Myeloma and other plasma cell disorders Flashcards Preview

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Flashcards in Myeloma and other plasma cell disorders Deck (11)
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1
Q

What is MGUS and how is it diagnosed ?

A

Sometimes, small clones of plasma cells develop and produce a low amount of paraprotein but you have no other features of myeloma. This is called monoclonal gammopathy of unknown significance (MGUS).

Diagnosed by exlusion

2
Q

What are the test results you need to diagnose MGUS by exlcusion ?

A

Paraprotein <30g/l

Bone marrow faulty plasma cells <10% (would be greater than 10% in the bone marrow in myeloma)

No evidence of myeloma end organ damage;

  • Normal calcium
  • Normal renal function
  • Normal Hb
  • No lytic lesions
  • No increase in infections
3
Q

What is Amyloid light chain amyloidosis ?

A
  • AL amyloidosis is caused by an abnormality in certain cells found in the bone marrow, called plasma cells.
  • The abnormal plasma cells produce abnormal forms of light chain proteins, which enter the bloodstream and can form amyloid deposits.
4
Q

What do the amyloid deposits result in ?

A

Depends on the organs affected

  • Kidney - Nephrotic syndrome
  • Heart - Cardiomyopathy
  • Liver - Organomegaly deranged LFT’s
  • Neuropathy - Autonomic, Peripheral
  • GI tract - Malabsorption
5
Q

What are the main symptoms which can present in amyloidosis?

A

Nephrotic symptoms:

  • Severe swelling (edema), particularly around your eyes and in your ankles and feet
  • Foamy urine, which may be caused by excess protein in your urine
  • Weight gain due to excess fluid retention
  • Fatigue
  • Loss of appetite

Cardiomyopathy:

  • shortness of breath
  • oedema
  • an abnormal heartbeat (arrhythmia)
6
Q

How is AL amyloidosis diagnosed ?

A

Organ biopsy confirming AL amyloid deposition - Use Congo red stain which shows “Apple-green” birefringence under polarised light

7
Q

How is the extent of AL amyloidosis staged ?

A
  • SAP scan
  • Echocardiogram
  • Heavy proteinuria
8
Q

What is Waldenstrom’s Macroglobulinaemia (IgM paraprotein)?

A
  • It is an uncommon condition seen in older men.
  • It is a lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein
9
Q

What are the clinical features of Waldenstrom’s Macroglobulinaemia (IgM paraprotein)?

A
  • Monoclonal IgM paraproteinaemia
  • B symptoms - weight loss, lethargy, night sweats
  • Hyperviscosity syndrome e.g. visual disturbance, confusion, coma, cardiac failure
  • Hepatosplenomegaly
  • Lymphadenopathy
  • Cryoglobulinaemia e.g. Raynaud’s
10
Q

What is the treatment of WM?

A
  1. Chemotherapy and
  2. Plasmapheresis (removes paraprotein from the circulation)
11
Q

Why is hyperviscocity such a problem in WM?

A

Because there is lots of circulating IgM and it is a pentamer so a big molecule so hence hyperviscocity is more of a problem in this condition