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Plasma Cell Disorders Flashcards

1
Q

Plasma cell

A

terminally differentiated, non dividing cell representing final stage of B cell lymphocyte

requires antigen dependent maturation

Main fucntion: production of antibodies (immunoglobin)

Life span: approx. 1 month

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2
Q

Plasma cell strutcure

A
  • round, eccentric nucleaus with coarse chromatin (may have clock face)
  • Abundant basophilic cytoplasm
  • Prominent perinuclear hof (clearing)
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3
Q

immunoglobin structure and function

A

Composed of 2 homologous heavy chains and 2 homologous light chains

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4
Q

Heavy chains of immunoglobin

A

gamma (IgG, alpha(IgA), mu(IgM), delta(IgD), episilon (IgE)

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5
Q

light chains

A

kappa or lambda

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6
Q

immunoglobin overproduction is

A

hallmark of plasma cell disorders

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7
Q

serum protein contains:

A

Albumin ( majority)
Alpha Globulins
- a1 - antitrypsin (small amt)
- a2 - macroglobulin

Beta Globulun
- beta-lipoprotein and transferring (small amt)

Gamma globulin - immunoglobin (remaining amt)

Serum preotein and albumin measurement allow us to approximate immunoglobin (Ig fraction)

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8
Q

estimated Ig Fraction =

A

total serum protein - albumin

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9
Q

Electrophoresis

A

Seperates the serum proteins into albumin, alpha, beta and gamma globulin

increased immunoglobin -> gamma region

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10
Q

a1 - antitrypsin TGB

A

a1

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11
Q

a2

A

haptoglobin
ceruloplasmin
a2- macroglobulin

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12
Q

monoclonal

A

Abs derived from single anestral cell

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13
Q

polyclonal

A

Abs derived from more than one Ab producing cell

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14
Q

spikes in electophoresis caused by monoclonal Ig

A

monoclonal spike or M-spike

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15
Q

further identification of the M-spike protein is carried out by

A

immunofixation

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16
Q

immunofixation

A
  1. Migrate 5 seperate lanes, mono-specific Ab (toward heavy or light chain) are added
  2. Mono-specific antibodies bind to antigen of serum antibodies (heavy or light chain and complex)
  3. complexes precipitate and are measured

An M spike is characterized by a combinaiton of
- sharp, well defined band associated with a single heavy chain
- sharp, well defined band associated with a specific light chain

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17
Q

plasma cell disorders display

A

monoclonal spike

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18
Q

Ig classes are quantitated by

A

rate nephleometry

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19
Q

patient serum is mixed with specific

A

reagent to create Ag-Ab complexes

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20
Q

Ag-Ab causes

A

light scatter which is measured

helps us accurately quantitate Ig, but does NOT assess monoclonality

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21
Q

nephelometry is useful in combination with

A

electrophoresis, in following plasma cell disorders

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22
Q

Ig light chains in urine

A

Bence-Jones Proteinuria

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23
Q

imbalanced Ig production most frequently yields

A

excess free light chains (FLC)

