Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Year 5 Pathology > Lymphoma 2: Chronic Lymphocytic Leukaemia and Lymphoproliferative disorder quiz > Flashcards

Lymphoma 2: Chronic Lymphocytic Leukaemia and Lymphoproliferative disorder quiz Flashcards

1
Q

HL: Diagnosis and Staging, Tx and Prognosis

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Lymphoma morphology process

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

HL: epidemiology

A

1% of all cancer
M>F (women sclerosis sub-type more often)
Bimodal age incidence: 20-29 (most common), >60 (smaller peak)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

HL: Signs and Sx

A

Painless enlargement of lymph nodes (-> may cause obstructive symptoms/signs)

  • B symptoms: fever, night sweats, weight loss
  • rarely: pruritus, alcohol-induced pain
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Nodular sclerosino HL sub-type

A
  • F > M (20-29yo)
  • Neck nodes and a mediastinal mass; may have B symptoms
  • Spreads contiguously
  • Needs tissue diagnosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

HL classification

A

Classical HL:

  • Nodular sclerosing 80% Good prognosis (causes the peak incidence in young women)
  • Mixed cellularity 17% Good prognosis
  • Lymphocyte rich (rare) Good prognosis
  • Lymphocyte depleted (rare) Poor Prognosis

Nodular Lymphocyte predominant HL 5% (disorder of the elderly multiple recurrences)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Staging of HL

A
  • FDG-PET / CT scan
  • Biopsy if other sites infilitrated

The diaphragm is key for staging  see diagram above…

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Staging of HL

A
  • FDG-PET / CT scan
  • Biopsy if other sites infilitrated

The diaphragm is key for staging  see diagram above…

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

HL tx

  • All patients with HL should receive chemotherapy however, radiotherapy is also often given after chemotherapy because HL is highly responsive to radiotherapy to clear up remaining cells
  • Combined modality = radiotherapy and chemotherapy used
  • Chemo and radiotherapy combined can lead to a HIGH risk of reoccurrence
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Outcome of HL therapy

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

HL Tx dilemma

A

Treatment dilemma:

  • HL is curable (~80%)
  • Intensify therapy → more cures (>80%) but more secondary cancers (>10%)
  • Reduce therapy → less secondary cancers (<10%) but less cures (<80%)

Non-Hodgkin’s Lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

NHL General

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Different types of NHL and their clinical behaviour

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Managing NHL

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Common and interesting lymphomas

A
  • COMMON = DLBCL, FL
  • INTERESTING = H. pylori MALToma, EATL, HIV-associated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

DLBC NHL (HIGH-GRADE, AGGRESSIVE)

A
  • Common form of NHL (30-40% of all NHLs)
  • Aggressive / high-grade
  • Prognosis and treatment determined by:
    • Histological diagnosis
    • Anatomical stage
    • IPI (International Prognostic Index)
16
Q

DLBCL Tx

A
17
Q

Follicular NHL (LOW-GRADE/INDOLENT) features

A
  • Indolent lymphoma
  • 35% of NHL
  • Associated with a t(14; 18) → over-expression of bcl-2 (an anti-apoptotic protein)
  • Used a FLIPI score (a modified version of IPI)
  • Incurable (median survival 12-15 years; requires 2-3 chemotherapy schedules over this period)
18
Q

Follicular NHL (LOW-GRADE/INDOLENT) initial tx options

A
19
Q

Extra-nodal Marginal Zone Lymphomas

A
20
Q

Enteropathy Associated T-cell Lymphoma (EATL)

A
21
Q

CLL definition and epidemiology

A
  • Proliferation of mature B cells
  • Epidemiology:
    • Most common (UK = 4.2/100,000/year) leukaemia in West
    • Tends to affect Caucasians
    • Median age at presentation: 72 years (10% aged <55yo)
    • Relatives have 7 x increased risk
22
Q

CLL Lab findings

A
  • Lymphocytosis (5-300 x 109/L)
  • Smear cells (weak cells so break when put on a slide)
  • Normocytic normochromic anaemia
  • Thrombocytopaenia
  • Bone marrow lymphocytic replacement of normal marrow elements

NOTE: as this is an indolent leukaemia, it is often only picked up during routine blood tests for other reasons

23
Q

How do we check maturity of B cells (what about T cells?)

