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FRACP > Haematology > Flashcards

Haematology Flashcards

1
Q

What do multipotent stem cells differentiate into?

A

Myeloid and lymphoid progenators

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2
Q

What do myeloid progenitor cells differentiate into

A

Megakaryocytes- platelets
Erythroblasts- retics- red cells
Myeloblasts- myeloblasts- neuts, basophils eosinophils and monocytes

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3
Q

What do lymphoid progenator cells differentiate into?

A

Plasma cells
T cells
B cells

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4
Q 
Name the CD4 numbers for the following 
NK cells 
T helper cells 
T killer cells 
B cells
A

NK- CD56
T helper- CD 4
T killer- CD 8
B cells CD 19 and 20

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5
Q

What are the three stages of haematopoiesis
When do neutrophils develop?

Can EPO cross the placenta?
How does red cell life span change over time

A

Mesoblastic- yolk sac
Hepatic- 2 to 24 weeks- liver
Myeloid

Myeloid stage
No
Increases over time

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6
Q

What is the normal structure of HbA
What chromosomes are involved
What are the three types of embryonic hb? What Greek letters are present?
What makes up Hb F
What makes up Hba2? How much is normally found in adult blood?

A

2 alpha, 2 beta 4 haem groups containing iron

Alpha-16
Beta-11

Gower 1,2 and Portland- zeta alpha and epsillon
HbF 2 alpha 2 gamma
HbA2 2 alpha 2 delta
HbA to A2= 30:1

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7
Q

What are the 5 subtypes of microcytic anaemia

A 
Thalassaemia
Anaemia of chronic disease
Iron def
Lead poisoning 
Sideroblastic
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8
Q

On a normal blood film, what do platelets and red cells look like

A

Red cells- discs with a pale centre

Platelets- smallest cells on the film

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9
Q

When do iron stores run out?
Can it be synthesised by the body?
What is the most common cause of iron deficiency in toddlers?
What type of anaemia is seen?
What is seen on blood film?
What should be given along with iron? Why?
How long will it take to see a reticulocyte response?

A 
5-6 months
No 
Excessive cows milk- poorly absorbed 
Hypochromic microcytic 
Ansiocytosis, rod cells and target cells 
Vitamin C- helps the absorption
3 days
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10
Q

Alpha thalassaemia
Which chromosome is the defect?
What are the 4 possibilities and how will they present?
Is it a quantity or a quality problem?

A

16

1 allele missing- silent carrier
2 missing- microcytic anaemia
3 missing hbH- above plus hepatomegaly and haemolysis
4- incompatible with life- hydrops

Quantity

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11
Q 
Beta thalassaemia 
What is the inheritance and which chromosome 
What are the three forms 
What are the complications of major?
When does major present and why
A

AR- 11
1) carrier= 1 allele defective. Asymptomatic. Increased hbf and a2

2) intermedia-2 allele (one less severe) mild symptoms

3) major 2 alleles- transfusion dependant, splenomegaly and abnormal faces
Colelothiasis and haemochromatosis

6 months- normal transition point to HbA

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12
Q

What is seen on blood film in lead poisoning

A

Basophilic stippling and sideroblasts

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13
Q

Sideroblastic
What is it?
How is it inherited?
What syndrome is it associated with?

A

Unable to use iron properly, iron accumulates in mitochondria
X linked
Pearson syn

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14
Q

What should be monitored for iron overload
When is chelation started
What infection is associated?

A

Serum ferritin levels
>1000
Yersinia

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15
Q

What do the direct and indirect Coombs pick up

A

Direct- antibodies on red cells

Indirect- antibodies to red cells in serum

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16
Q

What is the genetic defect in sickle cell anaemia

A

Point mutation in ch11 (abnormal beta chains)

Glutamine to valine

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17
Q

When does sickle cell anaemia present?
What is a sequestration crisis
How could Vaso occlusive disease present?
What is the infection risk
What normally triggers an aplastic crisis
What is seen on blood film. What indicates the spleen issues
What is the definitive diagnostic test?

A

6 months/ picked up on screening. Chronic haemolytic anaemia( jaundice, gallstones, tachycardia, splenomegaly and sickle faces- maxillary hyperplasia)

Sudden reduction in circulating volume, sequestration In The spleen

Pain dactylysis priapism

Encapsulated organisms (functionally asplenic from 5 yrs)parvovirus b19

Sickle cells target cells and Howell jolly bodies. Targets and HJBs

Hb electrophoresis

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18
Q

Treating sickle cell disease
What level of HbS should you aim for with transfusions
What else do they need

A

<30%

Pneumococcal and meningococcal vaccines Penicillin prophylaxis

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19
Q

What is the defect in g6pd def?
How is it definitively diagnosed
What is the inheritance

A

Missense mutation
Lacking nadph- prevents cells against oxidative stress
Enzyme activity analysis
X linked

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20
Q

Does g6pd have hepatosplenomegaly?

