  2. M Component in Serum or Urine
  3. Myeloma Defining Event - Pathological Fractures i.e
68
Q

Diagnosis of Multiple Myeloma

  1. 10%< CD138+ Plasma cell level with Kappa/Lambda Monoclonality.
  2. ________ in Serum or Urine
  3. Myeloma Defining Event
A

Diagnosis of Multiple Myeloma

  1. 10%< CD138+ Plasma cell level with Kappa/Lambda Monoclonality. (30 g/L)
  2. M Component in Serum or Urine
  3. Myeloma Defining Event - Pathological Fractures i.e
69
Q

Diagnosis of Multiple Myeloma

  1. 10%< CD138+ Plasma cell level with Kappa/Lambda Monoclonality.
  2. M Component in Serum or Urine
  3. ________________
A

Diagnosis of Multiple Myeloma

  1. 10%< CD138+ Plasma cell level with Kappa/Lambda Monoclonality.(30 g/L)
  2. M Component in Serum or Urine
  3. Myeloma Defining Event - Pathological Fractures i.e
70
Q

What are the Diseases on the DD of MM ?

A

Smoldering Multiple Myeloma

Monoclonal Gammopathy of Unknown Significance

71
Q

Monoclonal Gammopathy of Unknown Significance

Risk Factor for transition to MM ?

A

Non-IgG Subtype
Abnormal Kappa/Lambda Ratio (Not 60%-40%)
Serum M-Component above 15 g/L

72
Q

Smoldering Multiple Myeloma

Risk Factor for transition to MM ?

A

10%< CD138+ Plasma cell level
Abnormal Kappa/Lambda Ratio (Not 60%-40%)
Serum M-Component >30 g/L

73
Q

What are the common variants of MM ?

A

Solitary Bone Plasmacytoma

Solitary Extramedullary Plasmacytoma

74
Q

Solitary Extramedullary Plasmacytoma

A

Lesions of lymphatic tissue of nasopharynx or paranasal sinuses

75
Q

What is the physical examination principles for checking for MM ?

A

Looking for Masses and Tender Bones

76
Q

What is purpose of each Imaging test in MM ?

A

X-Ray: Lytic Bone Lesions of Skull
MRI: Spinal cord Compression or Vertebral Lesions
PET/CT: Follow up of MM and Diagnosis of Solitary Bone Plasmacytoma

77
Q

What are the lab tests from MM ?

A

CBC for Pancytopenia
ESR for Inflammatory state
Chemistry - Calcium, Urate, Creatinine, BUN
ALP - to rule out Paget Disease

78
Q

M-Component - How is it recognized?

A

Protein Electrophoresis

24 hour Bence Jones in Urine

79
Q

Which light chain usually has worse prognosis ?

A

Lambda

80
Q

What is the risk in patients with an IgM related MM ? tx?

A

Hyperviscosity Syndrome

Plasmapheresis!

81
Q

What is the strongest prognostic factor in MM ? Why?

A

B2 Microglobulin

Present on MHC-1 released by Tumor cells evade immune surveillance / cell turnover

82
Q

ISS - What are the lab values taken into account?

A

B2 Microglobulin & Albumin

83
Q

What are other prognostic factors apart from B2 Microglobulin ?

A

LDH
Microalbumin/B2 Microglobulin (80% Renal involv.)
Cytogenetic Variations
Patient disabilities

84
Q

What is the treatment for High risk transition type MGUS ?

A

Twice yearly - Electrophoresis for M component +

Observation: CBC, Creatinine and Calcium

85
Q

What is the most important question one has to ask after diagnosing MM ? What does it depend on? Why is it Important during MM medical care?

A

Transplant ineligible? - age above 70 and co-morbidities

If Eligible - Usage of Melphalan will prevent ASCT!

86
Q

What is the treatment of MM in Transplant ineligible patients?

A

Melphalan! , Prednisone and Bortezomib

Lenalidomide as maintenance later

87
Q

What is the treatment of MM in Transplant eligible patients?

A

Lenalidomide, Dexamethasone and Bortezomib

Lenalidomide as maintenance later

88
Q

What is maintenance therapy of MM ? What is the Adverse Effect? What is the prevention?

A

Lenalidomide, May lead to DVT, Prevention with Low-dose Aspirin

89
Q

What is the name of the proteasome inhibitor treatment for MM ? what is the Adverse effect?

A

Bortezomib - Immunosuppression - Herpes Zoster Flares

90
Q

What are the Osteoblast inhibitor & Osteoclast activators set out from malignant plasma cells in MM ?

A

Osteoblast inhibitor - DKK-1

Osteoclast activators - IL1 & IL6 lead to RANKL↑

91
Q

What kind of malignancy is yielding cells releasing specifically IgMs ?

A

Waldenstrom’s Macroglobulinemia

92
Q

What is the most common syndrome associated with WM ?

