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Haematology - Path > Lymphoma 2 > Flashcards

Lymphoma 2 Flashcards

1
Q

What do Reed-Sternberg cells on a blood film indicate?

A

Classical Hodgkin lymphoma

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2
Q
  1. What is Non-Hodgkin lymphoma?
  2. Describe the epidemiology of Non-Hodgkin Lymphoma
A
  1. Neoplastic proliferation of lymphoid cells

2.

  • Originates in lymphoid tissue (lymph nodes, bone marrow, spleen)
  • Incidence rising 200/million/population/year
  • Range
    • Burkitt’s lymphoma is the fastest growing human cancer
    • Indolent diseases with a possible 25 year survival
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3
Q

What is the presentation of Non-Hodgkin lymphoma?

A
  • Painless lymphadenopathy
  • Compression symptoms
  • B symptoms
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4
Q

Describe the stages of managing Non-Hodgkin lymphoma

A
  • Stage the disease
    • CT Scan
    • PET scan
    • BM biopsy
    • Lumbar puncture
  • Prognostic markers and important tests
    • LDH
    • Performance status
    • HIV serology
    • Hepatitis B serology (risk of reactivation if B cell depleting therapy given)
  • Plan therapy
    • Urgent chemotherapy
    • Monitor only
    • Antibiotic eradication (H.pylori gastric MALT lymphoma)
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5
Q

What are the main common subtypes of Non-hodgkin lymphoma?

A
  • Diffuse large B-cell lymphoma (30%)
  • Follicular lymphoma (22%)
  • NK/cell/T cell lymphomas (10%)
  • AND Marginal zone lymphoma (10%)
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6
Q

Describe whether the following are high grade of low grade lymphomas

  1. Burkitt lymphoma
  2. T or B cell lymphoblastic leukemia/lymphoma
  3. Diffuse large B cell
  4. Mantle cell
  5. Follicular
  6. Small lymphocytic/CLL
  7. Mucosa associated (MALT)
A
  1. Burkitt lymphoma - Very aggressive, high grade
  2. T or B cell lymphoblastic leukemia/lymphoma - very aggressive - high grade
  3. Diffuse large B cell - aggressive - high grade
  4. Mantle cell - aggressive - high grade
  5. Follicular - indolent - low grade
  6. Small lymphocytic/CLL - indolent - high grade
  7. Mucosa associated (MALT) - indolent - low grade
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7
Q

Describe diffuse large B cell lymphoma

A
  • Aggressive B cell Non-Hodgkin lymphoma
  • 30-40% of all NHL
  • Prognosis and treatment determined by:
    • Precise histological diagnosis
    • Anatomical stage
    • IPI (international prognostic index)
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8
Q

What is the DLBCL International prognostic index?

A

A point for each:

  • Age >60 years
  • Serum LDH > normal
  • Performance status 2-4
  • Stage 3 or 4
  • More than one extranodal site

5 year predicted survival by number of risk factors:

0-1: 73%

2: 51%
3: 43%

4-5: 26%

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9
Q

What is the treatment for diffuse large b cell lymphoma?

A
  • Treated by 6-8 cycles of R-CHOP (Rituximab-CHOP)
  • Combination chemotherapy using a mixture of drugs usually including an anthracycline
  • Combination drug regimes e.g. CHOP:
    • Cyclophosphamide 750mg/m2 IV
    • Adriamycin 50mg/m2 IV
    • Vincristine 1.4mg/m2 IV
    • Prednisolone 40mg/m2 p.o
  • R is immunotherapy using the anti-CD20 monoclonal antibody Rituximab
  • Aim of therapy is curative
  • Relapse: Autologous Stem cell transplant salvage 25% of patients
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10
Q

Describe Follicular NHL

A
  • Indolent lymphoma
  • 35% of NHL
  • Associated with t(14;18) which results in ‘over-expression’ of bcl2 an anti-apoptosis protein
  • Incurable, median survival 12-15 years
  • May require 2-3 different chemotherapy schedules over the 12-15 year period
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11
Q

