Concepts in malignant haematology Flashcards

1
Q

monocyte morphology

A

kidney shaped nucleus

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2
Q

how can we identify normal progenitors/stem cells?

A

immunophenotyping

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3
Q

Malignant haemopoiesis is usually characterised by :

A
  • increased numbers of abnormal & dysfunctional cells
  • loss of normal activity
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4
Q

acute leukaemia is characterised by:

A

Proliferation of abnormal progenitors
with block in differentiation/maturation
(e.g. Acute Myeloid Leukaemia)

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5
Q

Chronic myeloproliferative disorders/neoplasm (MPN)

A

Proliferation of abnormal progenitors,
but NO differentiation/maturation block
(e.g. Chronic Myeloid Leukaemia)

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6
Q

are haematological malignancies usually due to hereditary or acquired genetic mutations?

A

acquired
usually multiple ‘hits’ - contribution from recurrent cytogenetic abnormalities (e.g. deletions, chromosomal translocations etc)

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7
Q

is normal haemopoiesis polyclonal or monoclonal

A

polyclonal

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8
Q

what is a clone

A

population of cells derived from a single parent cell

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9
Q

is malignant haemopoiesis polyclonal or monoclonal

A

monoclonal

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10
Q

age of presentation of acute lymphoblastic leukaemia (ALL)

A

2-5

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11
Q

why are there so many different subtypes of haematological malignancies (4 points)

A
  • Cells at different developmental stages can undergo neoplastic transformation
  • Involvement of different anatomical regions unique to the cell-type, either at the point of origin, or after migration
  • Different clinicopathological characteristics (clinical and biological behaviour)
  • Patterns of antigen expression/signature (suggestive of developmental stage) –immunophenotyping/immunohistochemistry have a critical role in diagnosis
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12
Q

Classifying cancers of the haemopoietic and lymphoid systems (4 points)

A

Site

Lineage (myeloid or lymphoid)

Stage of development/histology (more primitive compartment or less primitive compartment)

Preservation of differentiation/maturation

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13
Q

what language/terms do we use to differentiate between more and less primitive stage of development in haem malignancies

A

“blastic” - more primitive
“high grade”, “low grade”, “cytic” - less primitive

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14
Q

which are more aggressive - acute or chronic leukaemias

A

acute

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15
Q

Features of histological aggression:

A

large cells with high nuclear-cytoplasmic ratio, prominent nucleoli, rapid proliferation

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16
Q

Acute leukaemias present (more commonly than chronic leukaemias) with failure of normal blood cell production, true or false

A

true

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17
Q

Acute lymphoblastic leukaemia:

A

blood/marrow involving primitive, lymphoid malignancy

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18
Q

Acute myeloid leukaemia:

A

blood/marrow involving, primitive, myeloid malignancy

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19
Q

Chronic lymphocytic leukaemia:

A

blood/marrow involving, less primitive, lymphoid malignancy

20
Q

High grade B/T cell (non Hodgkin) lymphoma:

A

nodal, lymphoid malignancy, less primitive, clinically aggressive

21
Q

Low grade B cell (non Hodgkin) lymphoma:

A

nodal, lymphoid malignancy, less primitive, clinically less aggressive

22
Q

Hodgkin lymphoma:

A

nodal, lymphoid malignancy, less primitive, less aggressive

23
Q

Myeloma

A

plasma cell malignancy
usually, not exclusively, in the bone marrow

24
Q

Chronic myeloproliferative neoplasms disorders:

A

primitive, myeloid compartment, maturation preserved (e.g. chronic myeloid leukaemia)

25
Q

does Chronic lymphocytic leukaemia involve blood or lymph nodes

A

often both!

26
Q

In advanced lymphoma, malignant cells from the lymph node can enter circulation and infiltrate the bone marrow – ‘_______ phase’ of lymphoma

A

leukaemic

27
Q

what does red pulp of the spleen contain?

A

sinusoids and cords

28
Q

what are sinusoids in the spleen?

A

a form of blood channel usually described as a large, irregular capillary, having a discontinuous lining of endothelium, with little or no adventitia

29
Q

sinusoids (in spleen) are fenestrated - true or false

A

true

30
Q

sinusoids are lined by ____________ cells

A

epithelial

31
Q

sinusoids are supported by hoops of __________

A

reticulin

32
Q

what cells do splenic cords contain?

A

macrophages, and some fibroblasts and cells in transit (RBC, WBC, PC and some CD8+T cells

33
Q

what do splenic cords do

A

provide the organ structure through reticulin and fibrils. The cords also contain a reservoir of monocytes to aid in wound healing. Splenic cords lead to splenic sinuses where macrophages respond to antigens and filter abnormal or aging erythrocytes out of blood flow.

34
Q

function of the spleen

A

filters the blood.

35
Q

how much of the spleen does the red pulp make up

A

3/4 to 4/5 of the spleen tissue

36
Q

in the spleen, which type of pulp comprises the peri-arteriolar lymphoid sheath (PALS)

A

white pulp

37
Q

how does antigen reach white pulp in the spleen

A

via the blood

38
Q

what is hyposplenism most commonly from?

A

splenectomy

39
Q

causes of hyposplenism

A

splenectomy
coeliac disease
sickle cell disease
sarcoidosis

40
Q

what are howell-jolly bodies?

A

indicate hyposplenism (they are Basophilic nuclear remnants, often seen with decreased splenic function)

41
Q

splenomegaly causes

A
  • infection e.g. EBV, malaria, TB etc
  • congestion - portal
  • haematological diseases
  • inflammatory conditions
  • storage diseases
  • miscellaneous
42
Q

portal congestion causes of splenomegaly

A

hepatic cirrhosis
portal/splenic vein thrombosis
cardiac failure

43
Q

haematological diseases that can cause splenomegaly

A
  • lymphoma/leukaemia - pattern of disease and immunophenotype
  • haemolytic anaemia
  • myeloproliferative disorders
44
Q

inflammatory conditions that can cause splenomegaly

A
  • rheumatoid arthritis
  • SLE
45
Q

storage diseases that can cause splenomegaly

A
  • Gaucher’s
  • Neimann-Pick disease
46
Q

miscellaneous causes of splenomegaly

A
  • amyloid
  • tumours (primary and metastatic)
  • cysts
47
Q

features of splenic enlargement

A
  • dragging sensation in LUQ
  • discomfort with eating
  • pain if infarction