Haematology 12 - Lymphoma 2 Flashcards by Adya Rao

What are the investigations you would do for suspected lymphoma?

**remember about HIV and HTLV tests which can be contributing factors to certain subtypes of lymphoma

**also HBV as treatment for lymphoma (immunosuppression) can lead to reactivation of the virus

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What are the clinical presentations of lymphoma?

painless lymphadenopathy, enlarging

—> palpable mass OR

–> extrinsic compression of tubes - bile duct, ureter, bowels etc.

infiltration of organs eg CNS, skin, liver failure
recurrent infections- tumour of immune cells
constitutional symptoms if rapidly enlarging and spreading

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How do Reed Sternberg cells appear on the blood film?

Giant cell surrounded by reactive eosinophils

**look like owl’s eyes

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Classification of lymphoid malignancies

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What is the age distribution of Hodgkin’s lymphoma?

20-29: mostly affects females. mostly of the nodular sclerosing type

Elderly: M=F

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Classification of HL

Key one is “nodular sclerosing” classical HL- this is the one that affects young people more

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What are the symptoms of Hodgkin’s lymphoma?

Painless lymphadenopathy, that becomes painful on drinking alcohol
Constitutional B symptoms- fever, night sweats, weight loss
If advanced lymphadenopathy, may –> obstructive symptoms

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What is the cause of constitutional B symptoms in lymphoma?

Hyper-catabolic state

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Which investigation is used to diagnose hodgkin’s lymphoma, and which diagnosis is used for staging?

Diagnosis: LN biopsy
Staging: FDG-PET

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Recall the different stages of lymphoma (Ann arbor staging)

I: one group of nodes
II: >1 group of nodes, on one side of the diaphragm
III: Nodes on both sides of the diaphragm
IV: extranodal spread
Then:
A: no B symptoms
B: one/ any of fever/ weight loss/ night sweats

**initially developed for Hodkin’s lymphoma but also now used in NHL

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Which subtype of lymphoma is most likely to affect young women?

Nodular sclerosing classical Hodgkin’s

*usually presents with a mediastinal mass

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What is the mainstay of tretament for HL?

Chemotherapy.

aka ABVD chemotherapy

A- adriamycin

B- bleomycin

C- vincristine

D- dacarbazine

can give radiotherapy as an adjunct but it is not sufficient on its own.

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What type of chemotherapy is used in Hodgkin’s lymphoma?

ABVD

**Hodkin’s lymphoma is the first cancer that was treated with chemotherapy

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After how many cycles of chemotherapy for Hodgkin’s lymphoma should the FDG-PET be repeated to check response?

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What is the risk of giving radiotherapy for Hodgkin’s lymphoma?

It produces a lot of collateral damage (eg risk of breast cancer, leukaemia/MDS, lung or skin cancer), and when given alongside chemotherapy increases the risk of secondary malignany significantly

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What is the prognosis of hodkin’s lymphoma?

in the first 10 years the greatest risk is posed by recurrence of lymphoma
in the next few years the greatest risk is posed by:
a) secondary malignancy
b) cardiovascular events
i. e. the consequences of giving high dose chemotherapy and radiotherapy

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What are the most common forms of NHL?

Follicular NHL - low grade
Diffuse large B cell - high grade

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Recall the clinical severity of 3 different NHL subtypes

Burkitt’s lymphoma: very aggressive
Follicular lymphoma- indolent
Gastric MALT- responsive to antibiotic therapy

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Recall 4 prognostic markers in NHL

Performance status

LDH and B2 microglobulin
HIV serology
Hep B serology

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How aggressive is diffuse large B cell non-Hodgkin’s lymphoma?

High grade

**this is diff from Burkitt’s lymphoma lol

How is diffuse large B cell non-Hodgkin’s lymphoma treated?

R-CHOP and immunotherapy

R- rituximab

C- cyclophosphamide

O -

How aggressive is follicular non-Hodgkin’s lymphoma?

