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Haematology and Immunology > Intro to Haematological Malignancies > Flashcards

Intro to Haematological Malignancies Flashcards

1
Q

Who does haematological malignancies affect?

A

All age groups, even children

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2
Q

Which gender of adult gets haematological malignancies more?

A

M > F

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3
Q

Pathology of haematological malignancies

A

Multi step process
Acquired genetic alterations to a long lived cell
Proliferative/survival advantage to that mutated cell (have to have a proliferative/survival advantage over the other cells to give it a malignant phenotype)
This produces a malignant clone
The malignant clone grows to dominate the tissue (e.g. bone marrow or lymph nodes)

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4
Q

Drivers of haematopoiesis

A

Multipotential haematopoietic stem cells

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5
Q

Features of multipotential haematopoietic stem cells

A

Can self renew

multipotential i.e. can produce all types of cells

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6
Q

Definition of haematopoiesis

A

Refers to the commitment and differentiation processes that lead to the formation of all blood cells from haematopoietic stem cells

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7
Q

Myeloid lineage of malignancies are called what?

A

Myeloid malignancies

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8
Q

Types of cells of myeloid malignancies

A

Red cells
Platelets
Granulocytes
Monocytes

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9
Q

Lymphoid lineage of malignancies are called what?

A

Lymphoid malignancies

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10
Q

Types of cells of lymphoid malignancies

A

B cells

T cells

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11
Q

What is the commonest malignancy of childhood?

A

Acute lymphoblastic leukaemia

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12
Q

Pathology of development of AML (acute myeloid leukaemia)

A

Genetic mutation in the stem cells
Early accumulate of myeloid progenitor cells which keep proliferating without maturing or differentiating -> bone marrow cells -> AML
so the bone marrow is stuffed with useless cells
No N cells in the blood

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13
Q

Pathology of the development of ALL (acute lymphoblastic leukaemia)

A

Genetic mutation in the stem cells
Ongoing proliferation at a slightly higher rate and an increase in differentiation
Accumulation of more N cells (red cells, neutrophils etc) in the blood
ALL

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14
Q

How do mature lymphoid malignancies (e.g. Hodgkin / non Hodgkin lymphoma) come about?

A

Mutations occur in a later place e.g. bone marrow, lymph node after already having ALL
Most likely occur in the germinal cell of the lymph node

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15
Q

Different mutations in the same stem cell compartment lead to what?

A

Different phenotypes

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16
Q

AML can lead to what?

A

Myeloproliferative disorders

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17
Q

What is the difference between leukaemia and lymphoma?

A

The distribution of the disease

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18
Q

Bone marrow malignancy is described as what?

A

Leukaemia

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19
Q

Lymphoma is found in what?

A

Lymph tissue or

Solid organs

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20
Q

Commonest form of leukaemia is what?

A

Chronic lymphocytic leukaemia

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21
Q

Features of chronic lymphocytic leukaemia

A

Perfectly well

Elderly usually

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22
Q

Features of Burkitt’s lymphoma / leukaemia

A

Most aggressive
commonly presents with lumps
Can affect young adults / children
Can be both lymphoma and leukaemia as it can present with both

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23
Q

Acute leukaemias

A

Acute lymphoblastic leukaemia (ALL)

Acute myeloid leukaemia (AML)

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24
Q

Chronic leukaemia s

A

Chronic myeloid leukaemias (CML)

Chronic lymphoid leukaemia (CLL)

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25
Q

The malignant lymphomas

A 
Non-Hodgkin Lymphoma (NHL)
Hodgkin Lymphoma (HL)
26
Q

What does MDS stand for?

A

Myelodysplastic syndromes

27
Q

Acute vs chronic leukaemia name is related to what?

A

The pace of the disease

28
Q

Pathology of acute leukaemia

A

Leukaemic cells do not differentiate
Bone marrow failure over a short period of time
Rapidly fatal if untreated
- being well to very unwell to dead within weeks

29
Q

Is acute leukaemia potentially curable?

A

Yes

30
Q

Clinical features of acute leukaemia

A 
Bone marrow failure
- anaemia
- thrombocytopenic bleeding (purpura and mucosal membrane bleeding)
- infection because of neutropenia (predominately bacterial and fungal) as cant make puss so infections spread easier 
Bleeding
- Petechial 
- GI 
- eye
31
Q

What is the triad of symptoms of bone marrow failure?