Describe the initial theraputic options for follicular NHL

A
  • Indolent slow progressing B cell NHL
    • Incurable
    • Variable/long natural history
  • At presentation watch and wait, and only treat ‘if clinically indicated’
    • Nodes compressing e.g. bowel, ureter, vena cava
    • Massive painful nodes, recurrent infections
  • Treatment:
    • Combination immuno-chemotherapy R-CVP
    • Maintenance rituximan delated time to next progression
    • Conventional treatment is not curative
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12
Q

Describe Marginal zone lymphomas

  • What it is
  • Cause
  • Median age of presentation
A
  • Marginal zone Non-Hodgkin lymphoma involving extranodal lymphoid tissue (i.e. mucosa associated lymphoid tissue)
  • Comprises 8% of all NHL
  • Caused by chronic antigen stimulation
    • Sjorgen’s syndrome - parotid lymphoma
    • H.pylori - gastric MALT lymphoma
    • Hashimoto’s thyroiditis - Thyroid
    • Lachrymal gland (?Psittaci infection)
  • Median age at presentation 55-60 year
  • Most commonly arise in stomach, usually present with dyspepsia or epigastric pain
    • Usual presentation is stage 1
    • B symptoms uncommon
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13
Q

Describe MALT lymphamogenesis

A
  • Proliferation of polyclonal antigen specific B cells caused by chronic gastritis caused by H.pylori infection
  • Chronic antigen stimulation leads to genetic changes (t(11;18)) leading to autonomous growth of antigen dependent B cells (autoantigens) and the development of a low grade lymphoma
  • Low grade MALT lymphomas can develop into high grade MALT lymphomas, my interaction with T cells and development of monoclonal B cells that are fast growing
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14
Q

What is the treatment for gastric MALT stage 1-2 disese?

A
  • Omprazole 20mg/Clarithromycin 500mg/amoxicillin 1gm bd
  • Repeat H.pylori breath test at 2 months
  • Repeat endoscopy every 6 months for first 2 years and then annually
  • Aim is to eradicate H.pylori and chronic antigen stimulation

if eradication therapy fails then they may require chemotherapy

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15
Q
  1. Describe Enteropathy Associated T cell lymphoma (EATL)
  2. Describe the presentation and clinical course
A

1.

  • Enteropathy Associated T cell lymphoma is a T cell Non-Hodgkin lymphoma see in patients with coeliac disease
    • mature T cells (not precursor)
    • Involcing small intestine jejunum and ileum
    • Has an aggressive clinical course
  • Chronic antigen stimulation
    • Gluten in a Gluten sensitive individual
  1. Presentation and clinical course:
  • Abdominal pain, obstruction and perforation, GI bleeding
  • Malabsorption
  • Systemic symptoms
  • Responds poorly to chemotherapy - generally fatal
  • Aim to prevent (need strict adherence to Gluten free diet)
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16
Q

Describe the epidemiology of Chronic lymphocytic leukemia

A
  • Proliferation of mature B cells
  • Commonest leukemia in the western world
  • Caucasian most affected
  • UK incidence 4.2/100,000 year
  • Age at presentation median 72 (10% aged 55 years)
  • Relatives x7 increased incidence
17
Q

What are the laboratory findings for CLL?

A
  • Lymphocytosis between 5 and 300 x109/l
  • Smear cells
  • Normocytic normochromic anaemia
  • Thrombocytopenia
  • Bone marrow lymphocytic replacement of normal marrow elements
18
Q

Which B cells proliferate in CLL?

A

Proliferation of mature B cells (CD19) Co-expressing CD5

CD5 +ve/CD19 +ve lymphocytes

19
Q

Describe what the following results would indicate

  1. Immature lymphoblasts (TdT positive)
  2. Small mature lymphocytes + smear cells, immunophenotyping shows mature B cells CD5 +ve
A
  1. Think Acute lymphoblastic leukemia
  2. Think CLL (could be with a lower score mantle cell lymphoma)
20
Q

What are the prognostic factors/what is available for prognosis?

