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Year 5 Path Charis > Haem 6 - Lymphoma 2 – CLL and lymphoproliferative disorders > Flashcards

Haem 6 - Lymphoma 2 – CLL and lymphoproliferative disorders Flashcards

1
Q

Hodgkin’s lymphoma epidemiology

A

• M > F
o Bimodal age incidence – 20-29yo (women, NS subtype, most common), >60yo (smaller peak)
o Women get a sclerosing sub-type more often

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2
Q

Hodgkin’s lymphoma symptoms

A

o Asymmetrical painless progressive lymphadenopathy
 Becomes painful after alcohol consumption
 Obstructive symptoms  Extrinsic compression of any tube (ureter, bile duct, large blood vessel, bowel, trachea, oesophagus)

o Infiltrate/impair an organ system
 Skin rash
 Ocular+ CNS
 Liver failure

o Recurrent infections

o B symptoms
 Drenching night sweats
 Weight loss >10% in 6 months unintentional

o Pruritis
o Coincidental e.g. FBC, Imaging

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3
Q

Hodgkin’s lymphoma histology

A

 Cells stain with CD15 + CD30

 Reed-Sternberg cell – bi-nucleate/multinucleate (owl eyed) cell on a background of lymphocytes + reactive cells

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4
Q

HL classification Hodgkin’s lymphoma

A

Classical HL
 Nodular sclerosing 80% - Good prognosis
 Mixed cellularity 17% - Good prognosis
 Lymphocyte rich (rare) – Good prognosis
 Lymphocyte depleted (rare) – Poor Prognosis

o Nodular Lymphocyte predominant HL 5% - disorder of the elderly, multiple recurrences

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5
Q

How do you stage HL? Hodgkin’s lymphoma

A

FDG-PET
CT scan
BM biopsy
+/-Lumbar puncture

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6
Q

Describe the Ann Arbor staging system

A

 Stage 1 – one LN region (LN region can include the spleen)
 Stage 2 - >2 LN regions on the same side of the diaphragm
 Stage 3 - >2 LN regions on the opposite sides of the diaphragm
 Stage 4 – extranodal sites (liver, BM)

 A – no constitutional symptoms
 B – constitutional symptoms

Constitutional symptoms
Fever
Unexplained Weight loss >10% in 6 months
Night sweats

https://www.lls.org/sites/default/files/National/USA/Image/get_support/HL_Staging_Diagram.JPG

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7
Q

classical HL

Nodular sclerosing subtype sx, epidemiology

A 
Most common (80%) 
F > M (20-29yo)

Neck nodes + mediastinal mass (may be massive and compress SVC or trachea)
May have B symptoms

Spreads contiguously
Needs tissue diagnosis

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8
Q

Treatment of classical HL

A 
CHEMO
All patients get chemotherapy - ABVD
Adriamycin 
Bleomycin
Vinblastine
Dacarbazine

2-6 cycles
4 weekly intervals

interim PET CT after 2 cycles to assess response to treatment
End of treatment PET CT to assess need for any radiotherapy

Preserves fertility

RADIO
Often given after chemo

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9
Q

Risks of consolidation radiotherapy in the treatment of HL

A
  • Risk of damage to normal tissues (collateral damage)
  • Associated with increased risk of breast/lung/skin cancer, leukaemia/myelodysplasia
  • Very high risk of breast cancer in women
  • Damage to normal tissue

 Combined modality (chemo + radio) – greatest risk of 2o malignancy

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10
Q

Which chemotherapy agents are used to treat classical HL?

Side effects?

A

ABVD

Adriamycin
Bleomycin
Vinblastine
DTIC Dacarbazine

Adriamycin - cardiomyopathy
Bleomycin - pulmonary fibrosis

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11
Q

Relapse of HL mx

A

Salvage chemo

Autologous HSCT

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12
Q

Autologous vs allogenic SCT where is it used

A

Autologous - stem cells from peripheral blood, BM, umbilical cord blood
MM, lymphoma
Not in leukaemias

Allogenic
Leukaemias

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13
Q

Deaths in cHL (classical hodgkin’s lymphoma)are due to

A

highly curable disease, excellent prognosis
80% will be long-term survivors

10% die from relapse of HL (first 10 years)
10% die from treatment complications (after 10 years)

~5 years, patients are more likely to die of a secondary malignancy or cardiovascular complications (complications of curative treatment)

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14
Q

Why is it important to test for Hep B in HL and NHL?

A

o Many patients are asymptomatic carriers of hepatitis B
o NHL patients may be given treatments that deplete B cells
o This may cure the lymphoma, but the patient might then present with fulminant liver failure because you have reactivated any asymptomatic hepatitis B

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15
Q

The two commonest types of NHL

A

Diffuse large B cell lymphoma

Follicular lymphoma

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16
Q

Which lymphomas respond better to chemotherapy; indolent or very aggressive?

