Introduction To Lymphoma And Myelanoma Flashcards

1
Q

Define lymphoma

A

A cancer of the white blood cells (lymphocytes)

Affects mature blood cells, mostly B lymphocytes but also T lymphocytes

Heterogeneous group

Many known to be due ti specific genetic mutations and chromosomal translocations

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2
Q

What are the main functions of the lymphatic system?

A

Blood filtration/purification

Removal of excess fluids from tissues

Absorption and transport of lipids

Immune system activation

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3
Q

What are primary lymph organs?

A

Sites where stem cells can divide and become immunocompetent

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4
Q

What are secondary lymph organs?

A

Sites where most of the immune responses occur

Eg. Bone marrow and thymus

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5
Q

How do lymphomas develop?

A
  1. Mutation occurs in lymphocytes in different maturation stages
  2. Uncontrolled division
  3. Organ size increase:
    Lymph node (adenopathy)
    Other lymph organs (splenomegaly)
  4. Spread to other tissues through lymphatic system
  5. Might infiltrate in bone marrow and/or other organs
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6
Q

How are lymphomas classified?

A

Non Hodgkins (more common)

Hodgkins

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7
Q

What are the warning signs of lymphoma?

A

Fever

Swelling of the face and neck

Lump in your neck, armpits or groin

Excessive sweating at night

Unexpected weight loss

Loss of appetite

Feeling of weakness

Breathlessness

Itchiness

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8
Q

What can be done to confirm the presence of a lymphoma?

A

Lymph node biopsy

Then :
- flow cytometry
- FISH
- NGS
- immunophenotyping

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9
Q

What is the staging in PET of lymphomas?

A

Stage 1 - localised disease (single lymph node or organ)

Stage 2 - two or more lymph node regions on the same side of the diaphragm

Stage 3 - two or more lymph node regions above and below the diaphragm

Stage 4 - widespread disease (multiple organs or without lymph node involvement)

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10
Q

What are the causes of lymphomas?

A

Multifactorial disorder:
- malfunctioning of the body’s immune system
- exposure to certain infections

Triggers are unknown - what causes the mutations in the DNA?

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11
Q

What is Hodgkin lymphoma?

A

Presentation: non-painful enlarged lymph node

Risk factors:
- 50% of cases due to Epstein Barr virus (EBV) - classic form
- HIV
- Family history

Diagnosis: excisional lymph node biopsy and Hodgkin cells (Reed sternberg cell) are seen.

Treatment: chemotherapy +/- radiotherapy. Stem cell transplant.

Prognosis: 5 year survival (50-60% depending on age and histology), especially good results in young adults

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12
Q

What is non Hodgkin’s lymphoma?

A

Presentation: enlarged lymph nodes + general lymphoma symptoms

Causes: chromosome translocations

Risk factors: virus infection

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13
Q

Describe how translocations can cause non Hodgkin lymphoma (FOLLICULAR LYMPHOMA)

A

Lymphomas carry chromosome translocations involving ig heavy chain or light chain loci

Ig genes are highly expressed in B-cells

Each Ig gene has a powerful tissue specific enhancer (high expression levels)

Translocation between chromosome 14 and 18

On chromosome 18 BCL2 is present

BCL2 is a apoptosis inhibitor and becomes over expressed after translocation

High rates of proliferation and survival

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14
Q

Describe how translocations can cause non Hodgkin lymphoma (BURKITTS LYMPHOMA)

A

Chromosome 8 and 14

C-myc is a potent proto-oncogene present on chromosome 8

After translocation it becomes a oncogene

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15
Q

How can viral infections be risk factors for non Hodgkin lymphoma?

A

It directly transforms B-lymphocytes in culture due to viral oncogene LMP-1

over half of all normal individuals carry latent EBV infection but do not develop lymphoma due to immune surveillance by cytotoxic T cells.

Highly immunosuppressive individuals the EBV is no longer eliminated by cytotoxic T cells

Develop high grade lymphoma

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16
Q

What is classifies as LOW GRADE non Hodgkin lymphoma?

A

Normal tissue architecture partially preserved - normal cell of origin recognised

Divide slowly

May be present for many months before diagnosis

Behave in an indolent fashion

17
Q

What classifies as HIGH grade non Hodgkin lymphoma?

A

Loss of normal tissue architecture- normal cell of origin hard to determine

Divide rapidly

Present for a matter of weeks before diagnosis

May be life threatening

18
Q

What is diagnosis of non Hodgkin’s lymphoma?

A

Immunophenotyping

Cytogenetics - FISH (for chromosome translocation)

Light chain restriction

PCR - for clonal ig rearrangement

19
Q

How is non Hodgkin’s lymphoma treated?

A

Chemotherapy

Radiotherapy

Stem cell therapy

Monoclonal Ab therapy - Rituximab (anti-CD20)

20
Q

What is the prognosis non Hodgkin’s lymphoma?

A

Overall five year survival rate ~ 70%

21
Q

What is multiple myeloma?

A

Tumour of the bone marrow that involves plasma cells (antibodies production)

22
Q

How is multiple myeloma presented in patients?

A

Absence on initial symptoms

Later:
- bone pain
- bleeding
- frequent infections
- anaemia

23
Q

What are the risk factors of multiple myeloma?

A

Obesity

Radiation exposure

Family history

Certain chemicals

24
Q

What is a paraprotein?

A

Monoclonal immunoglobulin which a light chain

Can be detected in blood or urine

Characterised by homogenous migration

25
Q

What are the three aspects of myeloma that give rise to different clinical features?

A
  1. Suppression of normal bone marrow, blood cell and immune cell function
  2. Bone resorption and release of calcium
  3. Pathological effects of the paraprotein - single monoclonal ig in the serum - high levels - malignancy)
26
Q

What clinical features in multiple myeloma arise due to suppression of normal bone marrow, blood and immune cell function?

A

Anaemia

Recurrent infections

Bleeding tendency

27
Q

What clinical features in multiple myeloma arise due to bone resorption and release of calcium?

A
  1. Myeloma cells produce cytokines
  2. Bone marrow stromal cells to release the cytokine RANKL
  3. Osteoclasts activation

(Lyric lesions of bone, bone pain, fractures)

Calcium released from bone causes hypercalcaemia (multiple symptoms including mental disturbance)

28
Q

What clinical features in multiple myeloma arise due to the pathological effects of the paraprotein?

A

Precipitates in kidney tubules cause renal failure

Deposited as amyloid in many tissues

2% of cases develop hyper viscosity syndrome (increased viscosity of blood)
- stroke
- heart faliure

29
Q

How is multiple myeloma diagnosed?

A

Serum electrophoresis for paraprotein

Urine electrophoresis

Bone marrow biopsy for increased level of plasma cells

Erythrocyte sedimentation rate (ESR) - high due to stacking of the RBC

Flow Cytometry and cytogenetics to detect cause

Radiological investigation of skeleton for lyric lesions

30
Q

What is the treatment for multiple myeloma?

A

Radiotherapy

Chemotherapy combinations

Targeted therapies

Immunotherapy

Allogenic haematopoietic stem cell transplant in young patients (ASCT)

31
Q

What is the prognosis of multiple myeloma?

A

Chemo + ASCT overall 5 year survival rate ~ 35%