Revision - Lymphoma

Question 1

What is one of the key features which distinguish most lymphomas from leukaemia?

What are the exceptions to this rule?

Answer 1

The malignant cells are MATURE lymphocytes, and they arise within sites OUTSIDE of the bone marrow (e.g. lymph nodes).

In contrast, leukaemia develops from immature blasts and arises within the bone marrow.

Exception –> lymphoblastic lymphomas (these develop from immature precursor lymphoblasts similarly to leukaemia).

Question 2

How is lymphoblastic lymphomas distinguished from lymphoblastic leukaemia?

Answer 2

By the degree of bone marrow infiltration by blasts:

<25% bone marrow involvement –> lymphoma

> 25% –> leukaemia

Question 3

What are lymphoblastic lymphomas treated the same as?

Answer 3

ALL

Question 4

What are B symptoms?

Answer 4

1) Fever

2) Drenching night sweats

3) Weight loss >10% in 6m

Question 5

What is Hodgkin’s lymphoma?

Answer 5

Uncontrolled proliferation of B lymphocytes.

Question 6

Pathophysiology of Hodgkin lymphoma?

Answer 6

B lymphocytes mutate which leads to the presence of multi nucleated giant cells (‘Reed Sternberg’ cells) and large, mono-nucleated cells (malignant ‘Hodgkin’ cells).

Question 7

What are the 2 main types of Hodgkin’s lymphoma?

Answer 7

1) Classical (95%)

2) Nodular lymphocyte-predominant HL (5%)

Question 8

What 4 subtypes can classical HL be further divided into?

Answer 8

1) Nodular sclerosis

2) Mixed cellularity

3) Lymphocyte-rich

4) Lymphocyte-depleted

Question 9

What is the most common type of classical HL?

Answer 9

Nodular sclerosis

Question 10

What infection is implicated in around 40% of HL cases?

Answer 10

EBV

Question 11

What are some risk factors for HL?

Answer 11

1) EBV infection

2) Immunosuppression e.g. organ transplant, immunosuppressant therapies

3) Autoimmune e.g. RA, SLE, sarcoidosis

4) FH (same-sex siblings of patients with Hodgkin’s lymphoma are 10x more likely to develop the condition)

5) HIV

6) Smoking

Question 12

What is the distinctive histological feature in Hodgkin’s lymphoma?

Answer 12

Reed-Sternberg cells

Question 13

Clinical features of Hodgkin’s lymphoma?

Answer 13

1) Lymphadenopathy

2) 2) B symptoms:
- fever >38
- night sweats
- unintentional weight loss of >10% over 6 months

3) Pel-Ebstein fever

4) Abdo pain (if abdominal lymphadenopathy is involved)

5) Pruritus

6) Clinical hepato/splenomegaly (rare)

7) Bone marrow involvement (5-8%)

Question 14

What lymph nodes are typically involved in HL?

(3)

Answer 14

1) Cervical
2) Supraclavicular
3) Mediastinal

Question 15

What is Pel-Ebstein fever?

Answer 15

A cyclical fever followed by periods of being afebrile for 1-2 weeks (rare)

Question 16

What are the NICE referral guidelines for Hodgkin’s lymphoma in adults?

Answer 16

2ww referral for any unexplained lymphadenopathy

Question 17

What are the NICE referral guidelines for Hodgkin’s lymphoma in children and young people?

Answer 17

Consider a very urgent referral (<48 hours) for specialist assessment for Hodgkin’s lymphoma in children and young people presenting with unexplained lymphadenopathy.

Question 18

Timeline of referral for unexplained lymphadenopathy in adults vs children & young adults?

Answer 18

Adults -> 2ww

Children & young adults -> <48h

Question 19

What is the investigation for definitive diagnosis of HL?

Answer 19

Lymph node excision biopsy

Question 20

What does a lymph node excision biopsy reveal in HL?

Answer 20

Reed-Sternberg cells (owl’s eye appearance)

Question 21

Is HL or non-HL more common?

Answer 21

Non-HL

Question 22

Which type of lymphoma is most associated with EBV?

Answer 22

HL

Question 23

What is the mainstay of treatment for HL?

Answer 23

Chemo +/- radiotherapy

Question 24

What staging system is used for HL?

Answer 24

Ann Arbor staging

Question 25

Describe stage I to IV of the Ann Arbor staging system

Answer 25

I - Involvement of one lymph-node region or lymphoid structure (e.g. spleen or thymus).

II - Two or more lymph node regions on the same side of the diaphragm.

III - Lymph nodes on both sides of the diaphragm.

IV - Involvement of extranodal site(s).

Question 26

Due to the increased risk of opportunistic infections following chemotherapy in lymphoma, what vaccines are patients offered?

Answer 26

1) Polyvalent pneumococcal vaccine

2) Influenza

3) Meningococcal group C conjugate vaccine

4) Haemophilus influenzae type b vaccine

Question 27

Regardless of stage, what is relapsed HL treated with?

Answer 27

High dose chemo followed by autologous stem cell transplant (ASCT).

Question 28

If a transfusion of blood products is required in HL, what products must patients receive?

Answer 28

irradiated blood products only –> to reduce the risk of transfusion-associated graft-versus-host disease.

Question 29

What is the most immediate and common complication arising from chemotherapy in HL?

Answer 29

Myelosuppression –> anaemia, thrombocytopenia, and neutropenia,

Question 30

Why can HL treatment cause cardiotoxicity?

Answer 30

Due to anthracycline-based regimens

Question 31

What 2ary cancers are long-term survivors of Hodgkin’s lymphoma at a significantly elevated risk of developing?

Answer 31

1) AML

2) Non-Hodgkin lymphomas

3) Solid tumours including breast and lung cancer

Question 32

Is non-HL more common in boys or girls?

Answer 32

Boys

Question 33

What are the majority of types of non-HL?

Answer 33

Of B-cell origin (e.g. Burkitt’s lymphoma)

Question 34

What is Burkitt’s lymphoma?

Answer 34

A type of non-HL (of B-cell origin)

Question 35

What is the main treatment for non-HL?

Answer 35

chemo

Question 36

What is seen on microscopy in Burkitt’s lymphoma?

Answer 36

‘starry sky’ appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells