Fatigue and Neck Swelling Flashcards

1
Q

What are the two pathways that can result from haematopoiesis?

A

Myeloid and lymphoid

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2
Q

What does the term ‘blasts’ refer to?

A

Immature forms of cells

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3
Q

Where are blasts normally seen?

A

In small amounts in the bone marrow

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4
Q

What does the presence of blast cells in the peripheral blood suggest?

A

Haematological malignancy

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5
Q

When should a monospot test for infectious mononucleosis be carried out?

A

In patients <40 presenting with glandular fever type symptoms (fever, sore throat and lymphadenopathy +/- jaundice).

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6
Q

What are the criteria for haematological 2 week wait referrals for myeloma, non-Hodgkin’s and Hodgkin’s lymphoma? (3)

A

-Suspected myeloma —> if results of protein electrophoresis or a Bence-Jones protein urine test suggest myeloma.
-Non-Hodgkin’s lymphoma —> adults presenting with unexplained lymphadenopathy or splenomegaly; also take into account associated symptoms
-Hodgkin’s lymphoma —> adults presenting with unexplained lymphadenopathy; also take into account associated symptoms.

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7
Q

Which haematological malignancy is alcohol-induced lymph node pain characteristic of (although rare)?

A

Hodgkin’s lymphoma

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8
Q

How do malignant lymph nodes tend to feel on palpation? (3)

A

Hard, painless and adherent to surrounding tissues.

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9
Q

What are Reed-Sternberg cells?

A

Large cells that are either multi-nucleated cells, or have bilobed nuclei; found under light microscopy in lymph node biopsies from patients with Hodgkin’s lymphoma.

(Typically, these cells have an ‘owl’s eye’ appearance.)

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10
Q

What size lymph node is considered to be clinically relevant in adults? (2)

A

> 1cm
OR
1.5cm at neck level 2

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11
Q

What size lymph node is considered to be clinically relevant in children?

A

> 2cm

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12
Q

What are three common causes of inflammatory cervical lymph node enlargement?

A

-Bacterial/viral infection (local, i.e tonsils, teeth, ear, scalp)
-Tuberculosis (TB)
-HIV

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13
Q

What are the two basic types of malignant neck lymph nodes?

A

Metastatic - malignancy has spread from elsewhere in the body, most often the head/neck
Lymphoma

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14
Q

What is lymphoma?

A

A type of cancer that originates in lymphocytes (white blood cells).

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15
Q

What is the difference between Hodgkin’s and Non-Hodgkin’s Lymphoma?

A

Primary difference is type of lymphocyte affected:
Hodgkin’s Lymphoma - Reed-Sternberg cells are present
Non-Hodgkin’s Lymphoma - no Reed-Sternberg cells are detected

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16
Q

What is the difference between diffuse large B cell lymphoma and follicular lymphoma?

A

Both are types of non-Hodgkin’s lymphoma that affect B cells:
-Diffuse large B cell lymphoma - most common type of non-Hodgkin’s lymphoma (NHL); proliferation of highly malignant lymphocytes infiltrate and efface the architecture of lymph nodes. 50% cure rate.
-Follicular lymphoma - second most common type of NHL, where the architecture of lymph nodes is preserved; disease is prone to relapse and as such deemed ‘incurable’

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17
Q

Why is lactate dehydrogenase (LDH) important in non-Hodgkin’s lymphomas?

A

It is a very important prognostic marker - a high lactase dehydrogenase (LDH) suggests a poorer prognosis for survival rate.

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18
Q

What is rituximab?

A

An anti-lymphocyte monoclonal antibody treatment given to cause lysis of B lymphocytes in various conditions such as rheumatoid arthritis, lymphoma, polyangiitis and pemphigus vulgaris.

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19
Q

What is T cell lymphoma of the skin?

A

A form of non-Hodgkin’s lymphoma in which malignant T cells are initially localised to the skin with no evidence of extracutaneous disease at time of diagnosis; almost always presents dermatologically.

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20
Q

What is the difference between low grade (indolent) and high grade non-Hodgkin’s lymphomas?

A

-Low-grade = grow slowly and may not require treatment for long periods; likely to respond well to chemo but rarely cured (i.e follicular lymphoma)
-High-grade = grow quickly, frequently symptomatic; more likely to be completely cured with chemo (i.e diffuse large B-cell lymphomas, Burkitt lymphomas)

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21
Q

What is the Ann Arbor System of clinical lymphoma staging? (5)

A

A staging system which gives each stage of lymphoma a number, as described below, and a letter (A/B) to indicate whether or not the patient has systemic symptoms (such as weight loss, fevers or night sweats).

