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Haematology > Myeloma and Plasma Cell Disorders > Flashcards

Myeloma and Plasma Cell Disorders Flashcards

1
Q

Where are B cells derived from?

A

Derived in bone marrow from pluripotent haemopoietic stem cells

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2
Q

What are the functions of B cells?

A

Antibody production, act as antigen presenting cell

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3
Q

What are immunoglobulins?

A

Antibodies produced by B cells and plasma cells = made of 2 heavy and 2 light chains

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4
Q

What influences B cells?

A

The microenvironment

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5
Q

Where is the Ig variable element of B cells generated from?

A

The VDJ region recombination = occurs early in development

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6
Q

What happens once self reactive cells are removed during B cell development?

A

Immature B cells with Ig on their surface exit the bone marrow

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7
Q

What do B cells do once they are released into the periphery?

A

Travel to follicle germinal centre of lymph node

Identify antigen and improve fit by somatic mutation or be deleted

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8
Q

What is the fate of B cells once they have identified an antigen?

A

May return to marrow as plasma cell or circulate as memory B cell

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9
Q

What is the function of plasma cells?

A

Pump out antibody

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10
Q

What are the histological features of plasma cells?

A

Clock face nucleus on H & E
Open chromatin = synthesise mRNA
Plentiful blue cytoplasm full of protein and pale perinuclear area

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11
Q

How does polyclonal Ig arise?

A

Ig produced by many different plasma cell clones

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12
Q

What causes polyclonal increase in Ig?

A

Reactive = infection, autoimmune, malignancy, liver disease

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13
Q

How does monoclonal Ig arise?

A

All derived from clonal expansion of a single B cell = identical antibody structure and specificity

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14
Q

What is the other name for monoclonal Ig?

A

Paraprotein = marker of underlying B cell disorder

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15
Q

What is used to detect Ig?

A

Serum electrophoresis = proteins move at different rates determined by size and charge, detects abnormal protein band

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16
Q

What is serum immunofixation used for?

A

Classifying abnormal protein band

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17
Q

What is Bence Jones protein?

A

Ig light chains = excess produced by plasma cells, leaks into urine and detected by urine electrophoresis

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18
Q

What are the causes of paraproteinaemia?

A

MGUS, myeloma, amyloidosis, lymphoma, plasmacytoma, chronic lymphocytic leukaemia, Waldenstrom’s macroglobinaemia

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19
Q

What is the progression of cells to myeloma?

A

Normal plasma cell - MGUS clone (premalignant) - asymptomatic myeloma (malignant but no organ damage) - myeloma

20
Q

What are the features of myeloma?

A

Bone lesion, increased calcium, bone pain, marrow failure, amyloid, immune suppression, hyperviscosity, renal failure

21
Q

How is myeloma classified?

A

By the type of antibody produced = IgG (59%), IgA (21%), Bence Jones (15%)

22
Q

What are the symptoms of hypercalcaemia?

A

Stones, bone pain, abdominal pain, psychiatric issues, thirst, dehydration, renal impairment

23
Q

How common is renal failure in myeloma?

A

30% have renal impairment at diagnosis = tubular cell damage by light chain deposition occurs causing cast nephropathy

24
Q

How is cast nephropathy treated?

A

Damage may be reversible with prompt treatment = hydration, stop nephrotoxic drugs, steroids/chemo to stop light chain production

25
Q

What is the mainstay of myeloma treatment?

A

Combination chemotherapy

High dose chemo or stem cell transplant used in fit patients

26
Q

What are some chemotherapy agents used to treat myeloma?

A

Corticosteroids, dexamethasone or prednisolone
Alkylating agents = cyclophosphamide
Novel agents = thalidomide, bortezomib, lenalidomide

27
Q

What is used to monitor myeloma treatment response?

A

Paraprotein levels

28
Q

What is used for symptomatic control in myeloma?

A 
Opiate analgesia (avoid NSAIDs)
Local radiotherapy = pain relief, spinal cord compression
Bisphosphonates = corrects hypercalcaemia 
Vertebroplasty
29
Q

What is the purpose of a vertebroplasty?

A

Stabilises fractured bone = sterile cement injected into bone

30
Q

How is MGUS defined?

A

Paraprotein <30g/l and bone marrow plasma cell <10%

No evidence of myeloma end organ damage

31
Q

What does MGUS stand for?

A

Monoclonal gammopathy of undetermined significance

32
Q

What test results would you expect in MGUS?

A

Normal Hb, normal Ca2+, normal renal function, no lytic lesion, no increase in infection

33
Q

What causes AL amyloidosis?

A

Small plasma cell clone with mutation in light chain = altered structure

34
Q

How does AL amyloidosis appear in tissues?

A

Precipitates as beta pleated sheets = causes slowly progressive organ damage

35
Q

What feature conveys poor prognosis of AL amyloidosis?

A

Cardiac amyloid

36
Q

What organ damage may occur due to AL amyloidosis?

A

Nephrotic syndrome, cardiomyopathy, hepatomegaly, neuropathy, malabsorption

37
Q

How is AL amyloidosis treated?

A

Chemotherapy

38
Q

How is AL amyloidosis diagnosed?

A

Organ biopsy for amyloid deposition

SAP scan, echocardiogram, cardiac MRI

39
Q

How is amyloid identified on biopsy?

A

Use congo red stain = causes apple green birefringence (may do fat or rectal biopsy)

40
Q

What is the purpose of SAP scans in AL amyloidosis?

A

Used to monitor disease burden = Indium 23-labelled SAP localises rapidly to amyloid deposits

41
Q

What paraprotein is associated with Waldenstrom’s microglobinaemia?

A

IgM paraprotein = pentameric structure

42
Q

What is Waldenstrom’s microglobinaemia?

A

Lymphoplasmacytoid neoplasm = clonal disorder of cells intermediate between a lymphocyte and plasma cell

43
Q

What are the features of Waldenstrom’s microglobinaemia?

A

Lymphadenopathy, splenomegaly, marrow failure, hyperviscosity, neuropathy

44
Q

What kind of syndrome does Waldenstrom’s microglobinaemia cause?

A

Hyperviscosity syndrome = fatigue, visual disturbance, confusion, coma, bleeding, cardiac failure

45
Q

How is Waldenstrom’s microglobinaemia treated?

A

Chemotherapy and plasmapheresis

Haematology (24 decks)

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