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YR5 Haematology > Chronic Lymphocytic Leukaemia (all within Lymphoma deck) > Flashcards

Chronic Lymphocytic Leukaemia (all within Lymphoma deck) Flashcards

1
Q

What is the typical presentation of lymphoma?

A

Painless progressive lymphadenopathy

  • Palpable node
  • Extrinsic compression of any “tube”: e.g. Ureter, bile duct, large blood vessel, bowel, trachea, oesophagus

Infiltrate/impair an organ system

  • e.g. Skin rash, ocular + CNS, liver failure

Recurrent infections

Constitutional Sx

Coincidental e.g. FBC, Imaging

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2
Q

What are the two broad classifications of B cell Non-Hodgkin’s Lymphoma?

A

Precursor B lymphoblastic leukaemia

Mature B lymphoblastic leukaemia

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3
Q

What are common types of B cell lymphoma?

A

Diffuse Large B-Cell Lymphoma (DLBCL)

Follicular NHL

CLL

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4
Q

What are the two broad classification of T cell lymphoma?

A

Precursor T lymphoblastic leukaemia or lymphoma (T-ALL)

Mature T + NK neoplasm

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5
Q

What are common types of T cell lymphoma?

A

PTCL

Anaplastic

Cutaneous

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6
Q

Summarise the epidemiology of Hodgkin’s Lymphoma.

A
  • 1% of all cancer, 3:100,000
  • HL M>F

Bimodal age incidence:

  • Most common 20-29y, young F NS subtype
  • smaller peak affecting elderly >60y
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7
Q

What are signs and symptoms associated with lymphoma?

A

Painless enlargement of LN(s).

May cause obstructive Sx/signs

Constitutional Sx:

  • Fever
  • Night sweats
  • Weight loss
  • Pruritis + rarely alcohol induced pain
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8
Q

What are the four types of classical Hodgkin’s Lymphoma?

A

Nodular sclerosing

Mixed cellularity

Lymphocyte rich

Lymphocyte depleted

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9
Q

What is the most common type of Hodgkin’s Lymphoma?

A

Nodular sclerosing

80%

Good prognosis

Causes peak incidence in young women

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10
Q

Which Hodgkin’s Lymphoma are rare?

A

Lymphocyte rich: Rare - Good prognosis

Lymphocyte depleted: Rare - Poor Prognosis

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11
Q

How common is mixed cellularity Hodgkin’s Lymphoma?

A

17% - uncommon

Good prognosis

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12
Q

How is Hodgkin’s Lymphoma staged and why is this important?

A

Following pathological dx of a LN biopsy patients are ‘staged’ this has prognostic significance + also may determine the best approach for therapy.

FDG-PET/CT scan

Consider biopsy of other site if possibly infiltrated e.g. liver

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13
Q

What is the staging for Hodgkin’s Lymphoma?

A

Stage:

  • I: 1 group of nodes
  • II: >1 group of nodes same side of the diaphragm
  • III: Nodes above + below the diaphragm
  • IV: Extra nodal spread

Suffix A if none of below, B if any of below

  • Fever
  • Unexplained Weight loss >10% in 6m
  • Night sweats
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14
Q

What is the management for classical Hodgkin’s Lymphoma?

A

Combination chemotherapy - ABVD:

  • Adriamycin
  • Bleomycin
  • Vinblastine
  • DTIC

ABVD is given at 4-weekly intervals + is effective tx. Preserves fertility (unlike MOPP the original chemo).

Can cause (long term):

  • Pulmonary fibrosis
  • Cardiomyopathy
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15
Q

How is chemotherapy administered in classical Hodgkin’s Lymphoma? What other management options are available?

A

Chemotherapy (essential for cure)

  • ABVD 2-6 cycles (depends: stage + interim response)
  • PET CT
  • Interim: After x2 cycles, response assessment
  • End of Tx: Guides need for additional radiotherapy

n+/- Radiotherapy

Relapse (salvage chemotherapy)

High dose chemotherapy + Autologous PB stem cell transplant as support

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16
Q

What risks are associated with radiotherapy for classical Hodgkin’s Lymphoma.

