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GLA Y3S1 > Haematology > Flashcards

Haematology Flashcards

1
Q

where are red blood cells made in the adults

A

bone marrow (vertebrae, ribs, sternum, skull, sacrum, pelvis and ends of the femurs)

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1
Q

where are red blood cells made in a 0-2 month old foetus

A

yolk sac

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2
Q

where are red blood cells made in a 2-7 month old foetus

A

liver, spleen

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3
Q

where are red blood cells made in a 5-9 old foetus

A

bone marrow

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4
Q

where are red blood cells made in infants

A

bone marrow (all bones)

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5
Q

what do pluripotent stem cells differentiate into

A

myeloid stem cells and lymphoid stem cells

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6
Q

where are haemopoietic stem cells found

A
  • in the bone marrow
  • in the peripheral blood after treatment with G-CSF
  • umbilical cord blood
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7
Q

what are the three fate decisions made by a HSC

A
  • self renewal (creating an identical copy)
  • apoptosis
  • differentiation (maturation and specialisation)
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8
Q

what are the three types of control for stem cell fate in terms of division

A
  1. symmetrical division = contraction of stem cell numbers (reducing them)
  2. asymmetrical division = maintenance of stem cell numbers
  3. symmetrical division = expansion of stem cell numbers
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9
Q

stroma

A

the bone marrow microenvironment that supports the developing haemopoietic cell

  • this is a rich environment for growth and development of stem cells
  • stroll cells supported by an extracellular matrix
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10
Q

what are some of the supporting cells of the stroma and their supporting proteins

A

cells:
- macrophages
- fibroblasts
- endothelial cells
- fat cells
- reticulum cells

proteins:
- fibronectin
- haemonectin
- collagen
- proteoglycans
- laminin

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11
Q

what is a trephine

A

the instrument used to perform a bone marrow biopsy

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12
Q

what are the principles of leukaemogenesis

A
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13
Q

when are haematological malignancies and pre-malignant conditions termed “clonal”

A

fi they arise from a single ancestral cell

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14
Q

what are myeloproliferative disorders

A

these are clonal disorders of haemopoiesis leading to increased numbers of one or more mature blood progeny

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15
Q

Myelodysplasic syndromes (MDS)

A

these are characterised by dysplasia and ineffective haemopoiesis in more than one of the myeloid series (RED cells PLATELETS and WHITE cells)

often secondary to previous chemotherapy or radiotherapy

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16
Q

autologous transplant

A

known as an autograft and uses the patients own blood stem cells

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17
Q

what is an allograft

A

an allogeneric transplant which the stem cells come from a donor

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18
Q

synergic transplant

A

a transplant between identical twins

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19
Q

allogenic transplant

A

HLA identical so between brother and sister or two sisters and two brothers

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20
Q

haplotype identical

A

a half matched family member which is usually a parent or a half matched sibling

21
Q

volunteer unrelated (VUD)

A

also known as matched unrelated (MUD)

22
Q

what are the indications for an autologous stem cell transplant

A
  • relapsed Hodgkins disease
  • non-hodgkins lymphoma
  • myeloma
23
Q

what are the advantages of umbilical blood transplants

A

more rapidly available that VUD
less rigorous matching to patient type as the patient immune system is more naive

24
Q

disadvantages of umbilical cord transplant

A

small amount - adults will often require a double cord transplant

slower engraftment
if relapse they cannot go back for DLI

25
Q

indications ofr an allogenic transplant

A

acute and chronic leukemias
relapsed lymphoma
aplastic anaemia
hereditary disorders

26
Q

what are the issues with stem cell transplants

A

limited donor availability
mortality of around 10-50% depending on risk factors
immunosuppression
infertility in both sexes
risk of cataract formation
hypothyroidism with dry eyes and mouth
risk of 2nd malignancy
risk of osteoporosis/avascular necrosis
replapse

27
Q

what are the four things needed for normal red cell production

A

drive - kidneys
recipe - genes
ingredients - iron, b12, folic acid and other minerals
functioning bone marrow - no increased loss or destruction of red cells

28
Q

what is the main role of RBC

A

CO2 removal and O2 delivery from lungs to tissues

29
Q

what is the role of transferrin (Tf)

A

it is a glycoprotein synthesised in hepatocytes which delivers iron to all tissues including erythroblasts, hepatocytes and muscle

