haematological malignancy Flashcards

1
Q

epidemiology of haematological cancers

A

account for ~11% of all cancers

occur in all age groups, incl children

adult males are more commonly affected than females

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2
Q

what is the commonest group of blood cancers

A

non Hodgkin’s lymphoma

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3
Q

what age does non Hodgkins lymphoma occur at

A

disease of middle and older age - 40/50s, majority of pts in 60s/70s

can occur in children and young adults

M>F

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4
Q

what age does Hodgkins lymphoma occur at

A

peak incidence between 18 and 35 and then again 60-80s

5-6/100 000 p/a

M>F except in younger people where F>M

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5
Q

pathogenesis of haematological malignancies

A

multi-step process

acquired genetic alterations in a long lived cell (e.g. stem cell)

proliferative/survival advantage to that mutated cell –> produces a malignant clone –> grows to dominate the tissue

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6
Q

where are multipotentential haematopoietic stem cells found

A

bone marrow

born with a fixed amount of these cells and we keep them throughout our life

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7
Q

special characteristics of multipotentential haematopoietic stem cells

A

when they divide they have the ability to self-renew - when the cell divides, one of the two daughter cells is retained

ability to give rise to every single cellular blood product (pluripotent)

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8
Q

growth of a malignant clone of cells in the development of leukaemia

A

genetic mutation in haematopoietic stem cells

clone of mutated cell - asymptomatic to begin with

additional mutations occur and clones continue

disease takes off, growth advantage and dominates normal cells

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9
Q

lineage of bone marrow malignancies

A

myeloid:
red cells, platelets, granulocytes, monocytes

lymphoid:
B and T cell

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10
Q

what happens to the cells in acute myeloid leukaemia

A

mutational events lead to growth advantage so daughter cells of stem cells can proliferate

differentiation is blocked so they grow but they can’t ‘grow up’ - remain as immature, useless cells (blast cells)

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11
Q

what condition is this

A

acute leukaemia

blocking the bone marrow

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12
Q

what happens to the cells in myeloproliferative disorders e.g. chronic myeloid leukaemia

A

growth advantage AND ability to differentiate

accumulate more useful cells than you need

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13
Q

what condition is this

A

chronic myeloid leukaemia

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14
Q

what happens to the growth of cells in acute lymphoblastic leukaemia

A

mutations in the stem cell population

proliferation but block mutation

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15
Q

what condition is this

A

acute lymphoblastic leukaemia

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16
Q

where do the mutations occur in lymphoid malignancies

A

sometime in the haemopoietic stem cell but sometimes in the more mature cells

17
Q

groups of diseases in lymphoid malignancies

A

chronic lymphocytic leukaemia

lymphomas - Hodgkin’s and non Hodgkin’s

multiple myeloma

18
Q

difference between Hodgkin’s and non Hodgkins

A

Hodgkin’s - distortions in the LNs in the neck and under the arm

non-Hodgkin’s - evenly distributed throughout the body, slower growing

19
Q

what can be seen here

A

bone marrow in multiple myeloma showing plasmacytosis - lots of protein production in the cells, eccentric nu and marked Golgi zone (plasma cells therefore increased antibody production)

multiple myeloma - malignancy of plasma cells

20
Q

leukaemia vs lymphoma

A

descriptive terms that describe the distribution of the disease in the body

disease predominantly in blood and bone marrow –> leukaemia

predominantly in LNs or other organs –> lymphoma

21
Q

acute lymphoblastic leukaemia vs lymphoblastic lymphoma

A

ALLA - most commonly develops in bone marrow and blood

young adult, near normal blood count, large central chest LN mass - same disease, now called LL due to presentation

22
Q

common presentation of chronic lymphocytic leukaemia vs small cell lymphocytic lymphoma

A

older person, feels well, abnormal white count - appearance of CLL cells

older person, large swollen LNs - same disease on biopsy - SCLL

23
Q

Burkitt’s lymphoma leukaemia presentation

A

very aggressive lymphoma

presents as lymphoma w/ swollen lymph nodes, esp in H+N (children in Africa)

24
Q

structure of the lymph node

A

medulla - where the lymphatic channels and blood vessels come into

germinal centres - where B cells grow up after release from bone marrow, exposed to antigens in LNs

25
Q

B cell maturation

A

lympocytes are released from bone marrow and then go into the germinal centre of LN

here they are exposed to antigens by APCs

B cell can then present immunoglobulin to bind the antigen and attack it

if it does this successfully it emerges from the germinal centre; if not the cells are deprived of survival factors and die

26
Q

what happens when the genetic rearrangement in B cell maturation goes wrong

A

B cell lymphoma develops - commonest type of lymphoma

27
Q

what does localised and painful lymphadenopathy indicate

A

bacterial infection in draining site

28
Q

what does localised and painless lymphadenopathy indicate

A

rare infections: cat scratch fever, TB
metastatic carcinoma from draining site - hard feel
lymphoma - rubbery feel
reactive, no cause identifies

29
Q

what does generalised and painful/tender lymphadenopathy indicate

A
viral infections: 
EBV
CMV
hepatitis
HIV
30
Q

what does generalised and painless lymphadenopathy indicate

A
lymphoma
leukaemia
connective tissue diseases, sarcoidosis
reactive, no cause identified
drugs
31
Q

lymphoma presentations

A

nodal disease (lymphadenopathy):

  • > 90% HL present w/ nodal disease
  • ~60% NHL present w/ purely nodal disease

extranodal disease:
- ~40% NHL present w/ an extranodal component, w/ or w/o nodal involvement

systemic symptoms:
- fever, drenching sweats, weight loss, pruritis, fatigue

32
Q

what are the major groups of haematological malignancies

A

acute leukaemias:

  • acute lymphoblastic leukaemia (ALL)
  • acute myeloid leukaemia (AML)

chronic leukaemias:

  • chronic myeloid leukaemia (CML)
  • chronic lymphocytic leukaemia (CLL)

malignant lymphomas

  • non-Hodgkin lymphoma (NHL)
  • Hodgkin lymphoma (HL)

multiple myeloma

myelodysplastic syndromes (MDS)

chronic myeloproliferative diseases (biologically malignant)