A

No!

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21
Q

What can precipitate haemolysis in g6pd- 1 food and 3 drugs

How quickly do symptoms occur after exposure

A

Favs beans
Co trim chloramphenicol and nitrofurantoin
24-48 hours

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22
Q

What is seen on blood film in g6pd def

A

Heinz bodies and bite cells

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23
Q

What is the pattern of inheritance in pk def. what does the defect cause

A

AR

Reduced atp. Rigid cells

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24
Q 
What causes HS 
What is the pattern of inheritance 
What is the typical presentation 
What is the definitive test?
What other fancy test might be used
A 
Abnormal spectrin protein
AD 
Intermittent jaundice. Neonatal jaundice. Mild NN anaemia 
Osmotic fragility year
EMA testing- lower than normal
25
Q

Fanconi anaemia
What phenotypically is seen
What is the definitive diagnostic test
What type of anaemia do you see?

A

Short, Bifid thumbs, urinary retention, cafe au lait spots
Lymphocyte breakage tests
Macrocyclic

26
Q 
Fanconi anaemia
What is the inheritance?
What is the pathophysiology?
What cell lines are affected?
What cancers are they most at risk of
A

Autosomal Dom or recessive
Increased fragility of chromosomes
Initially red and plt then neutrophils
SCC and AML

27
Q

Diamond blackfan anaemia
What does it cause
What cell lines will it affect
What are the classic phenotypical features

A

Early arrest of red cells maturing
Red cells only
Short, low IQ, webbed neck, triphalangeal thumb and renal anomalies

28
Q

TEC How does it differ from diamond blackfan anaemia + what test would help?

A

Resolved in 1-2 months
More likely toddlers
No abnormal phenotypes
Erythrocyte adeonsine deaminase activity normal not increased like DBA

29
Q

Pearson syndrome
Inheritance
What does it cause

A

Mitochondrial
Pancreatic dysfunction
Pancytopenia

30
Q

Dyskeratosis congenita
What type of problem is it?
What 3 classic features are seen
What cancers are they at risk of

A

Telomere breakage
Oral leukoplakia, nail dystrophy, lacy rash
AML

31
Q

Folate deficiency
What is the main source in the body?
How long do stores last?
Where is it absorbed

A

Dietary
3-4months
Small intestine

32
Q 
b12 def
Where is it absorbed and stored 
How long do stores last for 
What can severe deficiency cause?
What is seen on film?
A

Terminal ileum. Stomach
3-4 yrs
Subacute combined degeneration of the Ford- post column loss
Large hypersegmented neuts

33
Q

Cyclical neutropenia
What are the features
What gene is abnormal
What might gingivitis suggest?

A

Cycles of low neuts every 21 days, last 3-5 days. Fever, malaise, ulcers and sore throat

ELANE

Non benign cause

34
Q

Chronic neutropenia
What is the ANC normally?
What types of infections do they tend to get?

A

<200

Pseudomonas staph and E. coli

35
Q

ITP
What normally triggers it? Timing?
What type of antibody develops? To what ?
How long should it take to resolve if acute?
What is the most serious side effect?
What is Evans syndrome?

A 
Viruses- 1-4 weeks post 
IgG to glycoprotein 11b/11a
Under 6 months
Intracranial haemorrhage
ITP and haemolytic anaemia
36
Q

What enzyme is lacking in ttp
What might trigger it?
2 ways it’s different from hus ?
How is it treated?

A

AdamTS13
Quinine, pregnancy, surgery
Infections
Vague symptoms leading to CNS signs and less diarrhoea

Plasma exchange

37
Q

What is the difference between neonatal alloimmune and autoimmune thrombocytopenia
What type of antigens are usually found in alloimmune? Against what? If Asian what?
What is the risk of ICH

A 
Autoimmune= mum has ITP. Mum will have low PLT
Alloimmune= mums platelets normal, abs to baby’s platelets

IgG-HPA1A 4a if Asian
30%

38
Q

TAR syndrome

How do you differentiate from fanconi anaemia

A

Traombocytopenia with bilateral absent radii
TAR Have thumbs
Fanconi are bifid

39
Q

What is Bernard soulier disease
Do they have any other physical features?
How is it different from glanzman

A

Autosomal recessive defect of glycoprotein receptor.
Giant platelets
No!
Glanzman has normal platelet size

40
Q

What does the PT and APTT measure in terms of clotting pathways?
Which conditions are therefore screened by each?

