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Flashcards in general haem Deck (66):
1

What is term for primary polycythaemia?

polycythaemia rubra vera

2

how can we differentiate primary and secondary polycythaemia on blood test?

high EPO= secondary polycythaemia whereas normal EPO for primary polycythaemia

3

what is the type of disease is polycythaemia rubra vera?

myeloproliferative

4

what are 2 causes of a high haematocrit?

reduced plasma volume (dehydration) and polycythaemia

5

what are some causes of secondary polycythaemia

• Living at high altitudes
• Tumours like posterior cranial fossa, renal cell carcinoma
• COPD
• Cyanotic congestive heart disease- R to L shunts (tetratology of fallot)
• (bad lung disease)
• High affinity variance (changes O2 affinity to Hb)
• Hereditary persistence of HbF
Delta thalassaemias

6

is there any increased risk of thrombosis in secondary polycythaemia?

no

7

what drugs/foods can cause oxidative haemolysis?

Antimalarials
Antimicrobials
Fava beans
Henna

8

what can cause eosinophillia on FBE?

1. Parasitic infections
2. pneumonia
3. small cell vasculitis- chugg strauss
4. asthma?

9

what is paroxysmal nocturnal haemoglobinuria?

acquired clonal defect of red cells that results in production of erythrocytes with an increase susceptibility to lysis by complement.

-Patients intermittently pass bloody urine, more frequently at night

- associated with increase thrombotic risk

10

what are the three conditions that constitute myeloproliferative disorders?

what is a mutation commonly associated with them?

what do they predispose?

1. polycythaemia rubovera
2. Essential thrombocythaemia
3. Myelofibrosis

JAK2 mutation

predispose acute myeloid leukaemia

11

what is the difference between myelodysplastic disorders and myeloproliferative disorders?

Myelodysplastic syndrome is ineffective maturation whereas myeloproliferative neoplasm is an overproduction of mature cells.

12

oxygen saturation equation?

CO x [Hb] x %saturation x 1.34

13

what are some general features/symptoms of myeloproliferative disorders?

Thrombosis
stroke, TIA
digital ischaemia and gangrene
pruritis after exposure to water
splenomegaly

14

what are the 4 groups of causes of pancytopenia


1. Ineffective erythropoesis (megaloblastic anaemia, AIDS)
2. Increased peripheral destruction (SLE)
3. Bone marrow infiltration (leukaemia)
4. Bone marrow failure (drugs)

15

what happens when you have RhD+ve autoantibodies passing to the unborn child (from rhesus d neg mother to rhesus d positive baby)

fetal anaemia

haemolytic disease of the newborn

16

what is the difference between Group and Hold and crossmatched blood?

take patient's blood and order Group and Hold.

17

what is the difference between bone marrow aspirate and trephine?

aspirate- fluid
trephine= biopsy

18

how might anti thrombin 3 be pathologically/iatrogenically reduced?

nephrotic syndrome, plasma exchange and asparaginase chemotherapy drugs

19

what causes hyperviscosity?

1.very high red cell count (polycythaemia)
2.high white cell counts (e.g. leukaemias)
3.high paraprotein content like myeloma
4.drugs- OCP, EPO, chemo, diuretics, IVIg

20

what do we mean by venesection? when might we do this?

removal of blood for treatment
for conditions like haemachromatosis and high blood viscosity syndrome

21

general cause of hypersplenism?

sequestering abnormal blood cells in the spleen

22

what do you think when you see small white nodules affecting the entire parenchyma of the spleen (pathology)

lymphoma
miliary TB
Sarcoidosis
amyloidosis

23

what constitutes an Fe study?

ferritin
serum iron
transferrin
transferrin saturation

24

does haemolytic anaemia cause dark urine?

no

25

would you be worried about a slightly elevated platelet count in the setting of inflammation/infection?

no bc platelets are an acute phase reactant

26

what consists of haemoglobinopathy?

Sickle cell anaemia
thalassemia
porphyria

27

what is the mutation in sickle cell anaemia?

glutamate to valine in position 6 of the beta chain

28

where are B thalassaemias most prevalent?

mediterranean countries

29

where are A thalassemias most prevalent?

SE asia

30

what is HbH

tetramer of beta chains

31

what are normoblasts?

nucleated RBC

32

what is the difference between haemosiderin and ferritin?

haemosiderin= insoluble iron protein complex
ferritin= soluble iron protein complex

33

what is aplastic anaemia?

pancytopenia with hypocellularity in the bone marrow

34

what is haemophilia A also known as?

factor 8 deficiency

35

what is haemophilia B also known as?

factor 9 deficiency

36

what is the inheritance pattern of haemophilia A

x linked recessive

37

how many types of VWF disease is there?

