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Yr 4 - Paediatrics > Lymphoma > Flashcards

Lymphoma Flashcards

1
Q

What is lymphoma?

A

A group of malignancies which arise within the lymphatic system, which includes lymph nodes, the spleen, the thymus and the bone marrow.

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2
Q

What are the 2 main types of lymphoma?

A

1) Hodgkin’s lymphoma

2) Non-Hodgkin’s lymphoma

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3
Q

Who is lymphoma more common in?

A

Teenagers & young adults

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4
Q

What does lymphoma result from?

A

Genetic alterations which trigger the abnormal proliferation of lymphocytes.

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5
Q

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

What are the exceptions to this rule?

A

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.

Exceptions –> ymphoblastic lymphomas (B-cell lymphoblastic lymphoma, and T-cell lymphoblastic lymphoma) develop from immature precursor lymphoblasts similarly to leukaemia.

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6
Q

How is lymphoblastic lymphomas distinguished from lymphoblastic leukaemia?

A

By the degree of bone marrow infiltration by blasts:

<25% bone marrow involvement –> lymphoma

> 25% –> leukaemia

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7
Q

What are lymphoblastic lymphomas treated the same as?

A

ALL

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8
Q

Lymphadenopathy in reactive lymphadenopathy vs lymphoma or metastatsis?

A

Reactive lymphadenopathy –> tender & mobile

Lymphoma or metastasis –> non-mobile, non-tender, B symptoms

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9
Q

What are B symptoms?

A
  • Fever
  • Drenching sweats
  • Unintentional 10% weight loss in 6 months
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10
Q

What is Hodgkin’s lymphoma?

A

A rare haematological malignancy caused by the uncontrolled proliferation of B-lymphocytes.

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11
Q

Pathophysiology of Hodgkin lymphoma?

A

Occurs when B lymphocytes mutate and lead to the presence of large, multi-nucleated giant cells called ‘Reed-Sternberg’ cells and large, mono-nucleated cells called malignant ‘Hodgkin cells’.

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12
Q

What are the 2 main types of Hodgkin lymphoma (HL)?

A

1) Classical HL (95%)

2) Nodular lymphocyte-predominant HL (5%)

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13
Q

What 4 subtypes can classical HL be further divided into?

A

1) Nodular sclerosis

2) Mixed cellularity

3) Lymphocyte-rich

4) Lymphocyte-depleted

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14
Q

What is the most common type of classical HL?

A

Nodular sclerosis

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15
Q

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

A

Epstein-Barr virus (EBV)

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16
Q

What are some risk factors for HL?

A

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

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17
Q

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

A

Reed-Sternberg cells

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18
Q

What are Reed-Sternberg cells?

A

Post-germinal B lymphocytes that have undergone transformations and are intended for apoptosis.

However, the escape apoptosis which leads to unchecked proliferation.

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19
Q

Clinical features of Hodgkin’s lymphoma?

A

1) Lymphadenopathy

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%)

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20
Q

What is the most common symptom of HL?

A

A painless, rubbery, enlarged lymph node/nodes, typically in the cervical or supraclavicular region.

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21
Q

What lymph nodes are typically involved in HL?

A

Cervical, supraclavicular or mediastinal nodes.

22
Q

How can mediastinal lymphadenopathy present in HL?

A

Can can compress the airway and lead to dyspnoea, chest pain, and dry cough.

It may also cause SVCO.

23
Q

What is Pel-Ebstein fever?

A

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

24
Q

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

A

Referral under 2WW.

  • Unexplained lymphadenopathy.

When considering referral, take into account any associated symptoms, particularly fever, night sweats, shortness of breath, pruritus, weight loss or alcohol‑induced lymph node pain.

25
Q

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

A

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

26
Q

What exam findings may be presebt in HL?

A

1) Lymphadenopathy

2) Hepatomegaly

3) Splenomegaly

4) Superior vena cava (SVC) syndrome: a mediastinal mass may cause SVC obstruction

5) Paraneoplastic syndromes such as cerebellar degeneration, neuropathy or Guillain-Barré syndrome

27
Q

Lab investigations in HL?

A

1) FBC –> often anaemic, lymphopenic and thrombocytopenic, which can be associated with bone marrow involvement.

2) U&Es

3) LFTs

4) LDH

5) ESR

28
Q

What is the investigation for definitive diagnosis of HL?

A

Lymph node excision biopsy

29
Q

What does a lymph node excision biopsy reveal in HL?

A

Reed-Sternberg cells (owl’s eye appearance)

30
Q

What investigation is recommended for both staging and assessing treatment response post-chemotherapy in HL?

A

PET sscan

31
Q

What is the key differential for HL?

A

Non-Hodgkin’s lymphoma

32
Q

Is HL or non-HL more common?

A

Non-HL

33
Q

Typical age of HL vs non-HL?

A

HL –> Bimodal age group - 15-24 and >60

Non-HL –> 60s

34
Q

Which type of lymphoma is most associated with EBV?

A

HL

35
Q

Location of lymphadenopathy in HL vs non-HL?

A

HL –> Lymphadenopathy usually limited to the upper body (neck, chest or axilla)

Non-HL –> Lymphadenopathy occurs anywhere in the body but sometimes also in the groin and abdomen

36
Q

What is the mainstay of treatment for HL?

A

Chemotherapy alone or in combination with radiotherapy

37
Q

What staging is used for HL?

A

Ann Arbor staging system

38
Q

Describe stage I to IV of the Ann Arbor staging system

A

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) beyond that designated E.

39
Q

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

A

1) Polyvalent pneumococcal vaccine

2) Influenza

3) Meningococcal group C conjugate vaccine

4) Haemophilus influenzae type b vaccine

40
Q

Management of early-stage HL (stage IA, IB, IIA)?

A

One or more cycles of combination chemotherapy plus radiotherapy.

41
Q

Management of advanced stage HL (stage IIB or above)?

A

uUsually treated with a more intensive chemotherapy course; often without radiotherapy unless a particularly large mass is present.

42
Q

What are the 2 most commonly used chemotherapy combination regimes in Hodgkin lymphoma?

A

ABVD: Doxorubicin (used to be called Adriamycin®), Bleomycin, Vinblastine and Dacarbazine

BEACOPP: Bleomycin, Etoposide, Doxorubicin (Adriamycin®), Cyclophosphamide, Vincristine (Oncovin®), Procarbazine, Prednisolone

43
Q

Regardless of stage, what is relapsed HL treated with?

A

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

44
Q

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

A

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

45
Q

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

A

Myelosuppression –> anaemia, thrombocytopenia, and neutropenia,

46
Q

Why can HL treatment cause cardiotoxicity?

A

Due to anthracycline-based regimens

47
Q

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

A
  • AML
  • Non-Hodgkin lymphomas
  • Solid tumours including breast and lung cancer
48
Q

Is non-HL more common in boys or girls?

A

Boys

49
Q

What are the majority of types of non-HL?

A

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

50
Q

Lymphadenopathy in non-HL?

A

More rapidly growing bulky lymphadenopathy.

51
Q

What is the main treatment for non-HL?

A

Chemotherapy

52
Q
A

Yr 4 - Paediatrics (78 decks)

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