Lymphoma Flashcards

1
Q

Lymphomas are 5th common malignancy in the western world.

What are lymphomas?

A

Lymphomas are caused by malignant proliferation of lymphocytes and malignancies of the lymphoid system.

Most commonly presents with peripheral lymphadenopathy and B symptoms i.e. weight loss, fever and night sweats.

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2
Q

What are the primary and secondary lymphoid organs?

A

Primary organs:
=> Bone marrow
=> Thymus

Secondary:
=> Spleen
=> Lymph nodes
=> Tonsils 
=> Adenoids
=> Appendix
=> Peyer's patch in small intestine
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3
Q

Lymphoma can be histologically divided into Hodgkin’s and Non-Hodgkin’s lymphoma.

What is Hodgkin lymphoma?

A

Hodgkin’s lymphoma’s characteristic = Reed-sternberg cells (mirror image nuclei)

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4
Q

What is the underlying aetiology of Hodgkin lymphoma?

A

Link between infectious mononucleosis and Hodgkin’s lymphoma

Patient’s with Hodgkin’s lymphoma have a high titre of EBV antibodies

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5
Q

What are the 2 subclassification of Hodgkin’s lymphoma?

A

Classic Hodgkin’s lymphoma:
=> Hallmark = Reed-sternberg cells
=> 90-95% of Hodgkin’s lymphoma

Nodular lymphocyte-predominant Hodgkin’s lymphoma (NLPHL)

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6
Q

Who does Hodgkin’s lymphoma affect?

A

i. Two peaks:

=> Young adults - Hodgkin’s lymphoma is the commonest malignancy in the 15-24 years

=> Elderly

ii. Men > Women

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7
Q

What are the symptoms of Hodgkin’s lymphoma?

A

=> Enlarged, rubbery, superficial lymph node

=> Painless and non-tender

=> 70% cervical lymph nodes

=> Fever

=> Weight loss

=> Night sweats

=> Pruritus

=> Lethargy

=> Mediastinal lymph node involvement = mass effect e.g. bronchial or SVC obstruction

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8
Q

What are the signs of Hodgkin’s lymphoma?

A

=> Lymphadenopathy

=> Cachexia

=> Anaemia

=> Spleno / hepatomegaly

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9
Q

What are the risk factors of Hodgkin’s lymphoma?

A

Affected sibling

EBV (epstein-barr virus)

SLE

Post-transplant

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10
Q

What investigations are carried out for Hodgkin’s lymphoma?

A
Tissue diagnosis:
=> Lymph node excision biopsy
=> Image guided needle biopsy
=> Laparotomy 
=> Mediastinoscopy 
Bloods:
=> FBC
=> Blood film (reed-sternberg cells)
=> ESR
=> LFT
=> LDH (high LDH bc released during cell turnover)
=> Urate
=> calcium 

*high ESR or low Hb = worse prognosis

Imaging:
=> CXR
=> CT/PET of thorax, abdo and pelvis

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11
Q

What staging system is used to determine treatment and prognosis of Hodgkin’s lymphoma?

Briefly describe the stages.

A

Ann Arbor system - by imaging ± bone marrow biopsy if B-symptoms or stage 3-4 disease.

Stage 1 : Confined to a single lymph node

Stage 2 : Involvement of >2 nodal areas on the same side of diaphragm

Stage 3 : Involvement of nodes on both sides of the diaphragm

Stage 4 : Spread beyond the lymph nodes e.g. liver / bone marrow

*Each stage is either
A = no systemic symptoms except pruritus

OR

B = B symptoms i.e. weight loss >10% in last 6 months, unexplained fever >38degrees or night sweats

B = worse disease

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12
Q

How do you treat Hodgkin’s lymphoma?

A
  1. Radiotherapy + chemotherapy

=> Short course for stages 1A & 2A

=> Long course for stages 2A - 4B

  1. More intensive regimen if poor prognosis or advanced disease
  2. High dose chemotherapy followed by stem cell transplantation if relapsed disease
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13
Q

What are the complications of treatment in Hodgkin’s lymphoma?

A

Radiotherapy side effects:
=> increased risk of 2nd malignancies i.e. lung, breast, melanoma, thyroid etc
=> increased risk of IHD, hypothyroidism, lung fibrosis

Chemotherapy side effects:
=> myelosuppression
=> nausea
=> alopecia
=> infection

General;
=> Infertility
=> AML
=> Non-Hodgkin’s lymphoma

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14
Q

What is the emergency presentation of Hodgkin’s lymphoma?