3

Type 1: not enough VWF
Type 2: malformed VWF
Type 4: no VWF

38

what is the mutation involved in factor 5 leiden disease?

Factor V Leiden differs from normal factor V by a single
nucleotide substitution (Arg506Gln). This variation makes
factor V less likely to be cleaved by activated protein C.

--> increases likelihood of thrombosis

39

is anti-thrombin deficiency an inherited or an acquired condition?

it is BOTH
inherited and acquired

40

how many porphyria conditions are there? which is the most common?

8 porphyria conditions
Porphyria cutanea tarda is the most common

41

what is porphyria?

condition that affects haem synthesis, due to deficient enzymes. You get a build of porphyrins which is toxic to the body

42

what is the pathophysiology of porphyria cutanea tarda, PCT?

UROD deficiency (uroporphyrinogen decarboxylase).

43

symptoms and signs of porphyria cutanea tarda?

• Blistering skin lesions in sun exposed areas
• Skin hyperpigmentation
• ONYCHOLYSIS
• Red urine
Alopecia
- excess iron deposition

44

What is the inheritance pattern like for porphyria cutanea tarda?

autosomal dominant or SPORADIC (most are sporadic)

45

how to manage porphyria cutanea tarda?

- avoid alcohol, oestrogen and UV sunlight
-cease smoking
- phlebotomy to remove iron excess
-antimalarials

46

triggers for ITP?

1. Medications- Quinine (Gin and Tonic)
2. Viral infection- EBV, CMV, HIV
3. Lymphoproliferative disorders (B cell CLL/NHL)
4. Connective tissue disorders

47

what is ITP

autoimmune destruction to platelets

48

management of ITP

First line
• Remove the trigger
• Steroids
• Intravenous immunoglobulin (IVIg) (often combined with steroids INTRAGAM)

Second line
• Anti CD20 Rituximab
• Splenectomy
• Thrombopoietin receptor agonist

49

Ix for ITP

• FBE and blood film
• Viral screening
• Screen for lymphoma if relevant
Anti platelet antibody tests NOT usually relevant

50

pentad for TTP

1. Thrombocytopenia
2. Microangiopatic haemolytic anaemia
3. Neurologic abnormalities
4. Renal failure
5. Fever

51

what are the two key findings on blood film for TTP?

1. evidence of microangiopathy with red cell fragmentation
2. thrombocytopenia

52

triad for TTP?

1. Thrombocytopenia
2. Schistocytosis
3. Elevated LDH

53

what can trigger TTP?

It can be triggered by viral infection, shigellosis, pregnancy and malignancy

but mostly IDIOPATHIC

54

What deficiency is associated with TTP

ADAMTS13 deficiency --> leading to inability to cleave VWF precursor leading to micro thrombi formation

55

what is the key difference between DIC and TTP?

DIC is due to activation of the clotting cascade --> micro thrombi whereas TTP does not activate the clotting cascade

DIC- low fibrinogen prolonged APTT time
TTP- normal fibrinogen levels, normal APTT/PT time

DIC = abnormal clotting (micro thrombi) and haemorrhage
TTP= just micro thrombi

56

what is the mainstay treatment of TTP?

plasmapheresis + FFP

57

what part of the coagulation pathway does APTT look at?

intrinsic (TT is played indoors)

58

what part of the coagulation pathway does PT/INR look at?

extrinsic (tennis is played outdoors)

59

what coagulation study change can indicate VWF disease?

raised APTT levels and decreased levels of VWF antigen

60

what are some symptoms that make you suspicious of VWF disease?

menorrhagia!

epistaxis, bleeding gums, mucocutaneous bleeding

61

treatment of type 1 VWF disease?

desmopressin

62

why do we get haemorrhage in DIC?

consumption of clotting factors

63

do you treat B12 deficiency anaemia with blood transfusion?

NO. use IM hydroxycobalamin

64

what are the inherited thrombophillia disorders?

1. Factor 5 leidin mutation
2. Protein C and S mutation
3. Prothrombin mutation
4. Anti-thrombin 3 deficiency

65

what are the acquired thrombophilia disorders?

1. Anti phospholipid disorder
2. OCP

66

which clotting factors do the liver produce?

all apart from factor 8