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Leukaemia > Chronic Lymphoproliferative Disorders, Plasma Cell Myeloma And Amyloidosis > Flashcards

Chronic Lymphoproliferative Disorders, Plasma Cell Myeloma And Amyloidosis Flashcards

0
Q

How does one class chronic lymphoproliferative disorders?

A

Are either B or T cell malignancies.

*some are more leukaemic and some are more lymphomatous.

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1
Q

What is the difference between a lymphoma and leukaemia?

A

Lymphoma- solid lymph node mass.

Leukaemia- circulating cells in peripheral blood.

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2
Q

List the types of B Cell chronic leukaemias.

A

Chronic lymphocytic leukaemia (common)
Hairy cell leukaemia
Prolymphocytic leukaemia
Some typical Non-Hodgkin lymphomas may spill into peripheral blood when advanced

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3
Q

Types of chronic T cell leukaemias?

A

T prolymphocytic leukaemia
Large granular lymphocytic leukaemia
Adult T cell leukaemia/lymphoma

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4
Q

Some of the methods used to diagnose chronic leukaemias?

A 
Blood tests - chronic persistent lymphocytosis
Morphology 
Immunophenotype
Cytogenetics
Genetics
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5
Q

Who are most at risk with regards to chronic lymphocytic leukaemia (CLL)?

A

Older age group (60-80)

There is a genetic predisposition

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6
Q

What is the problem in chronic lymphocytic leukaemia (CLL)?

A

Accumulation of monoclonal mature B Lymphocytes in the:

  • blood
  • bone marrow
  • liver
  • spleen
  • lymph nodes
  • is due to reduced apoptosis
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7
Q

Clinical features or chronic lymphocytic leukaemia?

A 
Males affected 2:1
Lymph node enlargement --> non-tender, symmetrical 
Anaemia
Thrombocytopenia
Splenomegaly
Immunosuppression
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8
Q

Which sex is more affected with regards to chronic lymphocytic leukaemia?

A

M:F 2:1

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9
Q

Lab features of chronic lymphocytic leukaemia?

A

Lymphocytosis
Normocytic anaemia/autoimmune haemolysis (spherocytes)
Sometimes neutropenia/thrombocytopenia
Decreased serum Ig

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10
Q

Features of a peripheral blood smear regarding chronic lymphocytic leukaemia?

A

WCC - lymphocytosis
Small round lymphocytes
Smear cells (due to fragile B cells)

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11
Q

Which staging system is used to stage CLL?

Which end of the scale indicates a poorer prognosis?

A

Rai staging system.
Rai 0 or 1 - good prognosis.
2-4 associated with more advanced disease.

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12
Q

How to treat CLL?

A

Will partially respond to chemo but more about managing symptoms:

  • treat problamatic organomegaly
  • bone marrow suppression(anaemia, thrombocytopenia, neutropenia)
  • haemolytic episodes
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13
Q

Ehat are the aims of treatment with regards to CLL?

A

Support not cure (cure is rare)

Control symptoms and limit extent of leucocytosis (using chemo).

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14
Q

What group of people does one see Hairy Cell Leukaemia in?

A

More in males of older age (40-60yo).

Is rare!

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15
Q

How do people with Hairy Cell Leukaemia present?

A

Severe infectons.
Anaemia.
Splenomegaly.

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16
Q

What does hairy cell leukaemia NOT present with?

A

Lymphadenopathy.

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17
Q

Why is it called hairy cell leukaemia?

A

Affected B cells appear ‘hairy’ beneath a microscope due to radial projections from their surface.

18
Q

Where do abnormal cells accumulate in hairy cell leukaemia?

A

Bone marrow–> anemia, thrombocytopenia, infections

19
Q

How does one diagnose hairy cell leukaemia?

A

Peripheral blood morphology.
Flow cytometry.
Bone marrow biopsy.

20
Q

Prognosis of hairy cell leukaemia?

A

May respond well to certain chemotherapies.