Indolent

How does the median survival and response to chemotherapy vary between the different grades of NHL

As the tumour becomes more aggressive, the median survival decreases (without treatment), but response to treatment (i.e. “curability”) increases

with the indolent tumours you tend to have more relapses and longer clinical course

What mutation is commonly associated with follicular non-Hodgkin’s lymphoma?

t(14;18)
Translocation of Bcl2 –> oncogene

Which subtype of non-Hodgkin's lymphoma is associated with chronic H. pylori?

Extra-nodal marginal zone lymphoma Gastric Marginal zone lymphoma

What are the symptoms of extra-nodal marginal zone lymphoma?

Epigastric pain, ulceration and bleeding

How aggressive is enteropathy-associated non-hodgkin's lymphoma?

Very aggressive

What is the main association of enteropathy-associated non-hodgkin's lymphoma?

Coeliac

What finding on a blood film is typial of CLL?

Smear/ smudge cells

Recall the surface markers of intermediate B cells vs mature B cells vs CLL mature B cells

Intermediate B cell: CD5 positive Mature B cell: CD19 positive Mature CLL B cell: CD5 positive and CD19 positive i.e. they aberrantly express CD5 which should not be seen in mature B cells

What are the 2 staging methods that can be used in CLL?

Rai staging Binet staging

Is CLL a pre- or post-germinal centre malignancy?

50% pre, 50% post \*\*rmb it's a malignancy of naive B cells - i.e. they can still be pre or post germinal centre

How can pre- and post-germinal centre CLL be differentiated?

Look for VH mutation status - unmutated has worse prognosis than mutated

Which one has worse prognosis: pre or post germinal centre CLL?

Pre (unmutated) is worse prognosis

Which mutation is associated with a particularly poor prognosis in CLL?

17p deletion (TP53)

What is Richter's syndrome?

Rare transformation of CLL to high grade lymphoma Often presents with New-onset B symptoms on a background of CLL * fever * **elevated serum levels of LDH** * rapidly enlarging lymph nodes High grade lymphoma - diffuse large b cell lymphoma. treat it as you would for DLBL (R-CHOP)

How is CLL managed initially?

Like follicular NHL it is an indolent disease so initial approach is watch and wait.

What are the indications to move on from watch and wait approach for CLL and treat it?

If treatment is indicated for CLL what is it?

1) Good prognosis - TP53 intact - regular immuno-chemo-therapy (systemic) 2) Bad prognosis - TP53 mutation (17q deletion) - TARGETED THERAPY REQUIRED eg IBRUTINIB,

Which markers are expressed by CLL?

CD19 (normal) CD5 (abnormal) CD23 (abnormal)

What is hairy cell leukaemia?

a subtype of CLL occurs in middle aged men fine hair like projections seen on tumour cells

Prognostic markers of CLL

1. Clinical - based on burden of malugannt cells- determined via staging (Rai or Binet) 2. Molecular - cd38: poor prognosis **- TP53 deletion - most important prognostic factor** **- VH mutation status (immunoglobulin variable heavy chain): UNMUTATED HAS WORSE PROGNOSIS (i..e pre germinal centre has worse prognosis)**

How can young patients be cured of CLL?

allogeneic stem cell transplant chimeric antigen receptor t cell therapy (CAR-T)

What is a significant cause of death in CLL?

infections (rmb they have low serum immunoglobulin levels) - this is why you need aggressive prophylaxis of infections

what is a long term side effect of rituximab?

can cause reactivation of hepatitis C virus

which is the most common lymphoma in the UK?

diffuse large b cell lymphoma

CLL not NHL \*\*because CLL is more common in this age group compared to lymphoma\*\*

This is due to underlying EBV underlying EBV can lead to recurrent tonsillitis EBV also causes increased proliferation of lymphocytes - lymphocytosis EBV also causes neutropaenia (mechanism unknown)