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FRACP Shuyu > Alfred Haem 2 > Flashcards

Alfred Haem 2 Flashcards

1
Q

why is haptoglobin low in haemolysis

A

made by the liver and binds to free Hb

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2
Q

what condition would cause a low haptoglobin without haemolysis

A

liver disease (made by the liver)

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3
Q

does intravascualr or extravascular haemolysis cause lower haptoglobin

A

intravascular

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4
Q

what type of haemolysis have positive urinary haemosiderin

A

intravascular haemolysis

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5
Q

classiicaiton of intravascular haemolysis

A
  1. Red cell fragmentation
  2. PNH
  3. PCH
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6
Q

haemolysis + schistocytes/red cell fragments diagnosis

A

MAHA

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7
Q

what cell membrane surgace proteins protects the RBC from complement mediated lysis

A

CD 55 and CD 59 (implicated in PNH)

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8
Q

gold standard for diagnosis of PNH

A

flow cytometry - loss of expresison of GPI linke proteins on neutrophils or RBC in peripheral blood

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9
Q

what mAb is used for the management of PNH

A

eculizuman (humanized chimeric MoAb anti-C5)

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10
Q

mechanism of warm AIHA

A

IgG+/- C3d

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11
Q

causes of warm AIHA

A

SLE, CLL/lumphoma, drugs, idiopathic

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12
Q

diagnosis of warm AIHA

A

DAT (coomb’s test)

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13
Q

what class of antibodies cause cold AIHA

A

IgM

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14
Q

which AIHA is amendable to steroids

A

warm AIHA

steroids doesn’t work for cold AIHA

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15
Q

G6PD deficiency blood film

A

intravascular haemolysis

bites and blisters on film

self limiting

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16
Q

diagnosis of G6PD deficiency

A

enzyme assay when well (as false negative with reticulocytosis)

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17
Q

medications/foods that increase oxidative stress ( and can exacerbate G6PD

A

antimalarials

sulphur containins drugs

aspirin

vit K analogues

moth balls

probenecid

fava beans

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18
Q

when does haemoglobin switch occur?

A

12-18 months (often when beta thalassaemia manifest)

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19
Q

diagnosis of alpha thalassaemia

A

genetic studies because there is decrease in all the haemoglobins so might not show on gel electrophoresis

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20
Q

what haemoglobins are produced as a result of decreased alpha chain synthesis in alpha thalasseamia

A

tetrameters:

  1. Hb H (Betax4)
  2. Hb Bart’s (gamma4) - hydrops fetalis has Hb Bart’s only
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21
Q

what genotype of the parents will produce hydrops (thalassamia)

A

–/aa x –/aa or other combinations where both parents have a –/__

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22
Q

diagnosis of beta thalassaemia

A

haemoglobin electrophoresis - increased A2 and F

need to exclude Fe deficiency because Fe def can reduce beta globin production

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23
Q

sickle cell trait clinical characteristics

A

heterozygous

sickling may be precipitated by right shift in oxygen dissociation curve (fever, hypoxia, acidosis, general anaesthesia)

generally asymptomatic

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24
Q

management of sickle cell disease

A

chronic exchange therapy program

hydroxyurea - increases HbF levels and prevents crisis

folic acid

avoid triggers (note gen anaesthesia)

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25
Q

crizanlizumab

A

P-selectin antibody to prevent vaso-occlusion in sickle cell disease

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26
Q

are secondary or treatment related AML better or worse prognosis

A

worse

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27
Q

good risk cytogenetics

A

t(15:17) - APML

t(8:21)

inv16

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28
Q

bad risk cytogenetics (2)

A

monosomy 7

complex cytogenetics (asso with therapy AML)

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29
Q

bad risk molecular risk

A

FLT3 +ve

cKIT gene

30
Q

good risk AML molecular studies

A

NPM +ve

CEBPA +ve

31
Q

what TKI inhibits FLT3

A

midostaurin

32
Q

general CML TKI SE

A

pleural effusions/oedema, muscle aches, nausea

33
Q

EPO in polycythemia

A

low

34
Q

management of ET

A

aspirin +/- cytoreduction (hydrea) in high risk (age >60, plt >1000, Hx thrombosis)

interferon for cytoreduction in pregnancy

35
Q

5q minus syndrome clinical features

A
  • middle aged or older females
  • refractory anaemia
  • well preserved or even elevated platelet counts
  • good responses to lenalidomide
  • low rate of progression to AML
  • mortality from complications of iron overload
36
Q

causes of acquired vWD

A

lymphoproliferative disorders

myeloproliferative disorders

aortic stenosis

37
Q

why is chromosome 14 implicated in lymphoma

A

heavy chain promotor - translocation results in juxtaposition with oncogene

38
Q

what virus is implicated in the pathogeneis of splenic marginal zone lymphoma

A

Hep C

39
Q

where are Reed-Sternberg cells found

A

Hodgkin’s lymphoma

But these cells only make up a small amount of tumour bulk with the remaining tumour made up of supporting cells