Stage 1: one group of lymph nodes affected
Stage 2: two or more groups of nodes affected, but lymphoma restricted to one side of diaphragm only.
Stage 3: lymphadenopathy evident on both sides (above and below) of diaphragm
Stage 4: lymphoma has spread beyond lymph nodes to other organs such as spleen, bone marrow, liver or lungs.

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22
Q

What is radiotherapy?

A

The use of ionising radiation to treat malignant disease; it preferentially treats dividing cells and can be targeted to include the tumour and avoid normal tissue.

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23
Q

What is purpura and what does it signify?

A

The appearance of non-blanching purple-red spots of the skin that signifies bleeding vessels near the surface; can also occur in the mucous membrane. Petechiae are usually <1cm in size.

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24
Q

What do petechial rashes result from? (4)

A

Areas of haemorrhage into the dermis; primary pathophysiological causes of petechiae are:
-Thrombocytopenia
-Platelet dysfunction
-Disorders of coagulation
-Loss of vascular integrity

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25
Q

How can purpura be divided into two types?

A

-Non-Thrombocytopenic purpura —> pathology doesn’t affect platelets, and patient has a normal platelet count
-Thrombocytopenic purpura —> pathology involves a lack of platelets due to abnormalities anywhere along the platelet life cycle.

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26
Q

What categories can causes of thrombocytopenic purpura be divided into? (3)

A

-Impaired platelet production
-Excessive platelet destruction
-Sequestration of platelets (in splenomegaly)

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27
Q

What is thrombotic thrombocytopenic purpura (TTP)?

A

A rare but serious condition in which there is a deficiency of ADAMTS13 (an enzyme that breaks down von Willebrand factor when no longer needed), resulting in numerous small blood clots forming throughout the body.

28
Q

What is immune thrombocytopenic purpura (ITP)?

A

An autoimmune condition in which the body produces antibodies against its own thrombocytes/platelets, destroying them and resulting in purpura.

29
Q

What is leukaemia?

A

A malignant neoplastic process involving one of the white blood cell lines (neutrophils, lymphocytes, monocytes etc).

30
Q

How is leukaemia classified? (2)

A

Depending on the cell line that is affected:
-Myeloid (neutrophils)
-Lymphocytic (lymphocytes)
Depending on degree of cell proliferation:
-Acute (peripheral blood film is dominated by immature cells (or blast cells)
-Chronic

31
Q

What is Acute Lymphocytic leukaemia (ALL)?

A

A malignant clonal disease that develops when a B/T-precursor-stage lymphoid progenitor cell becomes genetically altered through somatic changes and undergoes uncontrolled proliferation.

32
Q

Which type of leukaemia accounts for 80% of leukaemias in paediatric patients?

A

Acute Lymphocytic leukaemia (ALL)

33
Q

What is Acute Myeloid Leukaemia (AML)?

A

The clonal expansion of myeloid blasts in the bone marrow, peripheral blood, or extra-medullary tissues.

34
Q

What are the key diagnostic factors of Chronic Lymphocytic Leukaemia (CLL)? (4)

A

-Lymphadenopathy
-Splenomegaly (in around 50% cases)
-Shortness of breath
-Fatigue

35
Q

What are the possible symptoms of Chronic Myeloid Leukaemia (CML)? (6)

A

-Fever
-Chills
-Malaise
-Weight loss
-Abdominal discomfort
-Night sweats

36
Q

What proportion of Chronic Myeloid Leukaemia (CML) cases are asymptomatic?

A

Approximately 1/3

37
Q

What are the common signs of Chronic Myeloid Leukaemia (CML)? (2)

A

-Elevated white blood cell counts (almost all patients)
-Splenomegaly (around 75% patients)

38
Q

How is diagnosis of Chronic Myeloid Leukaemia (CML) confirmed? (2)

A

Presence of Philadelphia chromosome and/or the BCR-ABL1 transcripts in peripheral blood or bone marrow cells.

39
Q

What causes tumour lysis syndrome?

A

Chemotherapy can rapidly kill neoplastic cells leading to the release of intracellular ions and metabolic by-products into the systemic circulation.

40
Q

What are the classical symptoms of tumour lysis syndrome? (7)

A

-Abdominal pain
-Distension
-Nausea
-Vomiting
-Anorexia
-Seizures
-Altered mental state

41
Q

What causes neutropenic sepsis?

A

Chemotherapy can effect bone marrow, reducing the amount of neutrophils produced and putting the patient at increased risk of infection.

42
Q

How must neutropenic sepsis be immediately managed?

A

Early diagnosis and IV antibiotics within one hour, as well as supportive IV fluids and paracetamol as required.

43
Q

What is Filgrastim?

A

A recombinant human Granulocyte-Colony Stimulating Factor (rhG-CSF) that stimulates the production of neutrophils; it reduces the duration of neutropenia and neutropenic sepsis.

44
Q

What blood test results would make you worry about a malignant cause of back pain? (2)

A

Normochromic anaemia and hypercalcaemia

[Indicative of spinal metastases from unknown primary cancer, or multiple myeloma.]

45
Q

What are the symptoms/signs of hypercalcaemia? (7)

A

-Confusion
-Depression
-Polyuria
-Polydipsia
-Abdominal pain
-Nausea and vomiting
-Renal stones

[Or can be asymptomatic]

46
Q

What may an ECG show in hypercalcaemia? (3)

A

-Shortened QT interval
-Osborn waves (J waves) in severe cases
-Ventricular irritability and VF arrest (in extreme hypercalcaemia)

47
Q

What is initial management for patients with hypercalcaemia? (2)

A

IV fluids, delivered according to fluid status of patient
Stop any drugs which may cause hypercalcaemia

48
Q

What is pamidronate?

A

A bisphosphonates, used to treat hypercalcaemia.

49
Q

With what symptoms can multiple myeloma present? (8)

A

-Bone pain (especially backache)
-Pathological fractures
-Lethargy
-Anorexia
-Dehydration
-Recurrent infections
-Amyloidosis features (cardiac failure, nephrotic syndrome)
-Hypercalcaemia symptoms (confusion, nausea, constipation, polydipsia, polyuria)

50
Q

What is multiple myeloma?

A

A malignancy arising from plasma cells; proliferating nests of plasma cells may form deposits in bones, and in bone marrow proliferation of plasma cells may result in reduced erythropoiesis and reduced production of platelets (thereby causing anaemia and thrombocytopaenia).

51
Q

How is multiple myeloma diagnosed? (3)

A

-Bone marrow biopsy shows a proportion of monoclonal plasma cells of 10% or greater.
-Presence of monoclonal antibodies/paraproteins in serum OR urine.
-One or more of following:
Hypercalcaemia
Renal failure
Anaemia
Lytic lesions

52
Q

What are plasma cells?

A

A type of white blood cells that are normally responsible for antibody production, in response to an infection.

53
Q

What are the classical complications of myeloma? (4)

A

‘CRAB’:
-hyperCalcaemia
-Renal impairment
-Anaemia
-Bony lesions

54
Q

How can non-myeloma causes of hypercalcaemia be excluded?

A

A lab assay for intact parathyroid hormone (PTH), which should be suppressed in response to hypercalcaemia, and vitamin D levels. Some labs also offer PTH related peptide assays to exclude paraneoplastic hypercalcaemia.

[If PTH levels are appropriately suppressed, an alternative explanation such as myeloma must be considered.]

55
Q

What proportion of the bone marrow is made up of plasma cells in a healthy individual?

A

<5%

56
Q

What is myelofibrosis?

A

A disease in which the bone marrow becomes replaced by connective tissue via fibrosis.

57
Q

What is the difference between primary and secondary myelofibrosis?

A

Primary myelofibrosis - caused by a gene mutation within haematopoietic cells causing rapid division and maturation of cells into megakaryocytes, which in turn stimulate fibroblasts, increasing fibrosis.
Secondary myelofibrosis - can develop from conditions like polycythaemia vera and essential thrombocythaemia.

58
Q

What are the classical symptoms of myelofibrosis? (5)

A

-Bone pain
-Fatigue
-Itching
-Fever
-Weight loss

59
Q

What are the treatment options for myelofibrosis? (4)

A

-Erythropoietin (for anaemia)
-Blood transfusions (for pancytopenia)
-Ruxolitinib (to inhibit proliferative pathways and relieve symptoms)
-Haemopoietic stem cell transplantation (has potential for cure)

60
Q

What is polycythaemia vera?

A

A condition involving increased red blood cell count due to overproduction in the bone marrow, resulting from a mutation in haematopoietic stem cells.

61
Q

What proportion of the total blood volume is normally made up of erythrocytes?

A

Around 45%

62
Q

What condition arises in the spent phase of polycythaemia vera?

A

Myelofibrosis - the mutated cells begin to die out, causing scar tissue (fibrosis) to form.

63
Q

What are the classical symptoms of polycythaemia vera? (8)

A

-Fatigue
-Dizziness
-Increased sweating
-Redness in the face
-Blurred vision
-Itchiness (especially after a hot shower)
-Splenomegaly
-Gout and kidney stones

64
Q
A
65
Q

What are ‘B symptoms’ referred to in staging of lymphomas? (3)

A

-Greater than 10% weight loss over 6 months
-Night sweats
-Unexplained fever