Case 20 - Haematological Malignancies Flashcards

1
Q

What is bilateral hilar lymphadenopathy on a chest X ray?

A

Bilateral enlargement of the lymph nodes of the pulmonary hila

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2
Q

What are the differentials for bilateral hilar lymphadenopathy?

A
Sarcoidosis 
Tuberculosis 
Lymphoma 
Glandular fever
Carcinoma 
Secondary metastases 
Lung cancer
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3
Q

What is a lymphoma?

A

Malignant proliferations of the lymphocytes which accumulate in the lymph nodes causing lymphadenopathy

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4
Q

Which organs can lymphomas infiltrate?

A

Any which are part of the reticuloendothelial system:

  • spleen
  • liver
  • bone marrow
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5
Q

What are the two histological types of lymphoma?

A

Hodgkin’s lymphoma (characterised by presence of Reid stenberg cells)

Non Hodgkin’s lymphoma

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6
Q

How does lymphoma typically present?

A

Painless lymphadenopathy
Painless, non tender ‘rubbery’ superficial lymph nodes (typically cervical, but also can be axillary or inguinal nodes)

Other symptoms can include night sweats, unintentional weight loss, fever, breathlessness and itching

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7
Q

What are the B symptoms of lymphoma?

A

Symptoms that are systemic

  • Fever >38 degrees
  • weight loss (over 10% is significant)
  • night sweats
  • pruritus (itching)
  • lethargy
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8
Q

How is lymphoma investigated?

A

Lymph node excision biopsy
Staging CT scan (or PET scan)
Chest X-ray
Bloods - FBC, blood film, ESR, LFT, LDH, urate, calcium

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9
Q

How is lymphoma staged?

A

The Ann Arbor system
Stage I - confined to single lymph node region
Stage II - involvement of two or more nodal areas on the same side of the diaphragm (north and south)
Stage III - involvement of nodes on both sides of diaphragm
Stage IV - spread to distant organs e/g, liver, spleen, bone marrow

Also given a letter code A or B if they have B (systemic symptoms) or not

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10
Q

What are the two peaks of incidence of age of Hodgkin’s lymphoma?

A

Age 15-30 years

Age >50 years

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11
Q

What are Reed-Sternberg cells?

A

Large cells that are multinucleated
Found in lymph node biopsies from patients with Hodgkin’s lymphoma
Cells typically have an “owls eye” appearance

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12
Q

What are the four classifications of Hodgkin’s lymphoma?

A
Lymphocyte rich (5%)
Nodular sclerosing (70%)
Mixed cellularity (25%)
Lymphocyte depleted (<1%)
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13
Q

What are the risk factors for developing haematological malignancies such as lymphoma?

A

Reduced immunity e.g, HIV, or post transplantation
Family history
If you have an autoimmune condition e.g, rheumatoid arthritis, lupus, Sjögren’s syndrome
Infection - associated between EBV and Hodgkin’s lymphoma
Previous treatment for cancers

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14
Q

What are the two different grading’s of non-hodgkins lymphoma ?

Give an example for each

A
Indolent (slow growing) - follicular lymphomas 
High grade (quick growing) - diffuse large B cell lymphomas
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15
Q

How do the different grades of non Hodgkin’s lymphoma react differently to chemotherapy?

A

Indolent (slow growing) - respond well to chemotherapy but are rarely cured

High grade (fast growing) - these are more likely to be cured with chemotherapy

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16
Q

What are the two main types of non-Hodgkin lymphoma and what type are they?

A

They are B cell line lymphomas:

Diffuse large B cell lymphoma - high grade lymphoma
Follicular lymphoma - indolent lymphoma

17
Q

How do lymphomas differ from lymphocytic leukaemia in their origins?

A

Lymphomas are solid tumours involving the lymph nodes

Lymphoid leukaemias originate in the bone marrow and therefore typically involve circulating lymphocytes

18
Q

What other type of cancer are patients with treated with chemotherapy and radiotherapy for Hodgkin’s lymphoma at risk of developing?