A

Low/negligible risk of relapse

Risk of damage to normal tissue (collateral damage)

  • Ca breast (risk 1:4 after 25 y)
  • Leukaemia/mds (3%@10y)
  • Lung or skin cancer

Combined modality (chemo + radio) is the greatest risk of 2o malignancy

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17
Q

What is the prognosis of classical Hodgkin’s Lymphoma?

A

Older patients + lymphocyte-depleted histology do less well

Cure rate ranges from 50-90%.

> 80% with stage I or II disease are cured

50% of stage IV patients are cured

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18
Q

What is Non-Hodgkin’s Lymphoma?

A

Neoplastic proliferation of lymphoid cells.

Clinical course highly variable :

  • Fastest proliferating malignancy (Burkitt Lymphoma)
  • Indolent diseases (eg Follicular NHL with possible 25y survival)
  • Abx responsive disease such as Gastric MALT
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19
Q

How is Non-Hodgkin’s Disease staged?

A

CT scan

PET scan (indicated in aggressive lymphomas)

BM biopsy

Lumbar puncture (if risk of CNS involvement)

20
Q

What are 4 prognostic markers of Non-Hodgkin’s Lymphoma?

A

LDH

Performance status

HIV serology (if appropriate HTLV1 serology)

Hepatitis B serology (risk of reactivation if B cell depleting therapy given)

21
Q

What is the most common type of Non-Hodgkin’s Lymphoma?

A

Diffuse Large B Cell Lymphoma

(Followed by Follicular Lymphoma)

22
Q

Which types of Non-Hodgkin’s Lymphoma are classified by the WHO as being high grade and very aggressive?

A

Burkitt’s Lymphoma

T or B cell Lymphoblastic leukaemia/ lymphoma

23
Q

Which types of Non-Hodgkin’s Lymphoma are classified by the WHO as being high grade and aggressive?

A

Diffuse Large B cell

Mantle cell

24
Q

Which types of Non-Hodgkin’s Lymphoma are classified by the WHO as being low grade and indolent?

A

Follicular

Small lymphocytic/ CLL

Mucosa associated (MALT)

25
Q

What is the predicted survival and curability of high grade, very aggressive forms of Non-Hodgkin’s Lymphoma?

A

Weeks 2-5 (Without Rx)

Curable

26
Q

What is the predicted survival and curability of high grade, aggressive forms of Non-Hodgkin’s Lymphoma?

A

Months 3-12 (Without Rx)

Moderately curable

27
Q

What is the predicted survival and curability of low grade, indolent forms of Non-Hodgkin’s Lymphoma?

A

Years 10-15

Incurable (long remission)

28
Q

How are very aggressive Non-Hodgkin’s Lymphoma managed?

A

These are treated like acute leukaemia

29
Q

What are the prognostic characteristics of DLBCL?

A

IPI (International Prognostic Index):

  • Age
  • Stage (Ann Arbor)
  • LDH
  • Extra-nodal disease sites
  • ECOG performance status
30
Q

What is the treatment of DLBCL?

A

R-CHOP

Rituximab: Anti-CD20 monoclonal antibody

Combination therapy e.g.

  • Cyclophosphamide
  • Adriamycin
  • Vincristine
  • Prednisolone

Aim of therapy is curative (overall ~50%)

Relapse: Autologous Stem Cell transplant salvage 25% of patients

31
Q

What is Follicular NHL?

A

A/w t(14;18) which results in over-expression of bcl2 an anti-apoptosis protein.

FLIPI score (modified IPI) to stage.

Incurable, median survival 12-15y

32
Q

What are extranodal marginal zone lymphomas?

A

Marginal zone NHL involving extra-nodal lymphoid tissue (e.g. Gastric mucosa-associated lymphoid tissue MALT/ H.Pylori, Parotid MZL / Sjogren syndrome).

  • Comprise ~ 8% of all NHL
  • Chronic antigen stimulation; H.Pylori infection
  • Median age at presentation 55-60y

Most commonly arise in stomach, usually present with epigastric pain, ulceration or bleeding. Usual presentation is Stage I[E].

Constitutional Sx uncommon.

H.Pylori eradication may cure 75% of patients.

