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Haematology > Plasma Cell Dyscrasias > Flashcards

Plasma Cell Dyscrasias Flashcards

1
Q

What are the two main functions of a B cell?

A

Antibody production

Antigen presenting cells

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2
Q

What part of immunity are B cells?

A

Active immune system

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3
Q

Where are B cells made?

A

Bone marrow from haematopoietic stem cells

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4
Q

Which immunoglobulin is the largest and what is its shape?

A

IgM

Pentamer

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5
Q

Which immunoglobulins are monomers?

A

IgD
IgE
IgG

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6
Q

Where do B cells go to identify antigens when released from the marrow?

A

Follicle germinal centre of the lymph node

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7
Q

Circulating B cells are referred to as?

A

Memory B cell

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8
Q

B cells which return to the bone marrow are referred to as?

A

Plasma cells

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9
Q

List some of the features of a plasma cell?

A

Blue cytoplasm
Pale perinuclear area
Open chromatin
Eccentric clock face nucleus

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10
Q

What is another name for a monoclonal immunoglobulin?

A

Paraprotein

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11
Q

What are paraproteins an indicator of?

A

Underlying clonal disorder of the B cells e.g. myeloma

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12
Q

How are immunoglobulins detected?

A

Serum electrophoresis

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13
Q

What is used to classify the abnormal protein band?

A

Serum immunofixation

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14
Q

What are Bence Jones proteins?

A

Immunoglobulin light chains

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15
Q

How do we detect Bence Jones proteins

A

Urine electrophoresis

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16
Q

List some of the main causes of paraproteinaemia:

A 
MGUS
Myeloma
Amyloidosis
Lymphoma
Waldenstrom's macroglobulinaemia
17
Q

How does myeloma occur?

A

Normal plasma cells take a ‘genetic hit’ which causes them to form an MGUS clone (premalignant)
Patients may be asymptomatic for a while but then as it progresses symptoms will occur

18
Q

What direct tumor cell effects occur in myeloma?

A

Bone lesions
Increased calcium
Bone pain
Replacement of normal bone marrow leading to marrow failure

19
Q

What effects will the paraproteins released in myeloma cause?

A

Renal failure (cast nephropathy)
Immune suppression
Hyperviscosity
Amyloid

20
Q

How is myeloma classified?

A

By the type of antibody produced e.g. IgG myeloma (most common)

21
Q

How does myeloma cause lytic bone disease?

A

Increase of myeloma cells leads to increased IL-6 which leads to an increased activation of osteoclasts and suppression of osteoblasts

22
Q

What is the median age of diagnosis of myeloma?

A

65 y/o

23
Q

What supportive treatments are given for myeloma?

A 
Analgesia for bone pain (avoid NSAIDs)
Bisphosphonates (reduce fracture risk)
Anaemia treatment (epo / transfusion)
Dialysis 
Vertebroplasty 
Anti-coagulation 
Broad spectrum antibiotics in infections
24
Q

What chemotherapy approach is used in fitter patients with myeloma?

A

VAD
Vincristine Adriamycin & Dexamethasone
Then autologous stem cell transplant

25
Q

What “novel agents” may be added in the treatment of more resistant myeloma?

A

Thalidomide
Bortezomib
Lenalidomide

26
Q

What is the survival for myeloma?

A

5 - 10 years (especially in younger/fitter patients)

Relapse is inevitable

27
Q

What are some of the complications of myeloma?

A

Hypercalcaemia
Spinal cord compression
Hyperviscosity
AKI

28
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance

29
Q

How is MGUS clinically defined?

A

Para-protein levels <30g/l
Bone marrow plasma cells <10%
No evidence of myeloma end organ damage

30
Q

What is amyloidosis?

A

Rare disorder of a small plasma cell clone leading to a mutation in the light chain. This forms a precipitate in tissues as an insoluble beta pleated sheet

31
Q

How is amyloidosis diagnosed?

A

Congo red stain from an organ biopsy

also apple green birefringence under polarized light

32
Q

What is Waldenstrom’s Macroglobulinaemia?

A

Lymphoplasmacytoid neoplasm

Clonal disorder of intermediate cells between a lymphocyte and plasma cell

33
Q

What type of immunoglobulin is characteristic in Waldenstrom’s Macroglobulinaemia?

A

IgM para-protein

34
Q

What are some of the clinical features of Waldenstrom’s Macroglobulinaemia?

A

Hyperviscosity syndrome

B symptoms

35
Q

What are “B symptoms”?

A

Night sweats
Weight loss
Fever

36
Q

What are some features of hyper-viscosity syndrome?

A 
Fatigue
Visual disturbances
Confusion
Coma
Bleeding
Cardiac failure
37
Q

How is Waldenstrom’s treated?

A

Chemotherapy

Plasmapharesis to remove the paraprotein

38
Q

List some symptoms of myeloma:

A 
Backache
Pathological fractures
Recurrent infections
Anaemia / neutropaenia
Moans / groans / bones / stones

Haematology (13 decks)

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