A

Lung cancer

This risk is increased x20 in smokers

19
Q

What is leukaemia?

A

Malignant neoplastic process involving one of the white blood cell lines (neutrophils, lymphocytes, monocytes)

20
Q

What are the 4 main types of leukaemia?

2 acute, 2 chronic

A

Acute lymphoblastic leukaemia (ALL)
Acute myeloblastic leukaemia (AML)

Chronic lymphocytic leukaemia (CLL)
Chronic myeloid leukaemia (CML)

21
Q

What does the finding of pupura signify?

A

Purpura is the appearance of non-blanching red spots on the skin. They are caused by small areas of bleeding under the skin. Usually purpura are <1cm

22
Q

What differentials should you consider if a young person presents with purpura

A

TTP - thrombotic thrombocytopenic purpura
HUS - haemolytic uraemic syndrome
Infections - such as meningococcal sepsis
Haematological malignancies - such as leukaemias

23
Q

What is multiple myeloma?

A

A type of bone marrow cancer

Malignancy arising from the plasma cells (the cells responsible for antibody production)

24
Q

What are the symptoms of multiple myeloma?

A

Bone pain (usually in the back) - due to osteolytic bone lesions
Bone fractures
Hypercalcemia
Anaemia and thrombocytopenia - proliferation of plasma cells results in reduced erythropoiesis (reduced production of platelets and RBCs)
Unusual bleeding

25
Q

What should you consider when you have persistent back pain in a patient over 50 years, associated with anaemia and hypercalcaemia?

A

Multiple myeloma

26
Q

What are bence jones proteins?

A

Monoclonal globulins that may be detected in urine in patients with multiple myeloma. They are considered significant if found in the context on other clinical features such as lytic bony lesions or anaemia

Bence jones proteins are present in approximately 60% of patients with multiple myeloma.

27
Q

Why do you get anaemia and thrombocytopenia in patients with multiple myeloma?

A

Malignant myeloma cells (plasma cells) pack the bone marrow
This leaves very little room for other cells to undergo erythropoesis
Patients therefore cannot develop RBCs, platelets and neutrophils
This results in anaemia, leukopenia and thrombocytopenia

28
Q

What are the side effects of chemotherapy?

A
Neutropenic sepsis - drop in platelets cause increase risk of infection 
Secondary malignancies 
Infertility 
Heart, liver, kidney and lung problems 
Alopecia (hair loss)
Fatigue 
Nausea and Vomiting 
Chemo brain (poor memory and concentration)
29
Q

What is the management plan for a patient presenting with neutropenic sepsis?

A

Isolate patient - as they are immunosupressed
Broad spectrum antibiotics
Blood cultures - to find out causative agent
IV fluids

30
Q

What type of anaemia is seen in multiple myeloma?

A

Normocytic normochromic anaemia

31
Q

What is the most common haematological malignancy in the UK?

A

Non-Hodgkin’s lymphoma

32
Q

What is the prognosis for multiple myeloma patients?

A

Myeloma is generally considered to be incurable

However with modern treatment 40-50% of patients will survive 5 years

33
Q

What are the most common forms of leukaemia in children and young adults?

A

Acute leukaemias

Acute myeloblastic leukaemia
Acute lymphoblastic leukamia

34
Q

What is the prognosis for Hodgkin’s lymphoma?

A

Over 80% are cured

Relapse after 5 years is rare

35
Q

What is the prognosis for diffuse Large B cell lymphoma?

A

50% of patients are cured

Relapses are rare after 5 years

36
Q

What is the prognosis for follicular lymphoma?

A

Responds well to treatment
But has recurrent relapses
Is incurable
Median survival 10 years

37
Q

What is the pneumonic for multiple myeloma?

A
CRAB:
C: hypercalcemia 
R: renal failure 
A: anaemia 
B: bone pain