Myeloma and Other Plasma Cell Disorders Flashcards

1
Q

what are B cells derived from

A

pluripotent haematopoietic stem cells in the bone marrow

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2
Q

what is the roles of B cells

A

part of the adaptive immune system

  • antibody production
  • antigen presenting cells
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3
Q

what are immunoglobulins

A

Antibodies produced by B-cells and plasma cells

Proteins made up of 2 heavy and 2 light chains

Each antibody recognizes a specific antigen

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4
Q

what are the monomer shaped immunoglobulins

A

IgD
IgE
IgG

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5
Q

what are the dimer shaped immunoglobulins

A

IgA

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6
Q

what is the pentamer shaped immunoglobulin

A

IgM

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7
Q

what is the normal pathway of lymphocyte maturation

A

stem cell&raquo_space; lymphoid progenitors&raquo_space; Pro B cells (IgM)&raquo_space; Pre B cells (IgM & IgD)&raquo_space; IgM B cells

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8
Q

what do IgM B cells divided to make normally

A

IgM plasma cells
IgA
IgE
IgG

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9
Q

what does IgG eventually go on to become

A

plasma cells

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10
Q

what is the function of a plasma cell

A

to produce large quantities of antibodies when needed

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11
Q

how does the nucleus appear in a plasma cell

A

“clock face nucleus”

nucleus off to one side, can look like a fried egg

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12
Q

what is meant when a blood film is described as polyclonal or monoclonal

A

polyclonal - multiple types of blood cells seen; can be seen as part of normal function

monoclonal - one type of cell seen overwhelming; suggests pathology

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13
Q

what are the causes of a polyclonal increase in immunoglobulins

A

infection
autoimmune
malignancy
liver disease

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14
Q

what does a monoclonal increase in immunoglobulins imply

A

implies that the antibody is all identical and produced by a single B cell

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15
Q

what is a monoclonal immunoglobulin also called and what is it a marker of

A

paraprotein

underlying clonal B-cell disorder

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16
Q

how can you detect immunoglobulins

A

serum electrophoresis

17
Q

what area is immunoglobulins seen in

A

gamma region

18
Q

what is bence-jones protein (BJP)

A

excess immunoglobulin light chains leaking into the urine
» paraproteinaemia

detected by urine electrophoresis

19
Q

what are causes of paraproteinaemia

A

1st - MGUS (completely harmless)
2st - myeloma
3rd - Amyloidosis
4th - lymphoma

  • asymptomatic myeloma
  • chronic lymphocytic leukaemia
20
Q

essentially what is myeloma

A

clone of a malignant plasma cell

21
Q

what are direct tumour effects of myeloma

A

bone lesions
increased calcium
bone pain
bone marrow failure

22
Q

what are the effects of paraprotein seen in myeloma

A

renal failure
immune suppression
hyperviscosity
amyloid

23
Q

how is myeloma classified

A

by type of antibody produced

24
Q

what are the most to least common type of myeloma

A

Most to least:

  • IgG
  • IgA
  • Bence Jones Myeloma
  • Nonsecretory
  • IgD
  • Biclonal
  • IgE
25
Q

what is myeloma’s affect on bone and what is commonly seen on x-ray

A

lytic bone disease

“punched-out lesions”
Pepper pot skull

26
Q

what are Sx of hypercalcaemia

A
Stones
Bones
Abdominal groans
Psychiatric moans
Thirst
Dehydration
Renal impairment
27
Q

how can myeloma cause kidney failure

A

free light chains first accumulate in the proximal tubules before entering the loop of Henle.

Here they can combined with Tamm-Horsfall protein to produce insoluble casts blocking the nephron i.e. cast nephropathy

This can lead to renal failure

28
Q

what is the Tx of myeloma

A

Chemotherapy with +/- prednisolone +/- Thalidomide or bortezomib or lenalidomide

If asymptomatic may just observe

29
Q

how is response to Tx monitored in myeloma

A

paraprotein

30
Q

what is symptomatic control of myeloma

A

opiate analgesia
local radiotherapy
biphosphonates - corrects hypercalcaemia
vertebroplasty - stabilises fractured bone

31
Q

what is MGUS

A

Monoclonal Gammopathy of Uncertain Significance

32
Q

what is the definition of MGUS

A

Paraprotein <30g/l
Bone marrow plasma cells <10%

No evidence of myeloma end organ damage;

  • Normal calcium
  • Normal renal function
  • Normal Hb
  • No lytic lesions
  • No increase in infections
33
Q

what is amyloidosis

A

accumulation of protein in abnormal fibrillar form
- in tissues can cause organ damage

caused by mutations in the light chain

34
Q

what can amyloidosis cause

A

nephrotic syndrome

35
Q

how is amyloid deposition confirmed

A

congo red staining

- causes “Apple-green” birefringence under polarised light

36
Q

what is Waldenstrom’s Macroglobulinaemia

A

Clonal disorder of cells intermediate between a lymphocyte and a plasma cell

Characteristic IgM paraprotein

37
Q

what are the Sx of Waldenstrom’s Macroglobulinaemia

A
lymphadenopathy 
splenomegaly
marrow failure 
hyperviscosity 
neuropathy
38
Q

what are features of hyper viscosity syndrome

A
Fatigue, visual disturbance, confusion, coma
Bleeding
Cardiac failure
Night sweats 
Weight loss
39
Q

Tx of WM

A
chemotherapy 
plasmapheresis (removes paraprotein from the circulation)