21
Q

Characteristics of B cell prolymphocytic leukaemia?

A

Very rare.
Aggressive.
Poor prognosis.

22
Q

Some characteristics of T prolymphocytic leukaemia?

A

Very high WCC.
Skin invlovement.
Serous effusions.

23
Q

Some characteristics of large granular lymphocyte leukaemia?

A

Cytopenias - anaemia, neutropenia

Associated with rheumatoid arthritis.

24
Q

Characteristics of adult T-cell leukaemia?

A 
Commonly seen in Caribbean and Japan.
Associated with HTLV1 virus associaton.
Signs+symptoms:
- skin lesions
- hepatosplenomegaly
- lymphadenopathy
- hypercalcaemia
25
Q

What is multiple myeloma (plasma cell myeloma)?

A

A neoplasm of plasma cells that infiltrate the bone marrow and disrupt regular function of the bone marrow. Tissue damage may be a feature.

26
Q

Group of people in which MM is mostly seen?

A

Develop after 40y.o.a.

Peak in the 60’s

27
Q

What is thought to be a key factor in the development of MM?

A

Overexpression of cyclin D.

28
Q

What may be a common complaint that is associated with MM?

A

An elderly patient who complains about persistent bone pain/back pain.

29
Q

What are the clinical features of MM?

A

Bone pain - RANKL activation–> stimulates osteoclast activity (bone lesions).
Anaemia - lethargu, weakness, pallor, dyspnoea, tachycardia.
Recurrent infections- neutropenia, lack of normal Ig.
Renal- failure (calculi due to hypercalcaemia, Ig’s), polyuria, polydipsia, anorexia.
Bleeding- paraprotein interferes with platelets and coagulation factors.
Amyloidosis.
Hyperviscosity- haemorrhage, loss of vision, CNS sx, neuropathies, cardiac failure.

30
Q

Diagnosis of MM?

A

Serum and urine protein electrophoresis looking for monoclonal protein.
Increased clonal plasma cells in bone marrow.
Increased serum calcium.
Cytogenetics and FISH

31
Q

Pneumonic for MM symptoms?

A

C - hypercalcaemia
R - renal impairment
A - anaemia
B - bone involvement

32
Q

What is seen in a trephine biopsy in MM?

A

Diffuse infiltrate of plasma cells.

33
Q

Immunophenotyping of MM?

A

Monoclonal Ab staining. (Light chain)

34
Q

Other investigations to diagnose MM?

A

Presence of paraproteins (mostly IgG).
Urine BJP - bence jones protein (monoclonal globulin protein)
Anaemia (normochromic/normocytic)
Neutropenia/thrombocytopenia
High ESR and rouleaux formation
Skeletal survey - osteolytic lesions or osteoporosis.

35
Q

Management (supportive) of MM?

A

Renal: rehydrate, management of hypercalcaemia and hyperuricaemia
Bone disease: biphosphonates
Anaemia and infections: transfusions, antibiotics, Ig, antifungals
Bleeding: plasmapheresis

36
Q

Management(specific) of MM?

A

Intensive - younger patients- intensive chemo

Non-intensive: oral chemo, steroids, thalidomide

37
Q

Prognosis of MM?

A

Depends on genetics and disease burden.
A few years with non-intense chemo.
Variable.

38
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance.
Is a precursor of myeloma.
No clinical complications at this stage.

39
Q

What is plasma cell leukaemia?

A

When large amounts of circulating plasma cells.

Rare + poor prognosis.

40
Q

What is amyloidosis?

A

A group of disorders –> deposition of protein in fibrillar form extracellularly.
*disrupts organ/tissue function.

41
Q

Amyloidosis: inheritance? Pattern of spread?

A

Is congenital or acquired.

Focal or systemic.

42
Q

Systemic effects of amyloidosis?

A

Heart - failure
Nerves - neuropathy, carpal tunnel
Kidneys - failure
Tongue - macroglossia

Leukaemia (4 decks)

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