Haem Malignancy- Lymphoma Flashcards

1
Q

What are Lymphomas?

A

Lymphomas are a group of cancers that affect the lymphocytes inside the lymphatic system.

These cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy).

Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. HL is a specific disease and non-Hodgkins lymphoma encompasses all the other lymphomas.

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2
Q

What are Lymphomas?

A

Lymphomas are a group of cancers that affect the lymphocytes inside the lymphatic system.

These cancerous cells proliferate within the lymph nodes and cause the lymph nodes to become abnormally large (lymphadenopathy).

Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. HL is a specific disease and non-Hodgkins lymphoma encompasses all the other lymphomas.

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3
Q

What is Hodgkin’s lymphoma?

A

a malignant proliferation of B lymphocytes characterised by the presence of the Reed-Sternberg cell.

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4
Q

Epideimiology of Hodgkins lymphoma?

A

Bimodal age distribution with peaks around aged 20 and 75 years.

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5
Q

Prognosis for Hodgkins lymphoma?

A

Overall- 75% of patients with HL survive for ten years or more.

Prognosis is better in the younger population than in the older population.
* < 40 yearshave a five-year survival rate of 95%
* > 70 years have a five-year survival rate of < 50%

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6
Q

What is the lymphatic system?

A
  • network of tissues and organs that play a key role in the immune system.
  • made up of: lymph nodes, lymphatic vessels, lymphatic organs and lymphatic fluid.
  • mutations in lymphocytes inside lymphoid tissues results in a lymphoma.
  • In HL - B lymphocytes, (derived from the germinal centres of lymphoid tissues), mutate and lead to large, multi-nucleated giant cells - ‘Reed-Sternberg’ cells and large, mono-nucleated cells called malignant ‘Hodgkin cells’.
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7
Q

How is Hodgkins lymphoma classified?

A

Nodular Sclerosing - most common

table probs overkill

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8
Q

Is Nodular lymphocyte-predominant Hodgkin lymphoma dangerous?

A
  • Nodular lymphocyte-predominant Hodgkin lymphoma is indolent, low grade - not much in common with classical HL
  • HOWEVER: there is a RISK it can TRANSFORM into a high grade (rapidly growing) non-Hodgkin lymphoma
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9
Q

What are some risk factors for Hodgkins lymphoma?

A
  • EBV / glandular fever
  • HIV
  • Immunosupression (also autoimmune drugs e.g. RA that require immunosupressive drugs)
  • Ciagarette smoking
  • Previous non-hodgkins lymphoma
  • First degree relative with Non-Hodgkins or chronic lymphocytic leukaemia
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10
Q

What would expect to hear in history of a patient with Hodgkin’s lymphoma?

A

MOST COMMON:
* painless, rubbery, enlarged lymph node/nodes, in the cervical or supraclavicular region.

B symptoms:
* fever >38°C
* drenching night sweats
* unintentional weight loss ( >10% in 6 months)
* Chest discomfort +/- cough or dyspnoea e.g. a mediastinal mass
* Abdominal pain: e.g. liver / spleen
* Alcohol-induced pain at nodal sites
* Pruritis
* Malaise
* Fatigue

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11
Q

What clinical examination would you do if suspect Hodgkin’s lymphoma? give findings too

A

DO a lymphoreticular system examination.

On examination:

  • Lymphadenopathy
  • Hepatomegaly
  • Splenomegaly
  • Superior vena cava (SVC) syndrome: a mediastinal mass may cause SVC obstruction
  • Paraneoplastic syndromes such as cerebellar degeneration, neuropathy or Guillain-Barré syndrome
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12
Q

Differencials for Hodgkins lymphoma?

A
  • Infectious mononucleosis
  • Non-Hodgkin lymphoma
  • Acquired immunodeficiency syndrome (AIDS)
  • Tuberculosis
  • Sarcoidosis
  • Leukaemia
  • Myeloma
  • Toxoplasmosis
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13
Q

Lab investigations for Hodgkins lymphoma?

A

FBC: platelets
* leukaemia, infectious mononucleosis and other causes of lymphadenopathy,
* WCC / CRP /
* Ca and bone profile - rule out myeloma
U&Es:
* baseline before treatment
LFTs:
* reduced albumin levels are associated with a poorer prognosis
LDH:
* increased levels are associated with a poorer prognosis
ESR:
* increased levels are associated with a poorer prognosis

Tests to exclude differential diagnoses:
* Monospot® test for infectious mononucleosis
* sputum culture for TB
* viral screen including HIV, hepatitis B virus (HBV) and hepatitis C virus (HCV) tests

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14
Q

Imaging for hodgkin’s lymphoma?

