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haematology and immunology > Lymphoid malignancy > Flashcards

Lymphoid malignancy Flashcards

1
Q

What is a lymphoma?

A

Cancers of lymphoid origen

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2
Q

What can a lymphoma present with?

A

> enlarged lymph nodes (lymphadenopathy)/ hepatosplenomegaly
Extranodal involvement
B symptoms
Bone marrow involvement

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3
Q

What are the systemic (B) symptoms of a lymphoma?

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

What are the lymphoproliferative disorders?

A 
○ Acute lymphoblastic leukaemia (ALL)
○ Chronic lymphocytic leukaemia (CLL)
○ Hodgkin lymphoma
○ Non-Hodgkin lymphoma (NHL)
- High-grade (diffuse large B-cell lymphoma)
- Low-grade (follicular, marginal zone)
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5
Q

What is acute lymphoblastic leukaemia?

A

○ Neoplastic disorder of lymphoblasts

○ Diagnosed by > 20% lymphoblasts present in bone marrow

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6
Q

What is the epidemiology of ALL?

A

○ Incidence 1-2/100,000 population/year
○ 75% cases occur in children < 6 years
○ 75-90% cases are of B-cell lineage

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7
Q

What is the presentation of ALL?

A

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

  • Bone marrow failure +/- raised WCC
  • Bone pain, infection, sweats
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8
Q

What are the standard treatments for ALL?

A

□ Induction chemotherapy to obtain remission
□ Consolidation therapy
□ CNS directed treatment
□ Maintenance treatment for 18 months

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9
Q

When would stem cell transplantation be used to manage ALL?

A

If the patient is high risk

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10
Q

What are the newer therapies for ALL?

A

□ Bispecific T-cell engagers (BiTe molecules) – e.g. Blinatumomab
□ CAR (chimeric antigen receptor T-cells)
® Patient/ healthy 3rd party T-cells harvested
® Transfected to express a specific T-cell receptor expressed on leukaemia cells (CD19)
® Expanded in vitro
® Re-infused into patient

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11
Q

What are the risk factors for a poor prognosis in ALL?

A
  • Increasing age
  • Increased white cell count
  • Immunophenotype (more primitive forms)
  • Cytogenetics/molecular genetics
    □ t(9;22); t(4;11)
  • Slow/poor response to treatment
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12
Q

How is chronic lymphocytic leukaemia diagnosed?

A
  • Blood > 5 x 10^9/L lymphocytes
  • Bone marrow > 30% lymphocytes
  • Characteristic immunophenotyping
    □ B-cell markers (CD 19, 20, 23) & CD5 positive
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13
Q

What is the presentation of chronic lymphocytic leukaemia?

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
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14
Q

What are the associated findings of CLL?

A
  • Immune paresis (loss of normal immunoglobulin production)
  • Haemolytic anaemia
    □ 20% have positive direct antiglobulin test
    □ 8% clinical evidence of haemolytic anaemia
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15
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
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16
Q

What is the treatment of CLL?

A
  • Often nothing: “watch and wait”
  • Cytotoxic chemotherapy e.g. fludarabine, bendamustine
  • 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
17
Q

What are the poor prognostic factors of CLL?

A
  • Advanced disease (Binet stage B or C)
  • Atypical lymphocyte morphology
  • Rapid lymphocyte doubling time (<12 months)
  • CD 38+ expression
  • Loss/mutation p53; del 11q23 (ATM gene)
  • Unmutated IgVH gene status
18
Q

How is a lymphoma assessed?

A

Lymph node biopsy/ CT scan/ bone marrow aspirate and trephine

19
Q

How is non-hodkin’s lymphoma classified?

A 
- lineage (B-cell or T-cell)
□ Majority are B-cell in origin
- grade of disease (high grade or low grade)
□ Low grade lymphoma
® Indolent, often asymptomatic
® responds to chemotherapy but incurable
® median survival varies by subtype
□ High grade lymphoma
® Aggressive, fast-growing
® Require combination chemotherapy
® Can be cured, but again varies widely
- histological features of disease
20
Q

What is a diffuse large B-cell lymphoma?

A

□ Commonest subtype of lymphoma (of any kind)
□ High-grade lymphoma
□ Treated with combination chemotherapy: typically anti-CD20 monoclonal antibody + chemo

21
Q

What is a follicular lymphoma?

A

□ 2nd commonest subtype of lymphoma
□ Low-grade lymphoma
□ Treated with combination chemotherapy: typically anti-CD20 monoclonal antibody + chemo

22
Q

What is the epidemioogy of Hodgkin lymphoma?

A
  • 30% of all lymphomas
  • bimodal age curve:
    □ 1st peak at 15-35y
    □ 2nd peak later in life
  • 1.9 males: 1 female
  • association with Epstein Barr virus; familial and geographical clustering
23
Q

What is the treatment of Hodgkin lymphoma?

A
  • Combination chemotherapy (ABVD)
  • +/- radiotherapy
  • Monoclonal antibodies (anti-CD30)
  • Immunotherapy (checkpoint inhibitors)
  • Use of PET scan to assess response to treatment and to limit use of radiotherapy

haematology and immunology (11 decks)

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