40
Q

what cells express CD30+

A

(i) classical Hodgkin’s lymphoma (originally identified on Reed-Sternberg cells)
(ii) anaplastic large cell lymphomas (ALCL)
(iii) primary cutaneous CD30+ T-cell lymphoproliferative disorders

41
Q

adverse effects of ibrutinib

A

bleeding (platelet inhibition) and AF

42
Q

what types of CLL would FCR (Fludarabine, Cyclophosphamide, Rituximab) have no effect in

A

del(17p) or p53mut

43
Q

imaging for diagnosis of bone lesions in MM

A

whole body myeloma CT scan

44
Q

bone disease multiple myeloma supportive therapy

A

IV zoledronic acid for 2 years

45
Q

most common cause of mortality following autograft SCT

A

relapse of disease

46
Q

which leukaemia has the highest risk of transplant after HSCT

A

ALL >AML > CML

47
Q

What mutation is Budd Chiari syndrome associated with

A

JAK2

48
Q

What are features of the MCV:Hb ratio in iron deficiency anaemia vs thalassaemia?

A

IDA has similarlly reduced Hb and MCV where as in thalassaemia, the MCV is a lot more reduced than the Hb

49
Q

What does hepcidin do to ferroportin?

A

Hepcidin causes ferroportin to be internalised and trapped in enterocytes and less system absorbtion of iron

50
Q

What condition is associated with pencil cells on blood film?

A

Iron deficiency anaemia

51
Q

What is the best screening test for haemochromatosis?

A

Transferrin saturation

52
Q

Where is transferrin produced?

A

In the liver

53
Q

How to delinate for IDA in anaemia of chronic disease?

A

Ferritin >100 unlikely to be iron deficiency anaemia

Ferritin <100 - look at soluble transferrin receptors

Soluble transferrin receptors are soluble fragments of transferrin receptors which are upregulated in low iron states

54
Q

What is functional B12 bound to?

A

transcobalamin II

55
Q

What is the best test for folate deficiency?

A

red cell folate

56
Q

What is the most specific for pernicious anaemia?

A

Parietal cell antibodies

57
Q

What are some causes of secondary cold agglutinin disease?

A
58
Q

What pathway is affected in G6PD deficiency?

A

Hexose-monophosphate pathway: pentose phosphate pathway

Involved in produxxtion of NADPH for protection of cell against oxidative stress and oxidation of G6P (into pentose 5-P)

59
Q

What is the inheritance of G6PD deficiency?

A
60
Q

What causes thorney apple cells on blood film?

A

Pyruvate kinase deficiency (another RBC membrane problem)

Dx by enzyme assay

61
Q

What does HPLC and electrophoresis show for -a/-a or –/aa?

A
62
Q

How to manage beta thal major?

A

Transfuse to Hb 9-10g/L to suppress X-medullary haematopoiesis

Iron chelation aim for ferritin <1000: desferrioxamine (subcut)

63
Q

What is the amino acid substitution in sickle cell disease?

A
64
Q

ATRA syndrome

A
65
Q

Management of PRV

A

Low risk (<60 and no PHx thrombosis) - phlebotomy for Hct <45%; low dose aspirin

High risk (\>60yo or PHx thrombosis) - as per low risk group 
AND 
cytoreductive therapy (hydroxyurea)
66
Q

What lymphomas are EBV associated with?

A

Burkitt’s, Hodgkins lymphoma, HIV assoc lymphoma, PTLD

67
Q

What is the most common cause of SVC obstruction in young adults?

A

Hodgkin’s lymphoma

68
Q

What are bad cytogenetics in CLL?

A

17p del, trisomy 12, 11q

69
Q

What is the MOA of venetoclax?

A

BH3 mimetic that inhibits function of BCL2

Bcl2 is usually located on the outer membrane of the mitochondria to inhibit pro-apoptotic proteins thus promoting cell survival

70
Q

What prophylaxis does bortezimib require?

A

Aciclovir for HSV prophylaxis

71
Q

What gender is a major risk factor for the development of GVHD

A

Female

FRACP Shuyu (65 decks)

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