A

Chest X-ray:
* intrathoracic lymphadenopathy and mediastinal expansion
Contrast CT neck, chest, abdomen and pelvis:
* when pt first presents
(PET-CT):
* staging in cHL
* repeated during treatment to allow guided therapy according to response (aiming to minimise toxicity by reducing chemotherapy intensity when possible)

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15
Q

Specialist investigations for hodgkins lymphoma? and findings?

A
  • lymph node excision biopsy for diagnosis and classification - (US or CT guided)
  • Light microscopy -Reed-Sternberg cells - giant multinucleated cell. Hodgkin cells are giant malignant mononucleated cells surround RS cells

Immunocytochemistry
* CD15 adn Cd30 antigens are expressed on Reed-Sternberg cells

Bone marrow biopsy
* less frequently needed as PET CT can detect BM involvement

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16
Q

Staging system for Hodgkins lymphoma?

A

Ann Arbor staging system

17
Q

Prior to starting treatment for Hodgkins lymphoma what do patients need?

A

due to the potential SE of chemo and radiotherapy
* cardiac function testing
* pulmonary function testing
* reproductive counselling

Increased risk of opportunistic infections vaccinate:

  • Polyvalent pneumococcal vaccine
  • Influenza vaccine
  • Meningococcal group C conjugate vaccine
  • Haemophilus influenzae type b vaccine
18
Q

What factors indicate a poor prognosis in hodgkins lymphoma?

A
  • age > 45 years
  • stage IV disease
  • haemoglobin < 10.5 g/dl
  • lymphocyte count < 600/µl or < 8%
  • male
  • albumin < 40 g/l
  • white blood count > 15,000/µl
19
Q

treatment of hodgkins lymphoma?

A
  • chemotherapy is the mainstay of treatment. Two combinations may be used
  • ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine): considered the standard regime
  • BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone): alternative regime with better remission rates but higher toxicity
    -
  • radiotherapy
  • combined modality therapy (CMT)
  • chemotherapy followed by radiotherapy
  • hematopoietic cell transplantation
  • may be used for relapsed or refractory classic Hodgkin lymphoma
20
Q

Blood transfusion cautions in patient with hodgkins lymphoma?

A

If a transfusion of blood products is required, patients with or treated for Hodgkin lymphoma (at any stage of the disease) must only receive irradiated blood products. This is a lifelong requirement.

Irradiated blood products are used to reduce the risk of transfusion-associated graft-versus-host disease.

21
Q

Disease related complications of hodgkins lymphoma?

A

Hodgkin lymphoma causes immunosuppression.

The clonal expansion of B lymphocytes are abnormal and do not function properly.

Patients are at a particularly higher risk of infection if there is bone marrow involvement.

22
Q

Treatment related complications of hodgkins lymphoma?

A
  • Neutropenia:
  • neutropenic sepsis
  • Secondary solid tumours: e.g. lung, skin, breast and GI
  • Secondary leukaemias: AML
  • Subfertility: counsel before treatment
  • CVS disease: due to adriamycin/doxorubicin
  • Lung fibrosis: due to bleomycin and presents months to years after treatment
  • Endocrine dysfunction
  • Neuropathy
  • Nausea and vomiting
  • Hair loss
23
Q

What is non-hodgkins lymphoma?

A

Non-Hodgkin’s lymphoma is an umbrella term for a group of malignancies affecting the lymphoid system.

It is defined as all lymphomas without Reed-Sternberg cells.

24
Q

How many types of non-hodgkins lymphoma?

A
  • 30-60 types

classified according to:
* cell of origin (B-cell or T-cell)
or
* pathological grade (high-grade or low-grade).

reg said 60, quesmed says 30

25
Q

causes / associations of non hodgkins lymphoma?

A

commonly associated with viral infection / immunodeficiency.

  • H-pylori gastric MALT (mucosa-associated lymphoma tissue).
  • EBV with Burkitt’s lymphoma and AIDS-related CNS lymphoma.
  • Hep C virus with diffuse large B-cell lymphoma and splenic marginal zone lymphoma.

