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A Haematology and Immunology > Lymphoid Malignancies > Flashcards

Lymphoid Malignancies Flashcards

1
Q

What is lymphoma?

A

Cancer of lymphoid origin which can present with

  • Lympadeonpathy OR
  • Extranodal involvement OR
  • Bone marrow involvement
  • Systemic B symptoms including weight loss (>10 % in 6 months), fever, night sweats, pruritus, fatigue
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2
Q

How is are differnt types of lymphoma defined?

A

By the malignant cell characteristics

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3
Q

What investigation tells us what type of lymphoma it is?

A

Biopsy

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4
Q

What investigations tells us where the lymphoma is?

A

Clinical examination and imaging

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5
Q

What is a high grade non-Hodgkin lymphoma?

A

A diffuse large B cell lymphoma

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6
Q

Give examples of lymphoproliferative disorders in order from most common.

A
  • Most common
  • Non-Hodgkin lymphoma (NHL)
    • High-grade (diffuse large B-cell lymphoma)
    • Low-grade (follicular, marginal zone)
  • Chronic lymphocytic leukaemia (CLL)
  • Hodgkin lymphoma
  • Acute lymphoblastic leukaemia (ALL) (common in children)
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7
Q

What is acute lymphoblastic leukaemia?

A

Cancerous disorder of lymphoid progenitor cells

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8
Q

How is acute lymphoblastic leukaemia diagnosed?

A

By >20% lymphoblasts present in bone marrow

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9
Q

What is the epidemiology of acute lymphoblastic leukaemia?

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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10
Q

What is the standard treatment for acute lymphoblastic leukaemia?

A
  • Induction chemotherapy to obtain remission
  • Consolidation therapy
  • CNS directed treatment
  • Maintenance treatment for 18 months
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11
Q

What other treatment is there for acute lymphoblastic leukaemia?

A
  • Stem cell transplantation (if high risk)
  • Bispecifc T-cell engagers (BiTe molecules): e.g. Blinatumumab
  • CAR (chimeric antigen receptor T-cells)
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12
Q

How is CAR treatment carried out?

A
  • Patient/ healthy 3rd party T-cells harvested
  • T-cells are genetically modified so that they target CD19 expressed on leukaemia cells
  • Expanded in vitro
  • Re-infused into patient
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13
Q

How does T-cell immunotherapy associated neurotoxicity present?

A
  • Confusion with normal conscious level
  • Seizure, headache, focal neurology, coma
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14
Q

How does T-cell immunotherapy associated cytokine release syndrome present?

A
  • Fever, hypotension, dyspnoea
  • CAR T-cell effect correlated to presence of CRS (significant number require ITU support)
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15
Q

What are poor prognostic factors associated with acute lymphoblastic leukaemia?

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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16
Q

What are the outcomes of acute lymphoblastic leukaemia?

A

Adults with ALL

  • Complete remission rate 78–91%
  • Leukaemia-free survival at 5y 30–35%

Children with ALL

  • 5y overall survival ~90%
  • Poor risk patients (slow response to induction or Philadelphia positive) 5y OS 45%
17
Q

What is the typical presentation of acute lymphoblastic leukaemia?

A
  • Bone marrow failure +/- raised white cell count
  • Bone pain, infection, sweats
18
Q

How is chronic lymphocytic leukaemia diagnosed?

A
  • Blood > 5 x 109/L lymphocytes (normal is <4)
  • Bone marrow > 30% lymphocytes
  • Characteristic immunophenotyping (B-cell markers (CD 19, 20, 23) & CD5 positive)
19
Q

What is the incidence of CLL?

A
  • > 1700 new cases CLL per year in the UK
  • Commonest leukaemia worldwide
  • M:F 2:1
  • Occasionally familial
  • Rare in far East
20
Q

How does CLL present?

A

Often asymptomatic

Frequent findings

  • Bone marrow failure (anaemia, thrombocytopenia)
  • Lymphadenopathy
  • Splenomegaly (30%)
  • Fever and sweats (< 25%)

Less common findings

  • Hepatomegaly
  • Infections
  • Weight loss
21
Q

What conditions/states are associated with CLL?

A
  • Immune paresis (loss of normal immunoglobulin production)
  • Haemolytic anaemia (20% have positive direct antiglobulin test, 8% clinical evidence of haemolytic anaemia)
22
Q

What system is used to stage CLL?

A

Binet

23
Q

What are the stages of CLL according to the Binet system?

A
  • Stage A -<3 lymph nodes
    • Same survival as age matched controls
  • Stage B -3 or more lymph node areas
    • -~8 year survival
  • Stage C -Stage B + anaemia or thrombocytopenia
    • ~6 years survival
24
Q

What are the indications for treatment in 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
25
Q

What is the treatment for 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 eg ibrutinib) (PI3K inhibitor eg idelalisib) (BCL-2 inhibitor eg venetoclax)
26
Q

What are poor prognostic factors in CLL?

A
  • Advanced disease (Binet stage B or C)
  • Atypical lymphocyte morphology
  • Rapid lymphocyte doubling time (<12 mth)
  • CD 38+ expression
  • Loss/mutation p53; del 11q23 (ATM gene)
  • Unmutated IgVH gene status
27
Q

How is chronic lymphocytic leukaemia different from acute lymphoblastic leukaemia?

A
  • Unlike ALL, the abnormal cells are mature - they usually resemble normal, well-behaved lymphocytes
  • Grow slowly (or not at all)
  • Classic example of a low-grade condition
  • Carry many of the normal markers that B lymphocytes have
28
Q

How does lymphoma present?

A
  • Lymphadenopathy/ hepatosplenomegaly
  • Extranodal disease
    • “B symptoms”
    • Bone marrow involvement
29
Q

How is lymphoma assessed and staged?

A
  • Lymph node biopsy
  • CT scan
  • Bone marrow aspirate and trephine
30
Q

What are the stages of lymphoma?

A
  • Can be classified by presence of B symptoms
  • Stage 1 = 1 node involved
  • Stage 2 = 2 nodes
  • Stage 3 = multiple nodes
  • Stage 4 = node and hepatosplenomegaly
31
Q

How is non-Hodgkin lymphoma classified?

A
  • Lineage (B-cell or T-cell) (Majority are B-cell in origin)
  • Grade of disease (high grade or low grade)
  • Histological features of disease
32
Q

What are the features of low grade NH lymphoma?

A
  • Indolent, often asymptomatic
  • Responds to chemotherapy but incurable
  • Median survival varies by subtype
33
Q

What are the features of high grade NH lymphoma?

A
  • Aggressive, fast-growing
  • Require combination chemotherapy
  • Can be cured, but again varies widely
34
Q

Give examples of specific NH lymphoma disease entitie.

A
  • Diffuse large B-cell lymphoma
    • Commonest subtype of lymphoma (of any kind)
    • High-grade lymphoma
  • Follicular lymphoma
    • 2nd commonest subtype of lymphoma
    • Low-grade lymphoma
35
Q

How is NH lymphoma treated?

A

Combination chemotherapy, typically anti-CD20 monoclonal antibody and chemo

36
Q

What is the epidemiology of Hodgkin lymphoma?

A
  • 30% of all lymphomas
  • Bimodal age curve with 1st peak at 15-35y and 2nd peak later in life
  • M:F 1.9:1
  • association with Epstein Barr virus; familial and geographical clustering
37
Q

What is the treatment for 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

A Haematology and Immunology (20 decks)

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