24. Plasma Cell Proliferation Flashcards

1
Q

What are plasma cell dyscrasias?

A

Neoplastic proliferation of plasma cells (ie. mature B cells) that secrete monoclonal immunoglobulins

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2
Q

What is the structure of an immunoglobulin?

A

2 heavy and 2 light chains

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3
Q

Classify plasma cell dyscrasias

A
Multiple myeloma
Smoldering multiple myeloma
Solitary myeloma
Monoclonal gammopathy of undetermined significance
Waldenstrom's macroglobulinaemia
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4
Q

What is multiple myeloma?

A

Multifocal bone marrow disease

Malignant proliferation of plasma cells and skeletal destruction

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5
Q

What immunoglobulins are usually produced in multiple myeloma?

A

IgG and IgA

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6
Q

What is the name of the light chain protein excreted in urine in multiple myeloma?

A

Bence Jones protein

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7
Q

What is the appearance of plasma cells?

A

Eccentric nucleus

‘Clock face’ appearance

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8
Q

What are the possible causes of multiple myeloma?

A

Irradiation
Asbestos
Herpes virus 8
Genetics

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9
Q

What results from the cytokines produced by myeloma cells?

A

Growth of myeloma cells
Increase osteoclast activation
Eg. IL6

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10
Q

How is multiple myeloma diagnosed?

A

Radiology
Bone marrow examination
Serum and urine electrophoresis
Ig levels

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11
Q

How do skeletal lesions appear in multiple myeloma?

A

‘soap bubble’
In axial skeleton
Multifocal

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12
Q

What features are found in bone marrow examination in multiple myeloma?

A

Increased plasma cells
-Atypical and blasts
Russel bodies, Mott cells and Dutcher bodies
Only one type of Ig and light chain on IHC

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13
Q

What is a Russel body?

A

Cell with Ig within cytoplasm

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14
Q

What is a Mott cell?

A

Lots of Ig in cytoplasm

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15
Q

What is a Dutcher body?

A

Ig in nucleus

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16
Q

What finding is present in serum analysis of multiple myeloma?

A

Monoclonal globulin spike on electrophoresis

17
Q

What finding is present in urine electrophoresis of multiple myeloma?

A

Proteinuria with Bence Jones protein

18
Q

What are the clinical features of multiple myeloma?

A
CRAB
Hypercalcaemia
Renal failure
Anaemia
Bone lesions and bleeding
19
Q

What 3 factors cause anaemia in MM?

A

bone marrow is replaced by tumour
Reduced RBC production due to cytokines
Reduced erythropoietin production

20
Q

What is the most common cause of death for MM?

A

Sepsis

-reduction in normal Ig and leukocytes

21
Q

What is the second most common cause of death for MM?

A

Kidney failure

22
Q

What are the causes of kidney failure in MM?

A
Myeloma kidney
AL type amyloidosis
Light chain nephropathy
Increased calcium and uric acid
Pyelonephritis
23
Q

What is the difference between myeloma kidney and light chain nephropathy?

A

Myeloma kidney is due to Bence Jones protein in epithelial cells
Light chain nephropathy takes place in the glomerulus

24
Q

What is a Rouleaux formation?

A

Ig reduces the charge on RBCs so they stick together

25
Q

What is the treatment for multiple myeloma?

A

Immunomodulatory and proteasome inhibitor therapies
Bisphosphonates
Bone marrow transplant

26
Q

What are the symptoms of smoldering MM?

A

None

27
Q

What is solitary plasmacytoma?

A

Solitary lesions on bone or soft tissue

28
Q

What is monoclonal gammopathy of undetermined significance?

A

Early, benign stage of MM

29
Q

What age group is lymphoplasmacytic lymphoma most common in?

A

Elderly

30
Q

Why is lymphoplasmacytic lymphoma also known as?

A

Waldenstrom’s macroglobulinaemia

Produces IgM which is the biggest Ig

31
Q

What are the symptoms of Waldenstrom’s lymphoma?

A
Weakness and weight loss
Lymphadenopathy
Hepatosplenomegaly
M spike
Autoimmune haemolytic anaemia
Hyperviscosity syndrome
32
Q

What are the symptoms of hyperviscosity syndrome?

A

Visual impairment
CNS symptoms
Bleeding
Cryoglobulinaemia

33
Q

What causes visual impairment in hyperviscosity syndrome?

A

Distension and haemorrhage of retinal veins

34
Q

Why is there bleeding in hyperviscosity syndrome?

A

IgM binds to clotting factors and interfere with platelet function

35
Q

What is cryoglobulinaemia?

A

In cold macroglobulins stick together

Known as Raynaud’s phenomenon