A

PT- extristic pathway
APTT- Intrinsic pathway

PT- vitamin k def, liver disease, DIC, anticoagulants

APTT- above plus haemophilia, VWD

41
Q

What factors are involved in the extrinsic pathway?
“ intrinsic
“Common

A

Tissue factor and 7

12,11,9 and 8

X to Xa, 2 to 2a (prothrombin to thrombin), 13

42
Q

What are vitamin K dependant factors?

A

2,7,9 and 10

43
Q

Haemophillia
What is the usual pattern of inheritance?
How does haemophillia C differ?
What happens to the APTT and PT?
How will it change with mixing of inhibitors present
What will happen to the bleeding time?
What will give a prolonged APTT but normal reptilase time

A 
X linked recessive
30% de novo 
Autosomal recessive
Prolonged APTT, normal PT
Won’t correct 
Normal
Heparin contamination
44
Q

Haemophillia
What are normal levels of factor 8 and 9
What classifies a mild, moderate or severe deficiency?
What is a target joint?
What is the common presentation in a neonate?
What is the most risk muscle to bleed into?

A

8-30-40
9-25-30

Mild-<5% mid 1-5%, severe <1%

Thinning of the synovium following a bleed. Means repeated bleeds more likely

Post circumcision bleeding

Ileopaoas

45
Q

Treating haemophillia

In a bleed what should you aim factor to be in mild or severe deficiency?
When does desmopressin have a role? What does it stimulate?
What percentage will develop inhibitors?
When do they occur? Are they equal in A&B?
What tests for them?

A 
Mild >30% 
Severe 100%
Mild A only- stimulates release of VWF from endothelial cells 
20%- A is more common 
20 exposure days. Bethesda assay
46
Q

How might factor 13 deficiency present

A

Delayed bleeding from the umbilical cord

47
Q 
Von Williebrand 
What is the inheritance? 
How do the three types differ? 
What types have low factor 8?
What happens to the APTT, pt and bleeding time?
Where do platelets and vwf bind to?
What medication may be useful?
Are boys more likely to have it than girls
A 
AD
1-Reduced production
2-reduced quality
3- absent factor 
1&amp;2
Long APTT and bleeding time, normal pt 
Glycoprotein 1b
Desmopressin
NO
48
Q

Name 5 inherited thrombophillias

A

Factor 5 leiden
Factor C&S def
Antiphospholipid syndrome
Homocysteinuria

49
Q

How do the pharmacomechanics of unfractionated and low molecular weight heparin differ?

What is the most commonly used thrombolytic drug in children?

A

Unfractionated- wider action by increasing activity or antothrombin 3 (blocks extrinsic pathway)
Lmw- acts directly on 10a

TPA

50
Q

Warfarin
What do you need to cover with initially
Which clotting factors does it inhibit

A

Heparin

2,7,9&10- vitamin K dependant

51
Q

What is commonly seen on blood film in aplastic anaemia

A

Macrocytosis

52
Q

What does cryoprecipitate contain

A

Fibrinogen, factor 8 and 13

53
Q

What does FFP contain

A

All clotting factors, albumin and immunoglobulins

54
Q

In DIC what should be given first

A

Cryoprecipitate to replenish fibrinogen then FFP

55
Q 
What is the half life of 
Factor 7
Factor 9 
Factor 10 
Factor 2
A

7- 4-6h
9- 24 hrs
10 24-48 hrs
2 60 hrs

56
Q

Alpha thalassaemia

For a child to have significant disease what do the parents have to have at least

A

Alpha zero (2 gene deletions)

57
Q

Other than haemophillia with inhibitors, what is a cause of prolonged APTT that won’t correct with mixing

A

Lupus

58
Q

What are examples of intravascular haemolysis. What is seen in blood
Physiologically what happens
“Extra vascular

A

Intra- haemolysis within the vessel. HUS, DIC mechanical valves
Raised LDH ++ And low haptoglobin. Hb in urine

Extra- haemolysis in RE system
Hereditary auto/allo immune causes
Urine clear, haptoglobin and LDH less elevated

FRACP (23 decks)

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