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HEMATOLOGY > Myeloma > Flashcards

Myeloma Flashcards

1
Q

Which immune system are the B-cells a part of?

What are their roles?

A
  1. part of the adaptive immune system

2. Roles: Ab prodn/ Antigen presenting cells

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2
Q

What are immunoglobulins?

A
  • Abs made by B-cells and plasma cells
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3
Q

Where do B-cells develop?

A

in the Bone marrow

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4
Q

How is the Ig variable element genrated from?

A
  • from the V-D-J region recombination found at Ag binding site
  • early in development
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5
Q

What do the naive B cells do in the periphery?

A
  • travel to the FOLLICLE germinal centre of the lymph node
  • identify the Ag and improve the fit by somatic mutation or be deleted.
  • may return to marrow as a plasma cell or as a memory B-cell
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6
Q

What does a b-cell appear on histology?

A
    • clock-face nucleus
  • open chromatin (synthesizes mRNA)
  • -plentiful BLUE cytoplasm (laden with protein)
  • –pale perinuclear area (gogli apparatus)
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7
Q

What is polyclonal incr. in immunoglobulins?

Why may it occur?

A
  • produced by MANY diff. plasma cell CLONES

- reactive to INFECTION/ AUTOIMMUNE/ MALIGNANCY/ LIVER DISEASE

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8
Q

What is monoclonal rise in immunoglobulins?

A
  • Ig all derived from clonal expansion of a SINGLE B-cell —-IDENTICAL Ab specificity and structure
    monoclonal Ig= paraprotein
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9
Q

What may monoclonal rise in Ig indicate?

A

marker of UNERLYING clonal B-cell disorder.

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10
Q

How are Ig detected?

A
  • by serum electrophoresis
  • —serate serum proteins appear as dstinct bands
  • –proteins move at differing rates determined by their SIZE and CHARGE
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11
Q

Where willl albumin be seen on electrophoresis?

A
  • given it’s the MOST NEGATIVELY charged molecule

- —it will be seen close to ANODE

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12
Q

Why is there a rise in the amount of free light chains in the plasma and in the urine?

A
  • when Ig is produced in the plasma cells, MORE LIGHT chains are made than heavy chains
  • –the free light chains are secreted into the plasma along with intact Ig
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13
Q

IN what conditions may free light chains INCREASE in the plasma?

A
  • polyclonal INCREASE (d.t INFECTION)
  • MONOCLONAL incr. in plasma cells (d.t multiple myeloma)

both of which will result in INCR. of free light chains in the plasma

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14
Q

Name causes of paraproteinaemia.

A
  1. Myeloma
  2. MGUS (56%)
  3. Amyloidosis (10%)
  4. Lymphoma
  5. aymptomatic myeloma
  6. solitary/ extramedullary plasmocytoma
  7. Chronic lymphocytic leukaemia
  8. Waldenstrom Macroglobulinaemia
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15
Q

What is Bence Jones protein?

A
  • immunoglobulin light chains

- detected by urine electrophoresis

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16
Q

How does myeloma develop?

A

normal plasma cells hit with genetic mutations> MGUS clone (benign) > few more genetic hits> ASYMPTOMATIC MYELOMA (MALIGNANT but no organ damage)
> more hits= MYELOMA

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17
Q

How does myeloma affect the body?

A
  • clonal PLASMA cells
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18
Q

How does myeloma bring about organ damage?

A
  1. Direct tumor cell effects

2. Paraprotein mediated effects

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19
Q

What are exs. of DIRECT tumor cell defect?

A
  • bone lesions
  • incr. CALCIUM
  • bone pain
  • replaced normal bone marrow–> MARROW failure
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20
Q

What are some paraprotein mediated efx?

A
  • renal failure
  • immune supression
  • hyperviscocity
  • amyloid
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21
Q

What kind of bony lesions are seen with multiple myeloma?

A

lesions arise from medullary cavity> erosion of cancellous bone> cortical bone destruction»FRACTURES (usually in vertebral column and femur) – compression fractures

  • punched out defects
  • diffuse SKELETAL demineralization (rise in Ca)
22
Q

How can myeloma be classified?

A
  • by the type of Ab produced (seen in serum/ urine)
  • –60% produce IgG
  • Bence Jones myeloma (15%)
  • —some don’t produce monoclonal proteins at all (Non-secretory myeloma)
23
Q

Why do myelomas cause lytic bone disease?

A
  • flourish of myeloma cells> RISE in IL-6> further rise in IL-6 and TGF-beta
  • the rise in these chemokines> OSTEOCLAST ACTIVATION (and suppression of osteoblasts)

—-BONE RESORPTION! in bone matrix

–RISE in Ca2+

24
Q

What is the risk of hypercalcemia?

A
  • renal stones
  • bone pain (spinal cord impingement is possible on vertebral #)
  • abdominal groans
  • neuro: confusion and lethargy
  • thirst and dehydration
  • renal impairment
25
Q

How common is renal impairment in myeloma pt?

A

30% present with renal impairment at dx

26
Q

Why are light-chains pathological in the kidney with myeloma?