A

Clinical - quantify the burden of malignant cells

  • Rai staging
  • Binet staging

Lab/malignant cell based:

  • CD38 expression = bad prognosis
  • Cytogenetics (FISH) panel
  • Immunoglobulin gene mutation status
    • IgH mutated
    • IgH unmutated
21
Q

Describe Binet staging of lymphoma - quantifying the burden of malignant cells

A
  • Stage A - <3 lymphoid areas, 12yr median survival
  • Stage B - >3 lymphoid areas, 5yr median survival
  • Stage C - Hb <100g/l, platelets <100x109/l, 2yr median survival
22
Q

Describe IgH and CLL

A

Multiple productive IgH (immunoglobulin heavy chain) rearrangements denote oligoclonality even in immunophenotypically monoclonal CLL.

IgH gene status:

  • 56% of CLL is unmutated
  • 44% of CLL IS mutated

Unmutated has a much poorer median survival rate

23
Q

Describe the different chromosomal abnormalities and their effect on prognosis

A
  • Normal karotype - median survival = 111 months

Abnormalities:

  • Deletion of 13q = 133 months
  • Trisomy 12 = 114 months
  • Deletion of 11q (ATM) = 79 months
  • Deletion of 17p (TP53, p53 deleted CLL) = 32 months
24
Q

What are the other clinical issues associated with each of the following:

  1. Malignant (non-functional) mature B cells + hypogammaglobulinaemia
  2. Proliferate within bone marrow
  3. Circulate to nodes, spleen and blood
  4. Acquired further mutations
  5. Disease of “immune cells”
A
  1. Increased risk of infection
  2. Bone marrow failure
  3. Lymphadenopathy +/- splenomegaly, lymphocytosis
  4. Transform to high grade lymphomas
  5. Auto-immune complications e.g. haemolytic anaemia
25
Q

What are the treatment principles in treating lymphoma?

A
  • Supportive treatment
    • Vaccination
    • Anti-infective prophylaxis and treatment
  • Specific scenarios
    • Auto-immune cytopenias
    • High grade (richter) transformation
  • Leukaemia directed treatment
    • Tailored to patient
26
Q

What supportive treatments are used and what are their complications?

A
  • Prophylaxis and treatment of infections
    • account for 50% of all CLL related deaths
    • Most are bacterial, but fungal amd viral are becoming increasingly prevalent
    • Prophylaxis
      • Aciclovir
      • PCP prophylaxis for those receiving Campath
      • IVIG is recommended for those with hypogammaglobinuemia and recurrent bacterial infections
      • Immunisation against pneumococcus, and seasonal flu
  • Autoimmune phenonmena
    • 1st line steroids, 2nd line Rituximab
27
Q

What are the variants of CLL?

A
  • Transformation to high grade lymphoma
  • Richter’s syndrome
    • 1% per year
    • Treat as a high grade lymphoma with CHOP-R
28
Q

Watch and wait unless:

  • Progressive lymphocytosis
    • Lymphocyte doubling time <6 months
  • Progressive marrow failure
    • Hb <100, platelets <100, Neutrophils <1
  • Massive or progressive lymphadenopathy/splenomegaly
  • Systemic symptoms (B symptoms)
  • Autoimmune cytopenias (treat with steroids)
A
29
Q

What is first line chemo-immunotherapy treatments when TP53 is intact for the following ages:

  1. Go go years (60-70)
  2. Slow go years (71-80)
  3. No go years (80+)
A

Most intensive treatment is for people who are in the Go go years

  1. Go go
  • FCR - Fludarabine, cyclophosphamide, Rituximab (anti-CD20 moab)
  • Rituximab-Bendamustine
  1. Slow go
    * Obinutuxumab (anti CD20) + Chlorambucil
  2. No go
    * Supportive care only
30
Q
  1. What are high risk CLL cases?
  2. What new agents are being used?
A
  1. High risk CLL:
  • Patients with TP53/17p deleted CLL 1st line
  • Refractory disease or early relapse (<24 months)
  1. New agents:
  • Ibrutinib (Bruton Tyrosine kinase inhibitor)
  • Venetoclax (anti-Bcl2 oral agent)

Haematology - Path (11 decks)

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