A

Very aggressive (curable)

indolent lymphomas are less treatable - incurable but long remissions

exception - enteropathy associated T cell lymphoma
quick clinical course but not responsive to treatment

17
Q

How are very aggressive lymphomas treated?

A

Like leukaemia

18
Q

Enteropathy associated T cell lymphoma mx

A

Responds poorly to chemo, generally fatal

aim is to prevent - strict adherence to gluten free diet

19
Q

Most likely subtype of cHL in

young women
eldelry

A

young women - nodular sclerosing

elderly - lymphocyte rich

both have a good prognosis

20
Q

NHL Monitoring only appropriate for asymptomatic small volume disease in this lymphoma sub type

A

Follicular lymphoma

21
Q

Gastric MALT mx -

A

indolent lymphoma but needs abx to prevent complications

22
Q

Which cells are proliferating in CLL?

A

Mature B cells

lots of monoclonal lymphocytes

23
Q

CLL clinical features

A 
Asymptomatic
Painless lymphadenopathy
BM failure
B symptoms
Hepatosplenomegaly
Associated with autoimmunity (Evan's syndrome) - AIHA, ITP
24
Q

CLL laboratory features

Bloods
Blood film
flow cytometry

A 
FBC
Massive lymphocytosis (>200x10^9/L)
Normocytic anaemia
thrombocytopenia
neutropenia
low serum immunoglobulin

Blood film
Smear cells (smear CLLs)
Normocytic normochromic anaemia
B cells - CD19 +, CD5+

flow cytometry to confirm monoclonal population

25
Q

What risk is there with CLL/SLL?

A

Can transform to the aggressive diffuse large B cell lymphoma via Richter transformation

26
Q

How can CLL be staged clinically?

A

 Rai or Binet staging

	CLL IPI score 
•	Age >60y
•	Serum LDH > normal
•	Performance status 2-4
•	Stage III or IV
•	>1 extra-nodal site
27
Q

Cell based prognostic factors in CLL

A 
•	IgH mutation status 
unmutated = bad
•	CLL FISH cytogenetic panel
•	tp53 mutation status
•	chr 17p deletion (tp53) [loss of tp53 tumour suppressor gene]
28
Q

Worst case scenario in CLL

A
  • Binet stage C
  • IgH unmutated
  • 17p del
  • p53 mutated
29
Q

CLL prognosis

A

Initially 5-10 years good health until progression to a 2-3 year terminal phase

Rapid progression to death within 2-3 years

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

30
Q

Binet staging

A 
Binet stage
•	A
          <3 groups of enlarged lymph nodes
          High WBC
          Usually no treatment required

• B
>3 groups of enlarged lymph nodes

• C
Anaemia (<100g/l) or thrombocytopenia (<100x10^9/l)

31
Q

Rai staging

A

0 - lymphocytosis only

I - lymphadenopathy

II - hepatosplenomegaly +/- lymphadenopathy

III - Hb <110 g/l

IV - plt <100x10^9/l

32
Q

Indication for Ig replacement therapy in CLL

A

Recurrent infections + IgG <5 g/l

33
Q

Vaccination in CLL

A

annual influenza
pneumococcal
covid19
avoid live vaccines

34
Q

Indications to treat CLL

A

Watch and wait unless

 Progressive lymphocytosis
• >50% increase over 2 months
• Count doubling < 6 months

 Progressive bone marrow failure (Hb < 100; Platelets < 100; Neutrophils < 1)

 Massive or progressive lymphadenopathy/splenomegaly

 Systemic symptoms (B symptoms)

 Autoimmune cytopenias
• Treat with immunosuppression not chemotherapy

35
Q

How to treat CLL in young people vs eldelry

A

Elderly
Watch and wait
Chemo to establish remission

Young patients
Allogenic SCT

36
Q

CLL targeted therapy

A

BCR kinase inhibitors
Ibrutinib (BTK)
- irreversibly binds to BTK
- for refractory CLL P53 mutation
- initially high lymphocyte count which then falls
- associated with
Bruton’s x-linked hypogammaglobulinemia (=Abnormal BTK (B cell tyrosine kinase) gene – mutation in B cell tyrosine kinase
• Pre-B cells cannot develop into mature B cell  Absence of mature B cells  no antibody production)

Idelasibe (PI3K)

BCL-2 inhibitors
Venetoclax
- for refractory CLL P53 mutation
(bcl-2 is an anti-apoptotic protein therefore ventoclax permits apoptosis of CLL cells)

37
Q

CLL therapy if it transforms to high grade B cell NHL through Richter transformation (diffuse large B cell NHL)

A

treat as high grade lymphoma

R-CHOP (6-8 cycles)

Rituximab (anti-CD10 monoclonal antibody) 
Cyclophosphamide
Adriamycin
Vincristine
Prednisolone
38
Q

Which is the biggest risk when initiating Venetoclax

A

 Risk of tumour lysis syndrome at start of therapy (potentially fatal) – rapid cell death over a 24h period, release of intracellular electrolytes, mediators

Year 5 Path Charis (44 decks)

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