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24
Q

increased FLC

A

leads to filtration in urine
- called Bence-Jones proteinuria

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25
FLC can deposit in
kidney - lead to more leakage of protein into the urine - severe -- kidney failure Use 24 hr urine collections to perform to perform a precipitation or electrophoresis assay for determination.
26
Excess monoclonal immunoglobin can lead to
1. Hyperviscosity 2. Decreased production of normal immunoglobin 3. Cryoglobins
27
Hyperviscosity syndrome
Excess immunoglobin causes blood to become viscuos - higher resitance to flow in bood vessels - decreased blood flow in small vessels of vital organs - increased workload on the heart Leads to symptoms of - confusion - headache - dementia - distrubances of conciousness - stroke - and/or coma Plasma exchange (**Plasmapheresis**) is an emergency therapy for symptomatic patients
28
plasmapheresis
plasma exchange
29
Decreased produciton of normal immunoglobin
Pateints have decreased levels of normal immunoglobin - from suppression of normal plasma cells Low "normal" Ig level leads to increased susceptibility to infections, especially respiratory infections such as - sinusitis - bronchitis - pneumonia Seen in multiple myeloma and waldenstrom's macroglobinemia - infection is the leading cause of death in multiple myeloma patients IV infusions of Ig can help decrease frequency of these infections.
30
Cryoglobulins
Serum immunoglobins that precipitate reversibly on exposure to cold temps Can affect organs, but usually patient suffers with painful extremeities exposed to cold In severe cases, may result in vasculitis and kidney damage
31
Multiple Myeloma (MM)
Most common plasma cell dyscrasia Older patients (media = 67 yrs old) Plasma cells generally stay localized in BM until late in disease - then we begin to see in PB - leading to plasma cell leukemia
32
MM Major symptoms
Anemia, bone pain, increased infections
33
MM survival
varies .. a few months - 15 years
34
MM pathophysiology
1. plasma cell population expansioon 2. BM stroma interaction 3. Osteoclast activation 4. Bone disease 5. Hypercalcemia
35
MM plasma cell expansion
Single cell establishes a malignant clone Malignant cell proliferates into plasma cell colonies Begins gradually replacing normal BM leding to pancytopenia - 1st anemia = decrease in RBCs - 2nd thrombocytopenia - decrease in platelets - 3rd neutropenia - decrease in neutrophils
36
MM interaction with BM stroma
BM microenvironment plays important role in supporting the malignant plasma cell (myeloma cells) - promotes growth and prevents apoptosis
37
Myeloma cells adhere to stromal cells inducing cytokine secretion which mediate the disease
- Bone destruction (activating osteoclasts) - Tumor cell proliferation - drug resistance
38
MM osteoclast Activation
Osteoclast activating cytokines stimulate tumor necrosis factor-related induced cytokine (**TRANCE**) - usually it is blocked by Osteoprotegrin (**OPG**), but OPG becomes trapped by myeloma cells and overwhelmed/unbalanced.
39
TRANCE activates
osteoclasts, which absorbs bone
40
MM bone disease
Results from disturbed balance of bone formation and absorption Increased bone absorption due to increased osteoclasts activation
41
MM bone disease causes
- lytic bone lesions - osteoprosis with fractures and/or spinal cord compression - vertebral collapse
42
MM hypercalcemia
increased bone turn over leads to calcium being released in the blood - constipation (intestine motility) - increased urination - dehydration - muscle weakness - kidney stones/ kidney failure (increased failure)
43
calcium balance
plays ciritical role in regulation of cellular function
44
MM hypercalcemia mental status
confusion
45
MM lab findings
- decreased RBC (normocytic, normochromic RBCs) - decreased WBC - decreased PLT Rouleaux - due to excess Ig in plasma Circulating plasma cells - late in disease - poor prognosis - can transition to Plasma Cell Leukemia
46
MM ESR
Increased Sedimentation Rate (ESR) - due to rouleaux and increased Ig in plasma
47
MM chemistry studies
increased calcium - due to bone destruction and low albumin Possible increased LDH (Lactate Dehydrogenase) - non-specific for tissue breakdown Monitor BUN (blood urea nitrogen) and Creatinine - asssesses kidney function
48
M-spike
- monoclonal peak in gamma region - immunoglobin IgG
49
Reduced albumin
production inhibited by cytokines secreted
50
MM BM examination
10-30% plasma cells Types of cells: - flame cells - Mott cells - Russel Bodies
51
flame cell (MM)
abundant glycoprotein and ribosomes give cell intense staining
52
Mott cells (MM)
contains multiple Russell bodies
53
Russel bodies (MM)
inclusions containing immunoglobulins
54
MM cytogenetics
Chromosomal abnormaltities found in 40% of patients **Chromosomes 14 band q32 are the most common** - **site of immunoglobin heavy chain locus**
55
MM diagnostic criteria
BM clonal plasma cells Monoclonal protein (M-spike); serum or urine
56
Related organ or tissue impatiment (CRAB)
Calcium Renal Anemia Bone
57
C (CRAB)
Calcium Ca > 11mg/dL
58
R (CRAB )
Renal Creatinine >2mg/dL
59
A (CRAB)
Anemia Hgb<10g/dL
60
B (CRAB)
Bone Lytic lesions/osteoprosis
61
other diagnostic criteria MM
recurrent bacterial infection, hyperviscosity, amyloidosis
62
Plasma cell leukemia
rare multiple myeloma variant (2-3%) Definde as: - circulating plasma cells >2000/uL - plasma cells more than or equal to 20% peripheral WBC Panocytopenia - decrease: WBC, RBC, PLT Hyperfammaglobulinemia - IgD and IgE moslty Can be found in advanced multiple myeloma - usually terminal event Organomegaly, anemia, bleeding
63
Waldenstrom's Macroglobulinemia
malignant lymphoproliferative disorder of plasma cytoid lymphocytes IgM monoclonal immunoglobulins - coat platelets impeding their function and causing complicagions Found often in older males
64
Waldenstroms macroglobulinemia symptoms
fatigue, bleeding, weakness
65
PB smear Waldenstroms Macroglobulinemia
rouleaux, small lymphs with plasmacytoid features
66
Serum protein electrophoresis Waldenstroms Macroglobulinemia
monoclonal peak - increased IgM
67
Waldenstrom macroglobulinemia
incresed blood viscosity increased cryoglobulins
68
monoclonal gammopathy of undetermined significance (MGUS)
Previously known as benign monoclonal gammopathy small M-spike with normal levels of uninvolved Ig Fewer than 10% plasma cells on BM aspirate No related organ impairment 20% will develop malignancy, such as multiple myeloma, lymphoma, or chronic lymphocytic leukemia No treatment needed, but must be monitered for life

Hematology (36 decks)

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