Lymphoma (Hodgkin's and non-Hodgkin's) Flashcards

1
Q

Define lymphoma.

A

Neoplasms of the lymphoid cells, originating in lymph nodes, lymphoid organs, skin or rarely “anywhere”.

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2
Q

Define Hodgkin’s lymphoma.

A

An uncommon haematological malignancy arising from mature B cells. It is characterised by the presence of Hodgkin’s cells and Reed-Sternberg cells.

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3
Q

Define non-Hodgkin’s lymphoma.

A

Malignancy of lymphoid cells originating in LNs without Reed Sternberg cells and consisting of 85% B cell, 14% T cell and NK cell forms, ranging from indolent to aggressive disease and referred to as low, intermediate and high grades.

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4
Q

What is the epidemiology of Hodgkin’s lymphoma?

A

Bimodal age distribution peaks at 20-30yrs and >50yrs

More common in males

Annual European incidence is 2-5 /100,000

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5
Q

What is the epidemiology of NHL?

A

Incidence increases with age

More common in males

More common in West

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6
Q

What is the aetiology of Hodgkin’s lymphoma? What are the risk factors?

A

50% of cases associated with EBV but pathogenesis unknown

Unknown aetiologies

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7
Q

What is the aetiology of NHL? What are the risk factors?

A

Associated with EBV, HIV, SLE, Sjogren’s, HBV, HCV

Oncogenic viruses can introduce foreign genes and cause NHL.

Radiotherapy, immunosuppressive agents, chemotherapy.

  • EBV –> Burkitt’s lymphoma and AIDs associated lymphomas
  • HTLV-1 –> ATLL
  • HHV-8 –> body-cavity-based lymphomas
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8
Q

What is the typical presentation of Hodgkin’s lymphoma?

A
  • Painless enlarging mass (most often in the neck, occasionally in axilla or groin)
  • May become painful after alcohol ingestion
  • Skin excoriations
  • Signs of intrathoracic disease (e.g. pleural effusion, SVC obstruction)
  • B symptoms - fever >38 (if cyclical referred to as Pel–Ebstein fever), night sweats, weight loss (>10% in last 6 months)
  • Examination: non-tender lymphadenopathy, splenomegaly +/- hepatomegaly
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9
Q

What is the typical presentation of NHL?

A
  • Painless enlarging mass - neck/axilla/groin -
  • Systemic symptoms - fever, night sweats, weight loss>10% body weight, symptoms of hypercalcaemia.
  • Symptoms related to organ involvement - extranodal disease more common than in cHL
    • Gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
  • Establish performance status of patient

Signs:

  • Examination: painless, rubbery lump. Oropharyngeal (Waldeyer’s ring lymph nodes) involvement.
  • Skin rashes - mycosis fungoides (well defined indurated scaly plaque-like lesions with raised ulcerated nodules caused by cutaneous T-cell lymphoma) and Sezary’s syndrome
  • Abdominal mass
  • Hepatosplenomegaly
  • Signs of bone marrow involvement - anaemia, infections, purpura.
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10
Q

What does presence of B symptoms imply?

A

Poor prognosis -

  • weight loss > 10% in last 6 months
  • fever > 38ºC
  • night sweats
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11
Q

How do you diagnose Hodgkin’s lymphoma?

A

Lymph node biopsy under microscopy = Reed Sternberg cells aka Owl’s eyes

Bloods:

  • FBC - anaemia of chronic disease, leukocytosis, raised neutrophils, raised eosinophils. Lymphopaenia with advanced disease.
  • ESR/CRP - raised
  • LFT -raised LDH, raised transaminases

Imaging:

  • CXR
  • CT thorax, abdo, pelvis
  • Gallium scan
  • PET scan

Bone marrow aspirate and trephine biopsy - involvement seen only in very advanced disease

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12
Q

What staging system is used for Hodgkin’s lymphoma?

A

Ann Arbour staging

  • I Single lymph node region.
  • II Two or more lymph node regions on one side of the diaphragm.
  • III Lymph node regions involved on both sides of diaphragm.
  • IV Extranodal involvement (liver or bone marrow).
  • A Absence.
  • B Presence of B symptoms.
  • E Localized extranodal extension.
  • S Involvement of spleen.
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13
Q

What are the histological subtypes of Hodgkin’s lymphoma?

A
  1. Nodular sclerosing (70%);
  2. Mixed cellularity (20%);
  3. Lymphocyte predominant (5%);
  4. Lymphocyte depleted (5%).
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14
Q

What cells are pathognomonic of Hodgkin’s lymphoma?

A

The Reed–Sternberg cell is pathognomonic. It is a large cell with abundant pale cytoplasm and
two or more oval lobulated nuclei containing prominent ‘owl-eye’ eosinophilic nucleoli
(can appear as lacunar or ‘popcorn’ cells).

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15
Q

What is the name for the cyclical fever sometimes seen in Hodgkin’s lymphoma?

A

Pel-Ebstein fever

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16
Q

What investigations would you do for NHL?

A

Bloods:

  • FBC - anaemia, neutropenia, thrombocytopenia (if BM involved)
  • U&E, uric acid
  • CRP and ESR - raised
  • LDH -raised
  • LFTS - raised transaminases with liver involvement
  • Ca2+ - may be high
  • HIV, HBV, HCV serology

Blood film - Hodgkin cells (aka Reed-Sternberg cells)

Lymph node biopsy (DIAGNOSTIC) and…

  • Histology - evaluation of architecture and cells
  • Immunophenotyping - CD markers and expression of abnormal proteins; clonality of B cells
  • Cytogenetics - FISH or PCR for chromosome translocations

Imaging - CXR, CT thorax, abdomen, pelvis, CT plus PET (in extranodal involvement)

Staging - as for Hodgkin’s (Ann Arbour for removed lymph node)

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17
Q

What is the most common subtype of lymphoma?

