Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

MRCP p1 > Haem-onc > Flashcards

Haem-onc Flashcards

1
Q

what gene is found to mutated in essential thrombocytosis?

A
  • CALR (calreticulin) is a more commonly found gene mutation in ET in around 20% of - JAK-2 - most common
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is the tx of essential thrombocytosis?

A
  • hydroxyurea (hydroxycarbamide) is widely used to reduce the platelet count
  • interferon-α is also used in younger patients
  • low dose aspitin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is the most common infection agent in neutropenic sepsis?

A

coagulase-negative, Gram-positive bacteria are the most common cause, particularly Staphylococcus epidermidis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what carcinogen is associated with hepatocellular carcinoma?

A

aflatoxin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what cancer does nitrosamine cause?

A

oesophagel and gastric cancer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what tx increases the live birth rate in antiphospholipid syndrome?

A
  1. low-dose aspirin should be commenced once the pregnancy is confirmed on urine testing
  2. low molecular weight heparin once a fetal heart is seen on ultrasound. This is usually discontinued at 34 weeks gestation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the tx for chronic lymphcytic leukaemia with patients with intact TP53 and with mutated IGHV?

A

CLL - treatment: Fludarabine, Cyclophosphamide and Rituximab (FCR)

Mutations of TP53 are associated with a poor response to chemo-immunotherapy (e.g. FCR) and warrant the use of novel agents such as Bruton tyrosine kinase inhibitors (e.g. ibrutinib), or BCL2 inhibitors (e.g. venetoclax)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is the tx for sickle cell anaemia?

A

Hydroxycarbamide, or hydroxyurea, increases the concentration of foetal Hb and has been shown to reduce the frequency of painful episodes and the risk of life-threatening complications.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what are the different cytotoxic agents and their mode of action?

A

Cyclophosphamide, Ciclosporin, Cispltin = Cross linking
Bleomycin = blows DNA = degrades formed DNA
Topotecan, Irenotecan = Topoisomerase inhibition
HydroxyuRea = Ribonucleotide Reductase inhibition

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what are the causes of methaemoglobinaemia?

A

Congenital causes
- haemoglobin chain variants: HbM, HbH
NADH - methaemoglobin reductase deficiency

acquired causes:
- drugs - sulphonamides, nittreates, dapsone, sodium nitroprusside, primaquine

DaPSoNe:
Dapsone
Primaquine
Sulfonamides
Nitrates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what is the mx of methaemoglobinaemia?

A
  • NADH methaemoglobinaemia reductase deficiency: ascorbic acid
  • IV methylthioninium chloride (methylene blue) if acquired
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

how do you differntiate bw myeloma and waldenstrom’s macroglobulinaemia?

A

Bony lesions with no Organomegaly: Myeloma
Organomegaly with no bony lesions: Waldenstroms

Benz Jones can be positive in both

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what are the sx of waldenstrom’s macroglobulinaemia?

A

lymphoplasmacytoid malignancy

  • systemic upset: weight loss, lethargy
  • hyperviscosity syndrome e.g. visual disturbance : the pentameric configuration of IgM increases serum viscosity
  • hepatosplenomegaly
  • lymphadenopathy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what is the typical presentation of methaemoglobinaemia?

A

respiratory distress and decreased oxygen saturations despite a normal pO2 on blood gas and respiratory examination.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what does high reticuloytes suggest?

A

haemolysis or bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what are the different types of primary immunodeficiencies?

A

Sh*tty B Cells: (B Cell Disorders)
S: Selective IgA Deficiency
B: Brutons
C: CVID

WASH your Bs and Ts: Combined B and T Disorders:
W: Wiskott Aldrich Syndrome
A: Ataxic Telengectesia
S: SCID
H: Hyper IgM Syndromes

DiGeorge is T cell

And the rest Neutrophil Function

17
Q

what are the types of cryogobulinaemia?

A

type 1 - monoclonal - IgG and IgM (associated with multiple myeloma, waldenstrom’s macroglbulinaemia)

type 2 - mixed monoclonal and polyclonal (associated with RF and conditions: HCV, RA and sjogren’s lymphoma)

type 3: polyclonal (associated with rheumatoid arthritis, sjogren’s)

18
Q

how do you differntate type 1 cryoglobulinaemia from other types?

A

raynayd’s only seen in type 1

19
Q

what transformation is common in polycytheamia rubra vera?

A

5-15% of patients progress to acute myeloid leukaemia

5-15% of patients progress to myelofibrosis

20
Q

what chromosomes are lymphomas associated with?

A

(11, 14) -> Mantle Cell Lymphome
(8; 14) B(8)urkitt lymphome
(14; 18) follicular lymphoma - > 18 alphabet! - increased BCL-2 transcription

21
Q

how is haemorrhagic cystitis caused by cylocphosphamide reduced?

A

Hydration and Mesna

A metabolite of cyclophosphamide called acrolein is toxic to urothelium

mesna binds to and inactivates acrolein helping to prevent haemorrhagic cystitis

22
Q

what is the tx of sickle cell crisis mx?

A
  1. analgesia e.g. opiates
  2. rehydrate
  3. oxygen
  4. consider antibiotics if evidence of infection
  5. blood transfusion
  6. exchange transfusion: e.g. if neurological complications
23
Q

what is the difference between MGUS and myeloma?

A
  • normal immune function
  • normal beta-2 microglobulin levels
  • lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA)
  • stable level of paraproteinaemia
  • no clinical features of myeloma (e.g. lytic lesions on x-rays or renal disease)
24
Q

how does desmopressin work in vWF?

A

desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells

25
Q

how do you differentiate leikaemoid reaction from chronic myeloid leukaemia?

A

Leukaemoid reaction:
-high leucocyte alkaline phosphatase score
-toxic granulation (Dohle bodies) in the white cells
-‘left shift’ of neutrophils i.e. three or fewer segments of the nucleus

Chronic myeloid leukaemia:
-low leucocyte alkaline phosphatase score

26
Q

what is a useful prognostic indicator in myeloma?

A

b-2 microglobulin levels

27
Q

which medications cause pancytopenia?

A
  • cytotoxics
  • antibiotics: trimethoprim, chloramphenicol
  • anti-rheumatoid: gold, penicillamine
  • carbimazole*
  • anti-epileptics: carbamazepine
  • sulphonylureas: tolbutamide
28
Q

how do you differentiate walderstorms and wiskott?

A

IgM more in walderstorm
IgM less in wiskott

29
Q

what are the features of wiskott-aldrich syndrome?

A

WATER

Wasp mutation , Thrombocytopenia , Eczema , Recurrent infection/ Recessive X linked

30
Q

how does acute promyelocytic leukaemia present?

A

classically disseminated intravascular coagulation

translocation with t(15:17)

31
Q

A 10-year-old boy is referred to you following his 7th course of antibiotics for lower respiratory tract infection in the last 6 years. He has difficult to control eczema for which he is currently on a topical steroid cream. His bloods are as follows

low platelts
what is the abnormaility

A

frequent infections, eczema and thrombocytopenia are characteristic of the Wiskott-Aldrich syndrome, which is caused by an abnormality in the WASP gene.

MRCP p1 (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions