Hereditary Haemolytic Anaemias Flashcards Preview

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Flashcards in Hereditary Haemolytic Anaemias Deck (13):
1

Hypotonic lysis test

Main use for HS
Déterminés surface area: vol
Mix blood w graded concentrations of hypotonic saline and measure lysis colorimetrically
plot and the lysis from each of 12 tubes to create fragility curve

2

Factors affecting osmotic fragility test

Vol of hypotonic saline mix
Temperature
Ph- fragility increased by decreases. Ph

3

Increased osmotic fragility

He, elliptocytosis, hereditary stomatocytosis, Aiha( due to spherocytes)

4

Decreased osmotic fragility

Thalassaemia, iron def anaemia,
May have dec in hereditary enzyme disorders due to inc in reticulocyte count
Hereditary xerostomatosis

5

False positive - dec osmotic fragility not due to hs

Pregnancy
Haemolysis
Renal failure(?pH issue)

6

Best test for HS ne principle

Flow looking at eosin-5- malaise labelled red cells

7

Results ema

a ratio of mean value of the test to control sample.
a ratio of 0.8 or more is regarded as normal.

8

Acidified glycerol lysis time test

Same principle- looking at lysis based on surface area:volume
Good for screening family if known HS and minimal morphology
Use acid and glycerol

9

Autohaemolysis test-
1 with glucose
2 sans glucose

Normal blood there is minimal lysis- even less w glucose
Defects 1. If the result is entirely normal, an intrinsic red cell abnormality is unlikely. 2. If abnormal haemolysis is fully corrected by glucose, a metabolic abnormality is unlikely and a membrane abnormality is likely. 3. If abnormal haemolysis shows little or no correction by glucose, a metabolic abnormality is likely, provided that obvious features of spherocytosis are not present on the blood film.

10

Autohaemolysis test-
1 with glucose
2 sans glucose

Normal blood there is minimal lysis- even less w glucose
Defects 1. If the result is entirely normal, an intrinsic red cell abnormality is unlikely. 2. If abnormal haemolysis is fully corrected by glucose, a metabolic abnormality is unlikely and a membrane abnormality is likely. 3. If abnormal haemolysis shows little or no correction by glucose, a metabolic abnormality is likely, provided that obvious features of spherocytosis are not present on the blood film.

11

What genetic abn found in 20%babeie s iif mum w AFLP

LCAT deficiency

12

False elevation of retic count

Neonatal
Post spleen
High wbc or nrc count abn lymph’s
Hj bodies
Cold agg
Large plts
Malaria
Heinz bodies

13

Disease associated with Heinz bodies

G6pd
Nadph
Chronic liver dx
Alpha thal