A

Hyperviscosity Syndrome

93
Q

What is the DD of WM?

A

MM
CLL
Marginal Zone Lymphoma

94
Q

What is the median age of WM ? what is the dominant sex?

A

64

Men

95
Q

What is the difference from MM, when examining WM bone involvement ?

A

10%< CD138+ Plasma cell level with Kappa/Lambda Monoclonality.(30 g/L) in BM BUT - not Lytic Bone Lesions therefore no Hypercalcemia and no AKI usually.

96
Q

In 80% of WM the light chain in the IgM will be -

A

Kappa

97
Q

What is the diagnostic test that allows differentiation of WM from MM(IgM8) , CLL and Marginal Zone Lymphoma?

A

Somatic Mutation of MYD88 in L265P position.

98
Q

What will we see in WM patient with MAG IgM? What is MAG?

A

MYELIN ASSOCIATED GLOBULIN

NEUROPATHY

99
Q

What are the common symptoms of WM and MM ?

A

Recurrent Infections
Weakness
Lethargy

100
Q

What are the symptoms unique for WM and less appearing on MM ?

A

Hyperviscosity Syndrome - Epistaxis, Blurry Vision, Neuropathy.
Hepatosplenomegaly.
Raynaud Phenomenon

101
Q

What are lab results unique for WM and less appearing on MM ?

A

Rouleaux Formations (ESR)
Positive Coombs test
Malignant Lymphocytes (Peripheral Blood Smear)
10% are Pure Cryoglobulins (leading to Raynaud)

102
Q

Parameters for high risk WM ?

A

Older age
Male
Symptomatic WM with Cytopenia

103
Q

What is the treatment for Hyperviscosity Syndrome leading to Paralysis or Loss of Consciousness? Why?

A

Plasmapheresis - 80% of IgM is Intravascular

104
Q

First Line Treatment for WM ?

A

No specific defined treatment - tailor for each patient:

Ibrutinib - BTKi, Rituximab , Cyclophosphamide. Bortezomib

105
Q

What is Amyloid?

A

Misfolded protein deposits in extracellular spaces that causes tissue damage.

106
Q

What is the biochemical form of an Amyloid?

A

Beta-Pleated Sheet

107
Q

What is the staining used in Amyloidosis? What other Laboratory may reveal it?

A

Congo Red on Histology

Apple Green Birefringence in Polarized light

108
Q

What are the clinical findings of Systemic Amyloidosis?

A

Nephrotic Syndrome; Ascites & Edema
RCM -Distant Heart sound, S4 & S3 and Arrhythmia
Glossomegaly and Hepatosplenomegaly
Carpal Tunnel Syndrome

109
Q

What are the subtypes of Systemic Amyloidosis?

A

Primary Amyloidosis

Secondary Amyloidosis

110
Q

What kind of Amyloidosis is derived from elevated Serum Amyloid A (SAA) in Chronic Inflammatory Diseases or Malignancies?

A

Secondary Amyloidosis

Possibly in FMFm Brucella, TB, HCV, RA, IBD, etc..

111
Q

What kind of Amyloidosis is derived from elevated Amyloid Light Chain (AL) in Plasma cell Dyscrasias?

A

Primary Amyloidosis

112
Q

What are the organ specific/localized Amyloidosis subtypes?

A

Senile Cardiac Amyloidosis
Familial Amyloid Cardiomyopathy
T2DM related Amyloidosis
Alzheimer’s Disease

113
Q

What are the organ specific/localized Amyloidosis subtype that is related to elevated Serum Transthyretin?

A

Senile Cardiac Amyloidosis

114
Q

What are the organ specific/localized Amyloidosis subtype that is related to elevated Mutated Serum Transthyretin?

A

Familial Amyloid Cardiomyopathy

115
Q

What are the organ specific/localized Amyloidosis subtype that is related to elevated Serum Amylin?

A

T2DM

116
Q

What are the organ specific/localized Amyloidosis subtype that is related to elevated Serum A-beta Amyloid?

A

Alzheimer’s Disease

117
Q

What kind of diseases are leading to Amyloidosis derived from elevated Amyloid Light Chain (AL)?

A

Multiple Myeloma
Waldenstrom’s Macroglobulinemia
CLL
Lymphoma

118
Q

What is the typical Light chain present in Primary Amyloidosis?

A

Lambda

119
Q

What is the Most common organ damage resulting from Primary Amyloidosis? Characteristic Features?

A

Renal - Nephrotic Syndrome (80%)

Proteinuria, Hypoalbuminemia, Hyperlipidemia and Edema with possible Anasarca.

120
Q

Primary Amyloidosis - What is the course of the disease?

A

Rapidly Progressing Disease

121
Q

What is the Second Most common organ damage resulting from Primary Amyloidosis? Characteristic Features? What is the DD ?

