Myeloma Flashcards

1
Q

What is the role of B cells?

A

adaptive immune system:
antibody production
antigen presenting cells

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2
Q

What is the Ig region of the B cell derived from

A

V-D-J region recombination

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3
Q

When immature B cells with Ig on their surface exit the bone marrow, where do they go?

A

follicle germinal center of the lymph node

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4
Q

When B cells return to the bone marrow from the follicle germinal center of the lymph node, what are they?

A

plasma cell

memory B cell

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5
Q

What are the features of plasma cells?

A

open chromatin for synthesising mRNA
blue cytoplasm
pale perinuclear area

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6
Q

What are causes of polyclonal increases in Igs?

A

infection
autoimmune
malignancy
liver disease

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7
Q

What are causes of monoclonal increases in Igs?

A

paraprotein

B cell disorder

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8
Q

How are immunoglobulins detected?

A

electrophoresis
albumin = +ve
gamma = -ve

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9
Q

What are Bence Jones protiens?

A

monoclonal Ig free light chain found in the urine

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10
Q

What causes Bence Jones proteins?

A

monoclonal increase in the amount of free light chains in proportion to heavy chains in the plasma meaning that they are secreted

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11
Q

How are Bence Jones proteins detected?

A

urine electrophoresis

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12
Q

What is paraproteinaemia?

A

proliferation of immunoglobulin producing cells

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13
Q

What is the presentation of Myeloma?

A
bone lesions
increased calcium - stones, bones, groans, psychiatric moans
bone pain - increased osteoclast, decreased osteoblast activity
bone marrow failure
paraprotein mediated affects:
renal failure
immunosuppression
amyloid hyperviscosity
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14
Q

How is myeloma classified?

A

type of antibody produced

most common - IgG, IgA, Bence Jones

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15
Q

How does myeloma cause kidney impairement?

A

light chains cause tubular cell damage - cast nephropathy

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16
Q

How is response to myeloma treatment measured?

A

paraprotein

17
Q

What is the median age at diagnosis of myeloma?

A

65

18
Q

How are fit patients with myeloma treated?

A

high dose chemo

autologous HSC transplant

19
Q

What is Monoclonal gammopathy of undetermined significance (MGUS)?

A

paraprotein <30g/L

bone marrow plasma cells <10%

20
Q

What is AL amyloidosis?

A

mutation in light chain leading to altered structure

precipitates in tissues as insoluble beta pleated sheets and causes organ damage (kidneys, heart, liver)

21
Q

How is AL amyloidosis treated?

A

chemo

22
Q

How is AL amyloidosis diagnosed?

A

organ biopsy - stains congo red (apple green bifringent under polarised light)
SAP scan - identifies amyloid deposits
ECHO
nephrotic range proteinuria

23
Q

What is Waldenstoms macroglobulinaemia?

A

IgM paraprotein

lymphoplasmacytoid neoplasm - clonal disorder of cells intermediate between a lymphocyte and plasma

24
Q

How does Waldenstoms macroglobulinaemia present?

A

hyperviscosity - bleeding, cardiac failure, fatigue, visual disturbance, coma
B symtoms - night sweats, weight loss

25
Q

How is Waldenstoms macroglobulinaemia treated?

A

chemo

plasmapheresis