Other cause associations include:
* Immunodeficiency states e.g. HIV/AIDS and post-organ transplant.
* Autoimmune e.g. Sjogren’s syndrome and coeliac disease.

26
Q

Risk factors for Non-Hodgkin’s lymphoma?

A
  • Elderly
  • Caucasians
  • History of viral infection (specifically Epstein-Barr virus)
  • Family history
  • Certain chemical agents (pesticides, solvents)
  • History of chemotherapy or radiotherapy
  • Immunodeficiency (transplant, HIV, diabetes mellitus)
  • Autoimmune disease (SLE, Sjogren’s, coeliac disease)

pass med

27
Q

Epidemiology of Non-Hodgkin’s lymphoma?

A
  • Non-Hodgkin’s lymphoma is much more common than Hodgkin’s lymphoma
  • different subtypes affect different ages, but it typically affects the elderly with 1/3 of cases occurring in > 75 years of age
28
Q

Symptoms of Non-Hodgkin’s lymphoma ?

A
  • Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
  • Constitutional/B symptoms (fever, weight loss, night sweats, lethargy)
  • Extranodal Disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
29
Q

Symptoms of Non-Hodgkin’s lymphoma ?

A
  • Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
  • Constitutional/B symptoms (fever, weight loss, night sweats, lethargy)
  • Extranodal Disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
30
Q

Contrast hodgkins to non-hodgkins lymphoma in terms of clinical features?

A
  • Lymphadenopathy in Hodgkin’s lymphoma can experience alcohol-induced pain in the node
  • ‘B’ symptoms typically occur earlier in Hodgkin’s lymphoma and later in non-Hodgkin’s lymphoma
  • Extra-nodal disease is much more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma
  • Non hodgkins: lymphadenopathy is more likely to be symmetrical, at multiple sites, and spread discontinuously across nodal sites
31
Q

Signs / exam findings in non-hodgkins?

A
  • Signs of weight loss
  • Lymphadenopathy (typically in the cervical, axillary or inguinal region) non tender, firm
  • Palpable abdominal mass - hepatomegaly, splenomegaly, lymph nodes
  • Testicular mass
  • Fever
  • Extra nodal disease e.g. skin lesions in cutaneous T-cell lymphoma or neurological disease) is more common in non-Hodgkin’s lymphoma.
32
Q

Lab Investigations for non hodgkins lymphoma?

A

Lab:
* FBC and blood film (patient may have a normocytic anaemia and can help rule out other haematological malignancy such as leukaemia)
* ESR (useful as a prognostic indicator)
* LDH (a marker of cell turnover, useful as a prognostic indicator)
* (LFT’s if liver metastasis suspected)
* HIV test (often performed as this is a risk factor for non-Hodgkin’s lymphoma)

quesmed: blood film typically reveals nucleated red cells and left shift (the presence of early white blood cell precursors) - particularly in the high grade cancers. may also reveal circulating lymphoma cells (i.e. abnormal lymphocytes)

33
Q

other investigations for Non-Hodgkin’s lymphoma?

(did lab bloods earlier)

A
  • Excisional node biopsy is the diagnostic investigation of choice (certain subtypes will have a classical appearance on biopsy such as Burkitt’s lymphoma having a ‘starry sky’ appearance)
  • CT chest, abdomen and pelvis (to assess staging)
  • PET CT or bone marrow biopsy to look for bone involvement
  • LP if neurological symptoms
34
Q

Management of non-hodgkins lymphoma?

A

Management is dependent on the specific sub-type of non-Hodgkin’s lymphoma and will typically take the form of watchful waiting w/ follow up 3-4 months (incase transforms low - high grade)
* chemotherapy or radiotherapy.
* All patients get flu/pneumococcal vaccines
* neutropenia may require antibiotic prophylaxis

High grade most common treatment is R-CHOP:
* Rituximab- monoclonal antibody that targets CD20
* Cyclophosphamide
* Doxorubicin
* Vincristine
* Prednisolone

35
Q

Complications of non-hodgkins lymphoma?

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
36
Q

prognosis of non-hodgkins lymphoma?

A
  • Low-grade non-Hodgkin’s lymphoma has a better prognosis
  • High-grade non-Hodgkin’s lymphoma has a worse prognosis but a higher cure rate
37
Q

What are some examples of high and low grade non- hodgkins lymphoma?

A

High grade:
* Burkitts
* Diffuse B cell lymphoma

Low grade:
* Follicular
* mantel cell