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Haematology > Lymphoid Malignancy > Flashcards

Lymphoid Malignancy Flashcards

1
Q

Features of lymphoma

A

Cancers of lymphoid origin
Can present with enlarged lymph nodes (lymphadenopathy) or with extra-nodal involvement or with bone marrow involvement
Systemic symptoms
> 50 different disease entities

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2
Q

How can lymphoma present?

A

Lymphadenopathy
Extra-nodal involvement
Bone marrow involvement
Systemic symptoms

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3
Q

What are the systemic symptoms associated with lymphoid malignancy?

A 
Weight loss - > 10% in 6 months
Fever
Night sweats
Pruritis
Fatigue
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4
Q

What is the disease entity in lymphoma defined by?

A

Malignant cell characteristics

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5
Q

What will determine the type of lymphoma?

A

Biopsy - lymph node or bone marrow

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6
Q

What does clinical examination and imaging of lymphoma tell us?

A

Where it is - not what it is

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7
Q

What are the lymphoproliferative disorders?

A

Acute lymphoblastic leukaemia
Chronic lymphocytic leukaemia
Hodgkin lymphoma

Non-Hodgkin lymphoma

  • high grade (diffuse large B cell lymphoma)
  • low grade (follicular, marginal zone)
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8
Q

Acute lymphoblastic leukaemia is a neoplastic disorder of

A

lymphoblasts

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9
Q

How is ALL diagnosed?

A

By > 20% lymphoblasts present in bone marrow

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10
Q

What is the incidence of acute lymphoblastic leukaemia?

A

1-2 per 100,000 population per year

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11
Q

What percentage of cases of ALL occur in children < 6 years?

A

75%

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12
Q

What percentage of cases of ALL are of B cell lineage?

A

75-90%

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13
Q

What is the typical presentation of ALL?

A

Present with 2-3 week history of bone marrow failure or bone/joint pain

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14
Q

What are the investigations of ALL?

A

FBC - Hb (low), WCC (high), platelets (high)
Bone marrow biopsy
Lymph node biopsy
Imaging

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15
Q

What are the treatment options of ALL?

A 
Chemotherapy 
Consolidation therapy 
CNS directed treatment 
Maintenance treatment 
Stem cell transplantation if high risk
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16
Q

What are poor risk factors for ALL?

A 
Increasing age
Increase WCC
Immunophenotype
Cytogenetics/molecular genetics - t(9;22), t(4;11) 
Slow/poor response to treatment
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17
Q

What is the outcome for adults with ALL?

remission rate and leukaemia-free survival

A

Complete remission rate 78-91%

Leukaemia-free survival at 5 years 30-35%

18
Q

What is the outcome for children with ALL?

5 year overall survival

A

5 year overall survival around 90%

Poor risk patients 5 year overall survival 45%

19
Q

What is the typical treatment of ALL?

A

Multi-agent intensive chemotherapy +/- allogeneic stem cell transplant

20
Q

How is CLL diagnosed?

A

Blood > 5 x 10^9/L lymphocytes
Bone marrow > 30% lymphocytes
Characteristic immunophenotyping - B cell markers (CD 19, 20, 23) and CD5 positive

21
Q

What is the incidence of CLL?

A

> 1700 new cases per year in UK
Commonest leukaemia worldwide

Rare in far-East
Occasionally familial

22
Q

What is the male:female incidence of CLL?

A

2 males:1 female

23
Q

What is the clinical presentation of CLL?

A

Often asymptomatic at presentation

Frequent findings

  • bone marrow failure (anaemia, thrombocytopenia)
  • lymphadenopathy
  • splenomegaly (30%)
  • fever and sweats (< 25%)

Less common findings

  • hepatomegaly
  • infections
  • weight loss
24
Q

What are the associated findings of CLL?

A

Immune paresis

Haemolytic anaemia

  • 20% have positive direct anti-globulin test
  • 8% have clinical evidence of haemolytic anaemia
25
Q

What is the Binet staging of CLL?

A

Stage A - < 3 lymph node areas

Stage B - 3 or more lymph node areas

Stage C - Stage B + anaemia or thrombocytopenia

26
Q

What is the median survival of Binet stage A, B and C CLL?

A

A - same as age-matched controls
B - around 8 years
C - around 6 years

27
Q

What are the indications for treatment of CLL?

A 
Progressive bone marrow failure 
Massive lymphadenopathy 
Progressive splenomegaly 
Lymphocyte doubling time < 6 months or > 50% increase over 2 months 
Systemic symptoms 
Autoimmune cytopenias
28
Q

What is the treatment of CLL?

A

Often nothing - watch and wait
Cytotoxic chemotherapy e.g. fludarabine, bendamusine
Monoclonal antibodies e.g. rituximab, obinutuzamab

Novel agents

  • bruton tyrosine kinase inhibitor e.g. ibrutinib
  • PI3K inhibitor e.g. idelalisib
  • BCL-2 inhibitor e.g. venetoclax
29
Q

What are the poor prognostic markers of CLL?

A 
Advanced disease - Binet stage B or C
Atypical lymphocyte morphology 
Rapid lymphocyte doubling time (< 12 months) 
CD38+ expression 
Loss/mutation of p53; del 11q23
Un-mutated IgVH gene status
30
Q 
27 y/o male
1 month impaired vision in both eyes 
1/2 stone weight loss
Breathless on minimal exertion 
Hb 38 g/L
WCC 370 x 10^9/L 
Platelets 68 x 10^9/L
Bone marrow 90% B lymphoblasts

What is the likely diagnosis?

A

Acute lymphoblastic leukaemia

31
Q

What is the presentation of lymphoma?

A 
Lymphadenopathy
Hepatosplenomegaly
Extranodal disease
B symptoms 
Bone marrow involvement
32
Q

What are the B symptoms?

A 
Fever
Night sweats (drenching) 
Weight loss (> 10% in 6 months)
33
Q

How is lymphoma assessed/staged?

A

Lymph node biopsy
CT
Bone marrow aspirate and trephine biopsy

34
Q

What is the epidemiology of Hodgkin lymphoma?

A

30% of all lymphomas
Bimodal age curve
- 1st peak at 15-35 years
- 2nd peak later in life

35
Q

What is the male:female incidence of Hodgkin lymphoma?

A

1.9 males:1 female

36
Q

What virus is Hodgkin lymphoma associated with?

A

Epstein Barr Virus

37
Q

What is the treatment of Hodgkin lymphoma?

A

Combination chemotherapy +/- radiotherapy

Use of PET scan to assess response to treatment and limit use of radiotherapy

38
Q

What is non-Hodgkin lymphoma classified according to?

A

Lineage - B cell or T cell, majority are B cell in origin
Grade of disease - high or low
Histological features of disease

39
Q

What are the grades of non-Hodgkin lymphoma and their features?

A

Low Grade Lymphoma

  • indolent, often asymptomatic
  • responds to chemotherapy but incurable
  • median survival varies by subtype

High Grade Lymphoma

  • aggressive, fast growing
  • requires combination chemotherapy
  • can be cured but varies widely
40
Q

What are the commonest specific disease entities of lymphoma and their features?

A

Diffuse large B cell lymphoma

  • commonest subtype of lymphoma
  • high grade

Follicular lymphoma

  • 2nd commonest subtype of lymphoma
  • low grade
41
Q

What is the treatment of Non-Hodgkin lymphoma?

A

Combination chemotherapy - typically anti-CD20 monoclonal antibody and chemotherapy

Haematology (11 decks)

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