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Hematology > Plasma Cell Disorders > Flashcards

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
Q

FLC can deposit in

A

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.

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26
Q

Excess monoclonal immunoglobin can lead to

A
  1. Hyperviscosity
  2. Decreased production of normal immunoglobin
  3. Cryoglobins
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27
Q

Hyperviscosity syndrome

A

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

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28
Q

plasmapheresis

A

plasma exchange

29
Q

Decreased produciton of normal immunoglobin

A

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
Q

Cryoglobulins

A

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
Q

Multiple Myeloma (MM)

A

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
Q

MM Major symptoms

A

Anemia, bone pain, increased infections

33
Q

MM survival

A

varies ..

a few months - 15 years

34
Q

MM pathophysiology

A
  1. plasma cell population expansioon
  2. BM stroma interaction
  3. Osteoclast activation
  4. Bone disease
  5. Hypercalcemia
35
Q

MM plasma cell expansion

A

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
Q

MM interaction with BM stroma

A

BM microenvironment plays important role in supporting the malignant plasma cell (myeloma cells)
- promotes growth and prevents apoptosis

37
Q

Myeloma cells adhere to stromal cells inducing cytokine secretion which mediate the disease

A
  • Bone destruction (activating osteoclasts)
  • Tumor cell proliferation
  • drug resistance
38
Q

MM osteoclast Activation

A

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
Q

TRANCE activates

A

osteoclasts, which absorbs bone

40
Q

MM bone disease

A

Results from disturbed balance of bone formation and absorption

Increased bone absorption due to increased osteoclasts activation

41
Q

MM bone disease causes

A
  • lytic bone lesions
  • osteoprosis with fractures and/or spinal cord compression
  • vertebral collapse
42
Q

MM hypercalcemia

A

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
Q

calcium balance

A

plays ciritical role in regulation of cellular function

44
Q

MM hypercalcemia

mental status

A

confusion

45
Q

MM lab findings

A
  • 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
Q

MM ESR

A

Increased Sedimentation Rate (ESR)
- due to rouleaux and increased Ig in plasma

47
Q

MM chemistry studies

A

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
Q

M-spike

A
  • monoclonal peak in gamma region
  • immunoglobin IgG
49
Q

Reduced albumin

A

production inhibited by cytokines secreted

50
Q

MM BM examination

A

10-30% plasma cells
Types of cells:
- flame cells
- Mott cells
- Russel Bodies

51
Q

flame cell (MM)

A

abundant glycoprotein and ribosomes give cell intense staining

52
Q

Mott cells (MM)

A

contains multiple Russell bodies

53
Q

Russel bodies (MM)

A

inclusions containing immunoglobulins

54
Q

MM cytogenetics

A

Chromosomal abnormaltities found in 40% of patients

Chromosomes 14 band q32 are the most common
- site of immunoglobin heavy chain locus

55
Q

MM diagnostic criteria

A

BM clonal plasma cells
Monoclonal protein (M-spike); serum or urine

56
Q

Related organ or tissue impatiment (CRAB)

A

Calcium
Renal
Anemia
Bone

57
Q

C (CRAB)

A

Calcium

Ca > 11mg/dL

58
Q

R (CRAB )

A

Renal

Creatinine >2mg/dL

59
Q

A (CRAB)

A

Anemia

Hgb<10g/dL

60
Q

B (CRAB)

A

Bone

Lytic lesions/osteoprosis

61
Q

other diagnostic criteria MM

A

recurrent bacterial infection, hyperviscosity, amyloidosis

62
Q

Plasma cell leukemia

A

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
Q

Waldenstrom’s Macroglobulinemia

A

malignant lymphoproliferative disorder of plasma cytoid lymphocytes

IgM monoclonal immunoglobulins
- coat platelets impeding their function and causing complicagions

Found often in older males

64
Q

Waldenstroms macroglobulinemia

symptoms

A

fatigue, bleeding, weakness

65
Q

PB smear

Waldenstroms Macroglobulinemia

A

rouleaux, small lymphs with plasmacytoid features

66
Q

Serum protein electrophoresis
Waldenstroms Macroglobulinemia

A

monoclonal peak - increased IgM

67
Q

Waldenstrom macroglobulinemia

A

incresed blood viscosity
increased cryoglobulins

68
Q

monoclonal gammopathy of undetermined significance (MGUS)

A

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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