A

Anaemia
Thrombocytopenic bleeding (mucosal, purpura)
Infection (because of neutropenia)

32
Q

Pathology of chronic leukaemia

A

Leukaemic cells retain ability to differentiate
Proliferation without bone marrow failure
Very white blood

33
Q

Survival of chronic leukaemia

A

Survival for a few years

Quite well for a long time

34
Q

Is chronic leukaemia potentially curable?

A

Yes, with modern therapy

35
Q

Bone marrow (aplasia) can fail for a number of reasons…..

A

Malignancies
other conditions
chemotherapy

36
Q

Structure of a lymph node

A 
Inner medulla
Outer cortex 
Follicles dotted in lymph node
- germinal centre (B cells mature here)
- mantle zone (around germinal centre)
- marginal zone (around mantle zone)
37
Q

Where do B cells mature in the lymph node after coming from the bone marrow?

A

Germinal centre

38
Q

Lymphomas commonly develop in what cells?

A

Mutations of the cells when in the germinal centre

39
Q

Presentation of lymphoma

A 
Nodal disease - lymphadenopathy 
Extra-nodal disease 
Systemic symptoms 
- fever - B symptom
- drenching sweats - B symptom 
- loss of weight (10% of weight) - B symptom 
- pruritic
- fatigue
40
Q

What are the B symptoms?

A

Fever
drenching sweats
Loss of weight

41
Q

What do people with B symptoms indicate?

A

A less favourable prognosis and may need more treatment

42
Q

Nodal disease HL vs NHL

A 
HL = > 90% present with nodal disease
NHL = approx. 60% present with purely nodal disease
43
Q

If lymphadenopathy is localised and painful - what does this indicate?

A

Bacterial infection in draining site

44
Q

If lymphadenopathy is localised and painless, what does this indicate?

A

Rare infections, catch scratch fever, TB
Metastatic carcinoma from draining site - HARD
Lymphoma - RUBBERY
reactive, no cause identified

45
Q

If lymphadenopathy is generalised and painful/tender, what does this indicate?

A 
Viral infections
EBV
CMV 
Hepatitis
HIV
46
Q

If lymphadenopathy is generalised and painless, what does this indicate?

A 
Lymphoma
Leukaemia 
Connective tissue diseases, sarcoidosis 
Reactive, no cause identified 
Drugs
47
Q

What about a lymph node indicates a metastatic carcinoma from a draining site?

A

HARD

48
Q

What about a lymph node indicates lymphoma?

A

RUBBERY

49
Q

What is multiple myeloma?

A

Malignancy of the plasma cells in the bone marrow

50
Q

What does multiple myeloma produce?

A

An abnormal immunoglobulin/antibody into the blood

51
Q

Presentation of multiple myeloma

A

Bone pain
Lytic lesions (destruction of skeleton by activation of osteoclasts - NOT sclerotic)
Anaemia
- renal failure
- anaemia of chronic disease
Recurrent infections
Renal failure (an immunoglobulin blocking the renal tubule)
Amyloidosis (deposition of chains in other tissues e.g. kidneys)
Bleeding tendency
Hyper viscosity syndrome (malignant cells producing huge amounts of paraproteins)

52
Q

Causes of thrombocytosis

A 
infections
post surgery / trauma 
malignancy 
iron deficiency 
inflammation - IBD / RA
primary myeloproliferative disorder
53
Q

Presentation of myelofibrosis

A 
Splenomegaly (can be massive)
Cytopenia symptoms
Spleen symptoms 
Weight loss
Extreme tiredness 
Leukoerythroblastic blood film, teardrop red cells 
Marrow fibrosis - reticulin stain
54
Q

Mutations common in myelofibrosis

A

JAK2
CALR
MPL

55
Q

What happens in myelofibrosis?

A

The bone marrow is replaced by fibrous (scar) tissue

56
Q

Possible causes of lymphocytosis

A 
Viral infection (e.g. EBV, HIV)
other infections e.g. TB, syphilis
Vasculitis 
Acute lymphoblastic leukaemia
Chronic lymphocytic leukaemia 
Lymphoma
57
Q

Treatment of neutropenia (if risk factors)

A

Filgrastim (as brings up neutrophil count more quickly in those at high risk)

58
Q

What is filgrastim?

A

Granulocyte colony stimulating factor

59
Q

Examples of patients at high risk for febrile neutropenia

A

Elderly
Specific malignancies (e.g. NHL, ALL)
Previous neutropenic episodes
Those receiving combination chemo and radio

60
Q

What is checked before each round of chemo and why?

A

Neutrophil levels

As if neutropenia, increased risk of developing an infection or neutropenic sepsis

Haematology and Immunology (18 decks)

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