A
  • Maturity of B cells can be determined from its antigen expression, identified through immunophenotyping– B-cells express different antigens as they progress through development – mature B-cells specifically express:
    • sIg
    • CD-19
24
Q

CLL Prognosis: VH mutations

A
  • VH = variable heavy chain (the part that undergoes somatic hypermutation by VDJ recombination)
  • Pre-germinal centre B cells will have Ig-genes (VH genes) that have NOT undergone somatic hypermutation. In other words, they are UNMUTATED and conform precisely to the germline sequence
  • Once antigen selection is complete, the B cell has undergone somatic mutations in the VDJ region and so it can be said that the B cell has had it’s VH region mutated (physiologically), post-germinal centre
  • Half of CLL patients have MUTATED VH (i.e. arising from post-germinal centre cells) and half have UNMUTATED VH (i.e. arising from pre-germinal centre cells)  prognosis much worse in patients with an UNMUTATED VH
    • Mutated = 25 years survival
    • Unmutated = 8 years survival
25
Q

CLL Prognosis: biological prognostic factors which common genetic abnormality is important

A
  • There is a collection of common genetic abnormalities that are screened using FISH:
    • Deletion of 13q ~133 months survival
    • Trisomy 12 ~114
    • Deletion of 11q (ATM) ~79
    • Deletion of 17p (TP53)  loss of p53 tumour suppressor gene
    • The most important is DELETION of 17p (TP53)
      • These don’t tend to respond to chemotherapy
26
Q

CLL clinical issues

A
  • Although high lymphocytes, the cells are mono-clonal and so NOT useful for antibody production and an abundance of non-functioning B cells  hypogammaglobulinemia as normal B cells are being suppressed
    • This leads to increased risk of infection (e.g. pneumonia, sinusitis)
    • Healthy bone marrow will become suppressed leading to bone marrow failure (anaemia, infection, bleeding)
    • Malignant lymphocytes can spread to lymphoid organs (e.g. lymph nodes, spleen)
    • Although this is an indolent disease, there is a 1% chance every year that CLL can acquire more mutations and undergo a RICHTER TRANSFORMATION (become a high grade lymphoma)
    • Presence of a population of malignant B cells  deregulation of the surviving normal B cell population
      • This can result in autoimmune disease (e.g. autoimmune haemolytic anaemia)
27
Q

CLL treatment (3)

A

(1) Supportive (as 50% of CLL-related deaths due to infections)

  • Vaccination (flu, pneumococcus) – no live vaccines (i.e. VZV)
  • Anti-infective prophylaxis and treatment
    • Acyclovir for viral infections
    • PCP prophylaxis for those that are immunosuppressed
    • IVIG for those with hypogammaglobulinaemia and recurrent bacterial infections

(2) Specific Scenarios:
* High-Grade (Richter) Transformation  treat as high grade lymphoma (R-CHOP)
(3) Leukaemia-directed Treatment:

  • Tailored to the patient
    • Elderly
    • Young
  • Need irradiated blood products if they are at risk of transfusion-associated graft-versus-host disease
  • Incurable by chemo → watch and wait preferred over any active treatment, esp. in elderly patients
    • The decision to treat is often dependent on the age and comorbidities of the patient
    • The aim of therapy is to establish remission
28
Q

CLL indications for tx (i.e. when to stop watching and waiting)

A
  • Progressive lymphocytosis (count doubling < 6 months)
  • Progressive bone marrow failure (Hb < 100; Platelets < 100; Neutrophils < 1)
  • Massive or progressive lymphadenopathy/splenomegaly
  • Systemic symptoms (B symptoms)
29
Q

CLL 1st line chemo-immunotherapy (TP53 intact)

A

(1) FCR or R-Bendamustine (combination chemotherapy)

  • Fludarabine
  • Cyclophosphamide
  • Rituximab (anti CD20 moab)

(2) Obinutuzumab (anti CD20) + Chlorambucil (single agent chemo)
(3) Supportive care only

30
Q

High-risk CLL cases

A

Patients with TP53/17p deleted CLL 1st Line

Refractory disease or early relapse (<24 months)

31
Q

CLL new agent tx (VERY EXPENSIVE)

A

Year 5 Pathology (27 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions