Haematological Malignancy Flashcards

1
Q

How common are haematological cancers?

A

10% of all human cancers

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2
Q

What gender do haematological cancers generally tend to affect most?

A

Men

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3
Q

What leukaemia is most common in young children?

A

ALL - peaks 2-4y

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4
Q

What leukaemia is most common in adults?

A

AML

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5
Q

What is Hodgkin lymphoma associated with?

A

EBV (especially in older patients)

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6
Q

Describe the incidence of Hodgkin lymphoma

A

Peak in 18-35yos and peak in 70s

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7
Q

Describe the pathogenesis of haematological malignancy

A

Multistep process:
Genetic mutation to long lived cell that conveys survival advantage –> produces malignant clones –> malignant clones dominate the tissue (e.g. bone marrow/lymph nodes)

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8
Q

If the malignant clones start dominating the tissue what can occur?

A

Bone marrow failure (anaemia, thrombocytopenic bleeding, infection due to neutropenia)

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9
Q

If a mutation occurs in the hemopoietic stem cell causing leukaemia what is the cell called?

A

Leukaemia stem cell

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10
Q

What are the two most important properties of the hemopoietic stem cell?

A

Multipotential - can go down myeloid/lymphoid lineage

Self-renewing - one daughter cell will differentiate another will replace the hemopoietic stem cell

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11
Q

What cells are granulocytes?

A

Eosinophils, neutrophils, basophils, mast cells

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12
Q

What cells are included in the myeloid lineage?

A

Granulocytes, monocytes, platelets, erythrocytes

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13
Q

What cells are included in the lymphoid lineage?

A

B cells

T cells

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14
Q

In ALL, where does the mutation occur and what cells will accumulate?

A

Occurs in hemopoietic stem cell or early lymphoid progenitor leading to accumulation of lymphoblasts

Lymphoblasts retain their ability to proliferate rapidly but do not differentiate

Rapidly accumulation of these cells –> bone marrow failure (acute presentation)

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15
Q

In AML, where does the mutation occur and what cells will accumulate?

A

Hemopoietic stem cells or early myeloid progenitor cells

Proliferation occurs, differentiation blocked –> accumulation of early myeloid cells in bone marrow –> bone marrow failure & acute presentation

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16
Q

Where do lymphomas most commonly occur?

A

Mutations of lymphocytes in the germinal centre of the lymph nodes (as this is where there is the most genetic pressure)

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17
Q

Differentiate between lymphoma and leukaemia

A

Leukaemia - mostly in blood and bone marrow

Lymphoma - in lymph tissue mostly

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18
Q

What is the most common leukaemia?

A

Chronic lymphocytic leukaemia

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19
Q

How is CLL different when it presents in the lymph nodes?

A

If presents with swollen lymph nodes, biopsy of lymph nodes will show exact same morphology as if it was in the bone marrow
BUT it is known as small cell lymphocytic lymphoma instead

20
Q

What are the features of Burkitt lymphoma?

A

Rapidly growing, very aggressive and tends to present with a lump

21
Q

What are the acute leukaemias?

A

Acute lymphoblastic leukaemia

Acute myeloid leukaemia

22
Q

What are the chronic leukaemias?

A

Chronic myeloid leukaemia

Chronic lymphocytic leukaemia

23
Q

What are the malignant lymphomas?

A

Non-Hodgkin lymphoma

Hodgkin lymphoma

24
Q

What are the main differences between the acute and chronic leukaemias?

A

Acute: leukaemic cells do not differentiate; chronic: able to differentiate (not fully)

Acute: bone marrow failure, chronic: proliferation without bone marrow failure

Acute: acute presentation, rapidly fatal if untreated, chronic: survival for a few years

25
Q

What is the triad of bone marrow failure?

A
Anaemia 
Thrombocytopenic bleeding (purpura/mucosal membrane bleeding)
Infection (due to neutropenia, predominantly fungal and bacterial infections)
26
Q

What is the appearance of the bone marrow in acute leukaemia?

A

Absolutely packed with blasts

27
Q

What is key about the petechiae seen in bone marrow failure?

A

Non-blanching

28
Q

Aside from petechiae what other abnormal bleeding may you see in bone marrow failure?

A

Mucosal bleeding

Subscleral bleeding

29
Q

What infections might you see in bone marrow failure?

A
Staph aureus (e.g. tunnel infections, cellulitis), pseudomonas
Aspergillosus
30
Q

Describe the structure of a lymph node

A

Paracortex surrounding outside
Cortex is outer area, with inner medulla
In medulla there are B cell follicles composed of a marginal zone, mantle zone and germinal centre

31
Q

Where are B cells found in the lymph nodes?

A

Follicles

32
Q

Where are T cells found in the lymph nodes?

A

Paracortex

33
Q

Where are plasma cells found in the lymph nodes?

A

Medulla

34
Q

What occurs in the germinal centre?

A

B cells undergoing expansion and selection

35
Q

Where do B cells mature?

A

In the germinal centre of lymph nodes where they are forced to rearrange their genetic material to change into a plasma cell against a specific antigen

36
Q

Where do T cells mature?

A

Thymus

37
Q

What is the name of the process of genetic rearrangement that occurs in the germinal centre of lymph nodes?

A

Somatic hypermutation

38
Q

What are the different presentations of lymphomas?

A

Nodal disease (lymphadenopathy) occurs in HL 90% and NHL 60% of the time

Extranodal disease, e.g. in CNS

Systemic (B) symptoms

39
Q

What are the systemic B symptoms?

A

Fever, drenching night sweats, >10% loss of body wt, pruritus, fatigue

Indicative of a poorer prognosis

40
Q

What is important to remember when trying to palpate and check lymph nodes?

A

Not all lymph nodes can be felt, e.g. mediastinal lymph nodes

41
Q

What is the reason for localised and painful lymph nodes?

A

Bacterial infection

42
Q

What are reasons for localised and painless lymph nodes?

A

Rare infections (TB, scratch fever)
Metastatic cancer - HARD
Lymphoma - RUBBERY
Reactive

43
Q

What are reasons for generalised and painful lymph nodes?

A

Viral infections (EBV, CMV, hep, HIV)

44
Q

What are reasons for generalised and painless lymph nodes?

A
Lymphoma
Leukaemia
Connective tissue diseases
Sarcoidosis
Reactive
Drugs, e.g. phenytoin
45
Q

What are the clinical features of multiple myeloma?

A

Lytic lesions and bone pain (cytokines –> osteoclast induction)
Anaemia (of chronic dx)
Recurrent infections (neutropenia)
Renal failure (deposition of light chains)
Amyloidosis (light chain accumulation in tissues)
Bleeding tendency
Hyperviscosity syndrome