HAEMATINICS Flashcards

1
Q

What are haematinics

A

analytes iron, cobalamin and folate

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2
Q

What are Haematinices important for

A

important markers of anaemia and can increase haemoglobin concentration

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3
Q

What is ferritin

A

major storage from of iron

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4
Q

where is ferritin stored and what is it composed off

A

composed of apoferritn and iron phosphate hydroxide core

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5
Q

the amount of circulating ferritin shows ?

A

the parallel concentration of storage iron

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6
Q

What is Haemosiderin

A

water insoluble heterogenous iron protein aggregate. long term storage iron. not readily mobilised

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7
Q

what colour is haemosiderin when stained with prussian blue. and when not stained

A

blue

golden brown

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8
Q

a low ferritin shows what?

A

iron deficiency

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9
Q

Borderline ferritin levels should be considered in the text of ….

A

CRP

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10
Q

what the 5 transferrin based measurements

A
transferrin
transferrin saturation .
soluble transferrin receptors .
unsaturated iron binding capacity
total iron binding capacity
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11
Q

variable affecting serum iron also affect what ?

A

transferrin measurements

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12
Q

main causes of cobalimin deficiency

A
Intrinsic factor 
impaired haptocorrin
drug/ normal malabsorption in ileum
inactivation of nitrous oxide 
inherited metabolic defect 
dietary but not as much as folate
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13
Q

folate related deficiency is caused by

A
mostly dietary 
jejunal abnormalities drug .induced malabsorption  
increased requirement .
increased loss.
acquired defects of folate metabolism.
inherited metabolic abnormalities
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14
Q

Cobalamin and folate relate to which anaemia

A

macrocytic anaemia

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15
Q

where does megablastic name come from

A

consequence of appearance of BM derived progenitors

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16
Q

what is responsible for morphological findings in megaloblastic anaemia

A

abnormal DNA

17
Q

Deficinecy of cobalamin can lead to what ?

A

neurological degeneration

18
Q

whats the 8 clinical symptoms of megaloblastic anaemia

A
lethargy
weakness 
yellow or waxy pallor
glottis with beefy red tongue / smooth pale tongue 
loss of appetite / weight
neurological symptoms in cobalamin deficiency 
peritheral neuropathy 
cognitive decline
19
Q

cord or peripheral nerve syndromes present with :

A

symmetrical distal sensory symptoms - feet>hands
diminished vibration sense and proprioception in the legs.
impaired distal cutaneous sensation
limb reflexes exaggerated/diminished/absent

20
Q

lateral column is involved with:

A

spastic paraparesis

autonomic bladder/ bowel or sexual symptoms

21
Q

FBC and blood films lack

cobalamin and folate

A

sensitivity in early deficiency

22
Q

Serum cobalamin measures both:

A

Inactive forms: transcobalamin I and holohaptocorrin bound cobalamin
Active form: holotranscobalamin

23
Q

Total Homocysteine increased in both cobalamin and folate deficiency, BUT

A

abnormalities can precede symptoms so can be useful

24
Q

Plasma Methylmalonic acid is increased in Cobalamin deficiency but is also affected by

A

renal disease, small bowel bacterial overgrowth and haemoconcentration

25
Q

Intrinsic Factor antibodies should be measured where pernicious

A

anaemia is suspected

26
Q

Serum folate can be measured to diagnose deficiency but

A

must be interpreted in context.

27
Q

Cobalamin deficiency complicates…

A

folate interpretation

28
Q

Genetic Assays can provide ?

A

genotype and good at identifying mutations. However, if new mutations are detected, then phenotypic characterisation of mutation required to determine structure/function impact.

29
Q

Phenotypic assays are good for?

A

Good for case management and can include activity within red blood cells.

30
Q

G6DP in qualitative test shows

A

G6PD activity measured but only determined to be above or below a certain threshold, for example 30% of normal = deficient.. Heterozygote females (activity can be 30-80%) may be missed.

31
Q

G6DP in quantitative test shows

A

Provides a precise measurement of G6PD and is normalised against haemoglobin concentration or red cell count. Will detect heterozygous females.

32
Q

Based on cytochemical reaction causing colour change can be measured by what 2 techniques

A

Can be measured by microscopy or flow cytometry

33
Q

Qualitative Fluorescent staining for G6PD activity works by

A

Fluorescent demonstration of G6PD activity by examining NADPH expression over a 10 minute period. Fluorescence (bright) shows oxidised NAPD is reduced to NADPH.

34
Q

Diagnosis of Pyruvate Kinase deficiency is based upon what 2 things :

A
  1. Demonstrating decreased PK enzyme activity in red cells

2. Identifying causative mutations in the PKLR gene.

35
Q

How PK deficiency measured

A

EDTA sample

36
Q

Results of PK may be affected by

A

Raised reticulocytes
Incomplete removed of Platelets and WBC
Presence of transfused red cells
Presence of the PK M2 isoenzyme (Muscle form, but also an important tumour marker)
Mutated PK that behaves ‘normally’ under in vitro conditions but ‘abnormally’ in vivo.

37
Q

How is EDTA sample reported

A

Reported as IU/gHb

38
Q

Phosphoenolpyruvate-Pyruvate-lactate. what becomes between the lines

A

Phosphoenolpyruvate-Pyruvate is converted by adp to ATP and pyruvate kinase.
Pyruvate is converted too lactate by LDH and NADH to NAD=

39
Q

What does PK biochemistry test show

A

In the presence of Pyruvate and LDH, the concentration of NADH reduces as it is oxidised to NAD+. This causes the optical
density of NADH to decrease (when using spectrophotometry @340nm) as NAD+ accumulates.
Where PK is deficient, [pyruvate] is reduced resulting in an increased NADH and high optical density.