A
  • ligh chain deposition in the DISTAL convoluted tubules and collecting ducts==> CAST nephropathy
  • tubular cell damage (necrosis or atrophy) d.t toxic effect of Bence Jones proteins (light chains)
27
Q

What are other causes renal problems in myeloma?

A
  • sepsis (bacterial pyelonephritis)
  • metastatic calcification of the kidneys (d.t hypercalcemia and bone resorption)
  • use of NSAIDS
  • hyperuricemia
  • amyloidosis —involving glomeruli and vessel walls
28
Q

How to casts form in the nephrons?

A
  • light chains usually meant to be reabsorbed at the PCT
  • when PCT is overwhelmed or damaged; light chains make it to the thick ascending limb where Tamm-Horsfall protein is formed

-this protein combined with free light chains==> insoluble CASTS; which BLOCK the nephron

29
Q

Name the most common ft of multiple myeloma

A
  • renal failure

- cast nephropathy is greatest cause

30
Q

How to manage cast nephropathy>

A
  • reversible with prompt rx
  • HYDRATION; stop nephrotoxic drugs
  • steroids and chemo will SWTICH OFF light chain production
31
Q

What is involved with the rx of myeloma?

A
    • corticosteroids (DEXAMEHTASONE or PREDNISOLONE)
  • alkylating agents (cyclophosphamide/ melphalan)
  • novel agents (thalidomide/ lenalidomibe/ bortezomib)
  • —–in FIT pts: high dose CHEMO and stem cell transplant
32
Q

How to monitor response of myeloma to rx?

A

monitor PARAPROTEIN levels

33
Q

Name novel agents for rx of myeloma.

A

thalidomide/ lenalidomibe/ bortezomib)

34
Q

What could be done to deliver very HIGH dose chemo, relatively safe?

A

AHSCT
(autologous haematopoietic stem cell transplant)
—-deliver v.HIGH dose chemo, safely
—–collect and freeze pts blood stem cells (after inducing stem cell release from the bone marrow) PRIOR to chem
- give chemo
- return blood cells by infusion to vein

35
Q

What is the survival rate for younger pts?

A

5-10 YEARS

- relapse is inevitable

36
Q

How to control symtpoms?

A
  1. opiate analgesia (AVOID NSAIDS)
  2. loacl radiotherapy (pain relief/ spinal cord compression)
  3. biphosphonates (corrects hypercalcemia and BONE pain)
  4. Vertebroplasty- inject sterile cement into FRACTURED bone
37
Q

What is MGUS?

A
  • monoclonal gammopathy of undetermined significance
    `
    —-paraprotein <3g/dL
    —–bone marrow plasma cells <10%
    —–no evidence of myeloma and organ damage!
38
Q

MGUS is commonly seen in whom>

A
  • 5% (greatest %) in their 8th decade
39
Q

What occurs in AL amyloidosis?

A
  • small plasma cell clone
  • –mutation in the LIGHT chain > altered structure
  • —precipitates in tissues as an insoluble beta pleated sheet
40
Q

What is the pathophysiology of AL amyloidosis?

A
  • accumulation (of beta-pleated sheets) in tissues> organ damage
  • slowly progressive
  • multisystem disease
  • —-POOR prog. if cardiomyopathy
41
Q

Rx of AL amyloidosis?

A
  • same as MYELOMA

- chemo to switch of light chain prodn

42
Q

What kind of organ damage occurs with AL amyloid?

A
  1. nephrotic syndrome
  2. cardiomyopathy
  3. organomegaly (LFTS messed up)
  4. autonomic and peripheral neuropathy
  5. malabsorption
43
Q

How to dx AL amyloidosis?

A
  • organ biopsy CONFIRM deposition of AL amyloid —-CONGO RED STAIN
  • -rectal and fat biopsy done; if HIGH clinical suspicion
44
Q

What evidence sought for AL amyloid deposition in other orgasn?

A
  • SAP scan
  • echocardiogram (cardiac MRI)
  • nephrotic range proteinuria
45
Q

What is used in SAP scan for AL amyloid?

A
  • I-123 labelled serum amyloid P used to monitor disease burden and response
  • —SAP localises rapidly and specifically to amyloid deposits —in proportion to the quantity of amyloid present
46
Q

What is Waldenstrom’s Macroglobulinaemia?

A

IgM paraprotein

  • neoplasm of the lymphoplasmacytoid
  • —tumor effects
  • -paraprotein effects
47
Q

What are tumor effects?

A
  • splenomegaly
  • marrow failure
  • lymphadenopathy
48
Q

What are protein effects?

A
  • hyperviscocity

- neuropathy

49
Q

How does hyperviscosity syndrome present as?

A
  • fatigue
  • bleeding
  • visual disturbance
  • confusion
  • coma

B sx: wgt loss/ night sweats

50
Q

Who is at risk of lymphoplasmacytoid?

A

those in 60s-70s

51
Q

What is the rx of Waldenstroms?

A 
chemo
plasmapheresis (removes paraprotein from circulation)
52
Q

How does plasmapheresis help?

A

removes pt plasma rich in IgM paraprotein —–replace with donor plasma

HEMATOLOGY (20 decks)

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