A

Infection, SVC obstruction => raised JVP, sensation of fullness in the head, dyspnoea, blackouts, facial oedema

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15
Q

What is Non-Hodgkin’s lymphoma?

A

Non-Hodgkin’s lymphoma - all lymphomas without Reed-sternberg cells:

=> 80% of B-cell origin (diffuse large B-cell lymphoma = commonest)

=> 20% of T-cell origin

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16
Q

What are the different types of Non-Hodgkin’s lymphoma?

A
1. B-cell lymphomas 
=> Diffuse large B-cell lymphoma 
=> Follicular lymphoma 
=> Burkitt lymphoma 
=> Mantle cell lymphoma
=> Lymphoplasmacytic lymphoma 
=> Marginal zone lymphoma 
=> Primary CNS lymphoma 
  1. Peripheral T-cell lymphoma
  2. Sézary syndrome (cutaneous lymphoma)
17
Q

What is the pathology underlying Non-Hodgkin’s lymphoma?

A
  1. Immunodeficiency / drugs or HIV causing immunodeficiency

2. EBV increases risk of Burkitt’s lymphoma, B-cell lymphoma in immunosuppression

18
Q

What are the signs and symptoms of Non-Hodgkin’s lymphoma?

A
  1. Superficial lymphadenopathy (75%)
  2. Extranodal disease (50%)
    i. Gut = commonest

=> Gastric MALT caused by H.pylori - may regress with eradication

=> Non-MALT gastric lymphomas due to diffuse B-cell lymphoma (60%)

ii. Skin = 2nd commonest

=> T-cell lymphoma

  1. Fever, night sweats, weight loss
  2. Pancytopenia from marrow involvement - anaemia, infection, bleeding (reduced platelets)
19
Q

What are the investigations for Non-Hodgkin’s lymphoma?

A

Bloods: FBC, U&E, LFT, LDH (raised = worse prognosis, reflects high cell turnover)

Marrow and node biopsy: for classification of lymphoma

Staging: Ann Arbor system + CT±PET of chest, abdo, pelvis

Cytology of any effusion

Lumbar puncture for CSF cytology if CNS symptoms

20
Q

High grade lymphomas = aggressive but often curable, rapidly enlarging lymphaedenopathy with systemic symptoms

  • Burkitt’s lymphoma
  • Diffuse B-cell lymphoma
  • Lymphoblastic lymphoma
A

Low grade lymphomas = indolent, often incurable, widely desseminated i.e.

  • follicular lymphoma,
  • marginal zone lymphoma/MALT,
  • lymphocytic lymphoma,
  • lymphoplasmacytoid lymphoma
21
Q

How do you treat Non-Hodgkin’s lymphoma?

A

Low grade:
=> radiotherapy curative in localised disease
=> chemotherapy in diffuse disease

High grade:
=> high dose chemotherapy

22
Q

What is the prognosis for Non-Hodgkin’s lymphoma?

A

Prognosis is worse if:

=> Age >60years

=> Systemic symptoms

=> Bulky disease i.e. abdo mass

=> Raised LDH

=> Disseminated disease

23
Q

Diffuse B-cell lymphoma

=> most common adult lymphoma worldwide

=> 2nd most common childhood lymphoma

A

Follicular lymphoma

=> 2nd most common Non-Hodgkin’s lymphoma

=> Rare in childhood

24
Q

Burkitt’s lymphoma

=> most rapidly proliferating lymphoma

=> most common childhood malignancy worldwide

=> male > female [3:1]

=> most common presentation = rapidly growing jaw tumour, abdominal mass assoc. with bone marrow involvement

A

Mantle cell lymphoma:

=> less common B-cell lymphoma

=> presents later in life

=> male > female [3:1]

25
Q

Marginal zone lymphoma

=> most common = Mucosa associated lymphoid tissue (MALT)

=> MALT of salivary gland linked to Sjögren’s syndrome

=> uncommon B-cell lymphoma assoc. with chronic inflammation / infection

A

Mycosis fungoides = most common cutaneous lymphoma ; confined to skin

=> rare leukaemic variant = Sézary syndrome

=> multiple erythematous lesions, plaques, tumours