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haematology > Non-malignant Plasma Cell Disorders > Flashcards

Non-malignant Plasma Cell Disorders Flashcards

1
Q

What does MGUS stand for

A

Monoclonal gammopathy of undetermined significance

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2
Q

What is the requirements for a diagnosis of MGUS

A

Paraprotein <30 g/l, bone marrow plasma cells <10% and with no evidence of myeloma end organ damage

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3
Q

What is a complication of MGUS

A

1% risk of progression to myeloma

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4
Q

What is MGUS

A

An isolated finding of monoclonal Paraprotein in the serum or urine that doesn’t fulfil the diagnostic criteria for smouldering or symptomatic myeloma

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5
Q

What is waldenstrom’s macroglobulinaemia

A

Chronic indolent B-cell lymphoproliferative disorder

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6
Q

What does indolent mean

A

Slow progressing

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7
Q

What characterises waldenstrom’s macroglobulinaemia

A

IgM monoclonal Paraprotein
Bone marrow infiltration by lymphoplasmacytic cells

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8
Q

Tumour effects of waldenstrom’s macroglobulinaemia

A

Lymphadenopathy
Splenomegaly
Marrow failure

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9
Q

Paraprotein effects of waldenstrom’s macroglobulinaemia

A

Hyperviscosity
Neuropathy

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10
Q

B symptoms of waldenstrom’s macroglobulinaemia

A

Night sweats
Weight loss

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11
Q

Bone marrow assessment of a patient with waldenstroms macroglobulinaemia

A

Infiltration by LPL

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12
Q

Management of waldenstroms macroglobulinaemia

A

Chemotherapy and plasmapheresis

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13
Q

How does plasmapheresis help in patients with waldenstroms macroglobulinaemia

A

Removes Paraprotein from the circulation so reduces viscosity

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14
Q

What causes AL amyloidosis

A

Extra cellular deposition of fibril-forming monoclonal immunoglobulin light chains

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15
Q

When does AL amyloidosis usually present

A

60s
<10% of patients are under 50

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16
Q

Common presenting symptoms of AL amyloidosis

A

Weakness and dyspnoea

17
Q

What is the most common manifestation of AL amyloidosis

A

Renal involvement
Nephrotic syndrome, proteinuria and impaired renal function

18
Q

Name another common manifestation of AL amyloidosis

A

Restrictive cardiomyopathy

19
Q

What is a diagnostic investigation of AL amyloidosis

A

Organ biopsy

20
Q

Positive result from organ biopsy in AL amyloidosis

A

Congo red stain
Apple green birefringence under polarised light

21
Q

How may we look for evidence of deposition in other organs in AL amyloidosis

A

SAP scan
echo/ cardiac MRI
nephrotic range proteinuria

22
Q

Management of AL amyloidosis

A

Chemotherapy

23
Q

What is the main adverse prognostic factor in AL amyloidosis

A

Presence and severity of amyloid heart disease

haematology (11 decks)

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