A

Diffuse large B-cell lymphoma is the most common type of lymphoma.

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18
Q

What is the difference between lymphoma and leukaemia?

A

Leukemia and lymphoma are both forms of blood cancer. The main difference is that leukemia affects the blood and bone marrow, while lymphomas tend to affect the lymph nodes.

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19
Q

Which subtype of NHL has a starry sky appearance under microscopy? How does it present?

A
  • Burkitt’s lymphoma
  • Usually in an African child
  • Large LN in the jaw which is fast growing
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20
Q

Which lymphoma is more common: HL or NHL?

A

NHL

Hodgkin’s makes up 15% of lymphomas

21
Q

What are smear cells characteristic of? How are these related to lymphoma?

A

CLL - may progress to high grade lymphoma if CLL gains extra mutations

22
Q

What CD is present on immature vs mature B cells?

A

Immature = CD5

Mature = CD19

23
Q

Name 3 low grade/indolent B cell NHLs.

A
  • Follicular
  • CLL a.k.a. small lymphocytic lymphoma
  • Mantle zone lymphoma (MALT)
24
Q

Name 2 high grade B cell NHLs.

A
  • DLBCL
  • Mantle zone lymphoma
25
Q

Name an aggressive NHL.

A
  • Burkitt’s
26
Q

Name 4 HLs.

A

Classical

  • Nodular sclerosing
  • Mixed cellularity
  • Lymphocyte-depleted
  • Lymphocyte-rich

Nodular lymphocyte predominant

27
Q

Describe the spread of HL vs NHL

A

HL spreads locally

NHL is found at multiple distant sites

28
Q

What weight loss is a significant B symptom in Hodgkin’s lymphoma staging?

A

10% in 6 months

29
Q

Which HL has the best prognosis?

A

Nodular sclerosing - best
Lymphocyte depleted - poor prognosis

30
Q

Which HL subtype is most common in young women?

A

Nodular sclerosing cHL - usually affects neck and mediastinum

31
Q

What is ABVD therapy? What are its advantages over R-CHOP in follicular NHL?

A

A - adriamycin

B - bleomycin

V - vinblastine

D- DTIC

Very effective and maintains fertility. Better than R-CHOP in this way. But causes cardiomyopathy and pulmonary fibrosis.

32
Q

What are the types of marginal zone lymphomas?

A

MZL of stomach - H pylori associated gastric MALToma

MZL of salivary glands - Sjogren’s syndrome

MZL of thyroid - Hashimoto’s thyroiditis

Small intestine EATL - coeliac disease associated

33
Q

Which NHL is antibiotic responsive ?

A

Gastric MALT

34
Q

What is the most common NHL?

A

DLBCL (30%)

Follicular (22%)

35
Q

Can you cure aggressive or indolent lymphomas?

A

Aggressive curable
Indolent not curable

36
Q

What blood test is part of the prognostic index for DLBCL?

A

LDH

37
Q

What is the survival in follicular lymphoma?

A

12-15yrs

38
Q

Which type of lymphoma is more common?

A

NHL (80%)

HL (20%)

39
Q

Which NHL is linked to viral infection?

A

HTLV1 virus → Adult T cell leukaemia lymphoma (ATLL)

40
Q

What are the common types of NHL B cell type?

A
  • Common B-cell NHL include:
    • Low-grade:
      • Follicular lymphoma
      • Small lymphocytic lymphoma/chronic lymphocytic leukaemia (CLL)
      • Marginal zone lymphoma (MALT)
    • High-grade:
      • Diffuse large B cell lymphoma
      • Mantle zone lymphoma
    • Aggressive:
      • Burkitt’s lymphoma
41
Q

Name 2 types of T cell lymphomas.

A
  • Anaplastic large cell
  • ATLL
  • EATL
  • Mycosis fungoides (cutaneous T cell lymphoma)
42
Q

How are aggressive lymphomas treated?

A

Like acute leukaemia

43
Q

What are the types of management approach for HL?

A
  • Chemotherapy
  • Radiotherapy
  • Combination of the two
  • Haematopoietic stem cell transplant
44
Q

What are the types of management approach for NHL?

A
  • watchful waiting
  • chemotherapy
  • radiotherapy
45
Q

What is the treatment regimen for DLBCL?

A

R-CHOP (rituximab-cyclophosphamide-adriamycin-vincristine-prednsolone) with curative aim

46
Q

What is the management of cHL?

A

Chemotherapy (essential for cure) +/- Radiotherapy

  • ABVD 2-6 cycles (depends:stage&interim response)
  • PET CT

Interim: After x2 cycles, response assessment

End of Treatment: Guides need for additional radiotherapy

Relapse {salvage chemotherapy}

  • High dose chemotherapy + Autologous PB stem cell transplant as support
47
Q

What is the prognosis in HL?

A
  • Overall 80% are long term survivors (can we improve)
  • 10% die from relapse of HL (first 10 years)
  • 10% die from treatment complications (after 10 years)
48
Q

What are the complications of NHL?

A
  • Bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
  • Superior vena cava obstruction
  • Metastasis
  • Spinal cord compression
  • Complications related to treatment e.g. Side effects of chemotherapy
49
Q

What are the complications of HL?

A
  • most patients now achieve long-term survival free of Hodgkin’s lymphoma with modern therapy
  • complications of treatment are therefore more of an issue for these patients
  • secondary malignancies are a risk, in particular solid tumours: breast and lung