A

Cardiac - Hypertrophy and following Restrictive Disease. Low Voltage ECG.
Diagnosed with TEE/TTE, the DD is Hemochromatosis or Sarcoidosis.

122
Q

What are the classical Neuropathy signs for Primary Amyloidosis?

A

Sensory and Motor Neuropathy are present bu Autonomic is the more specific - Neurogenic Bladder, Gastroparesis and Orthostatism.

123
Q

What is the Rare yet Pathognomonic Sign for Primary Amyloidosis?

A

Macroglossia (10%)

124
Q

Facial Features of Primary Amyloidosis?

Why is it present?

A 
Periorbital Ecchymosis ("Raccoon Eyes") with Alopecia
Easy Bruising - Deposition with Factor X binding.
125
Q

Primary Amyloidosis leads to ALP↑, GGT↑ and Direct Bilirubin↑.
Why is it present?

A

Cholestasis with Hepatosplenomegaly

Obstructive Hepatitis by Amyloid deposits

126
Q

What are the diagnostic work-up steps in suspicion of Primary Amyloidosis?

A
  1. Urine/Blood Collection of Monoclonal Light Chains.
  2. Non-Invasive Biopsy
  3. Invasive Biopsy
127
Q

Non-Invasive Biopsy - suspicion of Primary Amyloidosis, What are the collection sites?

A

Peritoneal
Rectal
Areolar
Vaginal

128
Q

Invasive Biopsy - suspicion of Primary Amyloidosis, What are the collection sites?

A

Renal
Cardiac
Liver
Spleen

129
Q

Easy Bruising is caused by Primary Amyloidosis Deposition with Factor X binding, this leads to higher risk for DVT, what else might lead in this case to a higher risk for DVT?

A

Amyloid Nephrotic Syndrome leads to higher loss of Antithrombin, Protein C and S - Hypercoagulable State

130
Q

What are the different heavy chain diseases?

A

Alpha Heavy Chain disease
Gamma Heavy Chain disease
Miu Heavy Chain disease

131
Q

What is the most common heavy chain disease? Symptoms and Treatment?

A

Alpha Heavy Chain Disease
Chronic Diarrhea, Weight Loss, Mesenteric and Para-aortic Lymphadenopathy.
Chemo with Antibiotics.

132
Q

What heavy chain disease will most probably lead to Palate Inflammation? Other Symptoms and Treatment?

A

Gamma Heavy Chain disease
May also cause Lymphadenopathy, Hepatosplenomegaly, Fever, Anemia, Myalgia.
Tx in Symptomatic Disease - Chemo with Rituximab

133
Q

What does POEMS Syndrome involve? (POEMS)

A 
Polyneuropathy
Organomegaly
Endocrinopathy
Monoclonal-protein
Skin Changes
134
Q

What does POEMS Syndrome includes in Polyneuropathy?

A

Sensory Neuropathy with Sclerotic Lesions in bone

135
Q

What does POEMS Syndrome includes in Organomegaly?

A

Lymphadenopathy
Hepatomegaly
Splenomegaly

136
Q

What does POEMS Syndrome includes in Endocrinopathy?

A

Hyperprolactinemia
DM1/2
Adrenal Insufficiency
Hypothyroidism

137
Q

What does POEMS Syndrome includes in Skin Changes?

A

Hyper-pigmentation
Hypertrichosis
Skin Thickening
Clubbing

138
Q

What is the classical Treatment for POEMS Syndrome?

A

Lenalidomide, Dexamethasone and Bortezomib
Plasmapheresis is NOT EFFECTIVE
Radiation of Sclerotic Lesions.

139
Q

What is the supportive treatment for Anemia resulting from Multiple Myeloma or Waldenstrom’s macroglobulinemia? What is the Type of Anemia is present ?

A

EPO, Iron, Folic Acid and Vitamin B12

For Normocytic Normochromic Anemia of MM or WM.

140
Q

What is a unique electrolyte abnormality present in MM? Why?

A

Pseudohyponatremia

Elevated proteins in serum cause that (M Component)

141
Q

What is an important immunological treatment for MM induced URTI or UTI? What is a side effect and response to it?

A

IVIG for Infections in MM

Side effect - Aseptic Meningitis: LP for Pressure release is needed.

142
Q

MM Parameters for Prognostic Stage - Stage I ?

A

B2M<3.5 & ALB>=3.5

143
Q

MM Parameters for Prognostic Stage - Stage II ?

A

B2M<3.5 & ALB<3.5
OR
B2M = 3.5-5.5

144
Q

MM Parameters for Prognostic Stage - Stage II ?

A

B2M > 5.5

145
Q

What is the actual process of Plasmapheresis?

A

Removal and Filtration of 15% of Plasma

GBA - Y4 (12 decks)

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