33
Q

What is Enteropathy Associated T-cell Lymphoma?

A

T cell NHL seen in pts with Coeliac disease

Mature T cells. Involving small intestine, jejunum + ileum. Aggressive

Chronic antigen stimulation, triggered by gluten in a gluten sensitive individual.

Presentation & Clinical course:

  • Abdo pain, obstruction, perforation, GI bleed
  • Malabsorption
  • Systemic Sx
  • Responds poorly to chemo, generally fatal
  • Aim to prevent (strict adherence to GF diet)
34
Q

What is chronic lymphocytic leukaemia?

A

Proliferation of mature B-lymphocytes.

Commonest leukaemia in the western world. Caucasian.

Age at presentation median 72 (10% <55y)

Relatives x7 increased incidence

35
Q

What are 5 common laboratory features of CLL?

A

Lymphocytosis: 5-300 x 109/l

Smear cells

Normocytic normochromic anaemia

Thrombocytopenia

BM: Lymphocytic replacement of normal marrow elements

36
Q

What is the timecourse of CLL?

A

Highly variable natural hx:

  • Initially 5-10y good health until progression to a 2-3y terminal phase.
  • Rapid progression to death within 2-3y.

In a disorder of elderly:

  • 1/3 Never progress
  • 1/3 Progress, respond to CLL Rx (death from unrelated disorder)
  • 1/3 Progress, require multiple lines of Rx, refractory disease, death from CLL
37
Q

What are prognostic features of CLL and how is it staged?

A

Cell based prognostic factors:

  • IgHV mutation status
  • CLL FISH cytogenetic panel
  • TP53 mutation status (Chr 17p del +/- TP53 point mutation)

Clinical staging systems:

  • Binet or Rai (clinical staging)
  • CLL IPI score
38
Q

What is the median survival of CLL?

A
  • Mutated: 25y
  • Unmutated: 8y
39
Q

What are clinical complications with CLL?

A

Population of malignant (non functional) mature B cells+ hypogammaglobulinaemia: Increased risk of infection

Proliferate within BM (efface): BM failure

Circulate to nodes, spleen + blood: Lymphadenopathy+/ splenomegaly, lymphocytosis

Acquire further mutations: Transform to high grade lymphoma; Richter Transformation (1% per year)

Disease of immune cells: AI complications e.g. Immune haemolytic anaemia

40
Q

How can sino-pulmonary infections in CLL be treated?

A

Early Rx with abx

Pneumocystis prophylaxis (may also require zoster ppx)

Recurrent infection + IgG < 5g/l > IVIG replacement therapy

41
Q

Which vaccinations should be offered to patients with CLL?

A

Pneumococcal

Covid19

Seasonal flu

Avoid live vaccines

42
Q

Watch and wait is the preferred approach to CLL.

When should treatment be given?

A

Progressive lymphocytosis:

  • >50% Increase over 2m
  • Lymphocyte doubling time <6m

Progressive BM failure:

  • Hb < 100, platelets <100, neutrophils <1

Massive or progressive lymphadenopathy/splenomegaly

Systemic Sx

AI cytopenias (treat with immunosuppression not chemotherapy)

43
Q

What therapy can be offered for CLL patients?

A

Combination Immuno-chemotherapy (being superseded by targeted Rx)

Targeted Therapy:

  • BTK inhibitor
  • BCL2 inhibitor

Cellular therapy only for relapsed high risk cases:

  • Allogeneic SCT
  • CAR-T therapy
44
Q

Which BCR kinase can be used to treat CLL?

A

Ibrutinib (BTK)

Idelalisib (PI3K)

45
Q

Which BCL2 inhibitors can be used to treat CLL?

A

Venetoclax

46
Q

Which experimental cell based therapies can be used to treat CLL?

A

Chimeric Antigen Receptor T cells (CAR-T)

47
Q

How does venetoclax work in CLL?

A

Orally active Bcl 2 inhibitor, permits apoptosis of CLL cells.

In high risk CLL p53 mutated 85% response + maintained at >1y.

Main risk is tumour lysis syndrome when initiating therapy (potentially fatal).

YR5 Haematology (20 decks)

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