30
Q

what is the enzyme responsible for converting iron into haem

A

ALA-s2

EXCESS iron is stored as ferritin

31
Q

how do macrophages get iron

A

they swallow dying blood cells and break down the gglbin into amino acids and release them into the amino acid pool, the haem is broken down into bilirubin which is unconjugated so it makes its way to the liver to become conjugated

haem is also broken down into iron and this can be stored inside the macrophage as ferritin (the soluble form of iron)

32
Q

hepcidin

A

this is the low iron hormone and it acts to reduce the levels of iron in the plasma (this is what is lost in HC)

  • it acts by binding derroportin and degrades it - reducing iron absorption (enterocyte) and decreasing iron release from the RES
  • Hepcidin is synthesised in the liver
33
Q

Hypochromic microcytic RBC conditions

A

IDA = not enough haem
Thalassaemia = not enough globin

34
Q

what are the causes of IDA

A
  • dietary (premature neonates and adolescent females)
  • malabsorption
  • blood loss
35
Q

when is IV iron indicated

A
  • intolerant of oral iron
  • complicance
  • renal aneamia and Epo replacement
36
Q

causes of anaemia of chronic disease

A

infection
inflammation
neoplasia

RES iron blockage where the iron is trapped in macrophages and results in a raised level of hepcidin

reduced repo response

depressed marrow activity or cytokine marrow depression

37
Q

what is the definition of haemolytic anaemia

A
  • anaemia related to reduced RBC lifespan
  • no blood loss
  • no haematinic deficiency
38
Q

what happens due to the lifespan of RBC reducing

A

can begin to raise reticulocytes as it drops and increases bilirubin due to increased breakdown

then when this hits below 20 days then Hb drop
reticulocytes increase
bilirubin increases
splenic activity increases

39
Q

haemolytic example of an RBC enzyme related anaemia

A

pyruvate kinase deficiency anaemia
1. chronic/extravascular anaemia
2. ATP depletion
3. autosomal recessive

glucose 6 phosphate dehydrogenase deficiency
- acute episodic intravascular haemolytic
- x-linked recessive
- acute haemolytic from oxidative stress
1. favism
2. drugs such as antimalarials, sulphonamides and others

40
Q

what are the three types of acquired haemolytic anaemia

A

autoimmune = warm (IgG) and cold type (IgM)
isoimmune = haemolytic disease of newborn (HDN)
non-immune = fragmentation haemolytic

41
Q

polycythaemia

A

too much red cell production

42
Q

B12/flate functions

A
  • essential for DNA synthesis and nuclear maturation
  • required for all dividing cells
  • results in megaloblastic anaemia intially but will effect other organs
43
Q

how is b12 absorbed

A

b12 is ingested
gastric parietal cells produce intrinsic factor
b12 released by enzymes/acid in stomach and duodenum
intrinsic factor binds to b12
IF-B12 complex binds to cubulin which is a specific receptor in the ileum and B12 is absorbed into the blood and is then bound to transcobalamin

44
Q

what are the main tissues effects by b12 or folate deficiency

A

bone marrow
epithelial surfaces such as the mouth, stomach, small intestine, urinary and female genital tracts

45
Q

clinical b12 deficiency neurological manifestations

A

bilateral neuropathy or demyelination fo the posterior or pyramidal tracts of spinal cord

biochemical basis unclear - likely related to problem with homocysteine to methionine

46
Q

increased cell turnover causes of folate deficiency

A

haemolytic
severe skin disorders
pregnancy

47
Q

alpha gene permutations

A
48
Q

thalassaemia clinical significance of alpha

A

1 missing = mild microcytosis
2 missing = microcytosis with increased red cell count and sometimes very mild often asymptotic anaemia
3 missing = significant anaemia and bizarre shaped small red cells - HbH disease
4 missing = incompatible with life as you need alpha chains for metal haemoglobin

49
Q

HbH

A
  • lack go alpha chains means there is an excess of beta chains
  • these end up joining together
  • blood transfusion required during periods of stress
50
Q

what is the clinical result of sickle cell disease

A
  • reduced red cell survival (anaemia) = haemolytic which can also cause chronic endothelial dysfunction
  • vaso-occlusion
    tissue hypoxia/infarction
  • pain and tissue damage
51
Q

moya moya

A

when new collateral vessels are made in the circle of willis - 10% of children with sickle cell also have a stroke

GLA Y3S1 (13 decks)

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