Brainscape's Knowledge GenomeTM

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


See full index

MRCP > Clinical haematology & oncology > Flashcards

Clinical haematology & oncology Flashcards

1
Q

haemophilia

  • types? what are they def in? which is more common
  • inheritance?
  • fts on blood tests
A

A: lack of factor 8 (90% of haemophilia)
B: lack of factor 9

Long APTT w normal bleeding time/thrombin time/PT

X-linked recessive

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

tear drop poikilocytes

A

myelofibrosis

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

TTP

  • pathology?
  • causes?
  • features?
A

Most common cause of TTP: acquired inhibition of ADAMTS13 (usually cleaves vWF multimers)

NOTES
Features:
	• Fever
	• Fluctuating neuro signs (microemboli)
	• MAHA
	• Low plts
	• Renal failure

Causes:

- Post-infection (UTI, GI etc)
- Pregnancy
- Drugs: COCP, cyclosporin, penicillin, clopidogrel, aciclovir
- Tumours
- SLE
- HIV

Pathology
• abnormally large and sticky multimers of vWF cause platelets to clump within vessels
• deficiency of ADAMTS13 (a metalloprotease enzyme) which breakdowns (‘cleaves’) large multimers of von Willebrand’s factor
overlaps with haemolytic uraemic syndrome (HUS)

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

hereditary angioedema

  • pathology?
  • screening?
  • tx?
  • features
A

· C1-INH deficiency (C1 inhibitor)
· C4 = screening (low)

NOTES
	· AD
Ix
	· During an attack: low C1-INH
	· Always: low C2 & low C4

Symptoms
· attacks may be proceeded by painful macular rash
· painless, non-pruritic swelling of subcutaneous/submucosal tissues
○ may affect upper airways, skin or abdominal organs
○ (can occasionally present as abdominal pain due to visceral oedema)
· urticaria is NOT usually a feature

Management
• acute
1. IV C1-inhibitor concentrate
2. If not available: FFP

• prophylaxis: anabolic steroid Danazol may help
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

cryoglobulinaemia

  • symptoms
  • bloods
  • type 1?
  • type 2?
  • type 3?
A

symptoms: • Raynaud’s = type I
• cutaneous: vascular purpura, distal ulceration, ulceration
• arthralgia
• renal involvement (diffuse glomerulonephritis)

Bloods: low complement (esp C4), high ESR

tx: immunosuppression, plasmapharesis

type 1: raynauds ass. IgG or IgM. ass w MM/waldenstrom’s

2: mixed mono&polyclonal. usually RF. hep C, RA, Sjogren’s, lymphoma
3: polyclonal w RF. RA, sjogren’s

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

cryoglobulinaemia

  • symptoms
  • bloods
  • type 1?
  • type 2?
  • type 3?
A

symptoms: • Raynaud’s = type I
• cutaneous: vascular purpura, distal ulceration, ulceration
• arthralgia
• renal involvement (diffuse glomerulonephritis)

Bloods: low complement (esp C4), high ESR

tx: immunosuppression, plasmapharesis

type 1: raynauds ass. IgG or IgM. ass w MM/waldenstrom’s

2: mixed mono&polyclonal. usually RF. hep C, RA, Sjogren’s, lymphoma
3: polyclonal w RF. RA, sjogren’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

sideroblastic anaemia

  • causes?
  • diagnostic feature?
  • bloods?
A

· Acquired causes: alcohol, lead, anti-TB meds, myelodysplasia
· Dx: ring sideroblasts in BM iron/prussian blue stain

more
• FBC: hypochromic microcytic anaemia (more so in congenital)
• iron studies
○ high ferritin
○ high iron
○ high transferrin saturation
• blood film: basophilic stippling of red blood cells

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

spherocytes - ddx (3)

A

AI haemolysis, transfusion reactions & hereditary spherocytosis
If direct coombs test negative = not AI

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

hereditary spherocytosis

  • inheritance?
  • dx?
  • fts
A

AD
Dx: EMA binding test

Presentation 
	• FTT
	• jaundice, gallstones
	• splenomegaly
	• aplastic crisis precipitated by parvovirus infection
	• degree of haemolysis variable
	• MCHC elevated

Diagnosis

- FHx, typical clinical fts, lab Ix (spherocytes, high MCHC, high reticulocytes): no further tests needed
- Equivocal dx: EMA binding test, cryohaemolysis test 
- Atypical presentation: electrophoresis analysis of erythrocyte membranes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what can PRV progress to?

A

myelofibrosis

AML

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

key difference between TACO and TRALI

A

TRALI: hypotension. TACO: hypertension

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

causes of haemolysis

  • intravascular
  • extravascular
A 
Intravascular haemolysis: causes
	• mismatched blood transfusion
	• G6PD deficiency
	• red cell fragmentation: heart valves, TTP, DIC, HUS
	• paroxysmal nocturnal haemoglobinuria
	• cold autoimmune haemolytic anaemia
Extravascular haemolysis: causes
	• haemoglobinopathies: sickle cell, thalassaemia
	• hereditary spherocytosis
	• haemolytic disease of newborn
warm autoimmune haemolytic anaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

most common inherited thrombophilia

A

activated protein C resistance (factor V leiden)

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

most common inherited thrombophilia

A

activated protein C resistance (factor V leiden)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

irradiated blood products

  • key change to them?
  • used to avoid what complication
  • used in what situs
A

Depleted in T-lymphocytes
- used to avoid transfusion-ass graft versus host disease (TA-GVHD) caused by engraftment of viable donor T lymphocytes.

used in intra-uterine, neonates up to 28ds post-delivery, BM/stem cell transplant, immunocomp, previous or current hodgkin’s

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

gene mutations/translocations seen in

  • CML
  • burkitt’s lymphoma
  • acute promyelinocytic leukaemia
A
  • T(9;22): philadelphia chr (BCR-ABL gene) = CML
    • T(15;17): PML and RAR-alpha fused = acute promyelocytic leukaemia (M3)
    • T(8;14): MYC = burkitt’s lymphoma
    • T(14;18): follicular lymphoma (increased BCL-2)
    • T(11;14): mantle cell lymphoma (BCL-1/cyclin D1 gene deregulated)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

most common inherited bleeding disorder

A

von willebrand’s

17
Q

VwD

  • inheritance
  • presentation
  • mx?
  • bloods?
  • what does vwf do?
A

AD
Like a platelet disorder: nose bleeds, menorrhagia, bruising without trauma

Mx:

- Mild bleeding: tranexamic acid
- Desmopressin: induces release of VwF from endothelial cells
- Factor 8 (VIII) concentrate

Ix:

- Long bleeding time
- Can have long APTT
- Can be low Factor VIII

Vwf: carrier molecule for factor 8, promotes plt adhesion to damaged endothelium
80% type 1 vwd = partial reduction in vwf

18
Q

anaphylaxis: do you ever give adrenaline IV?

A

no - always IM

19
Q

neoplastic spinal cord compression

  • lesions above L1: signs?
  • lesions below?
A

neuro signs dep on the level of the lesion.

  • Lesions above L1: UMN signs in the legs and a sensory level.
  • Lesions below L1 usually cause LMN signs in the legs and perianal numbness.
  • Tendon reflexes tend to be increased below the level of the lesion and absent at the level of the lesion

Features
• back pain
○ the earliest and most common symptom
○ may be worse on lying down and coughing
• LL weakness
• sensory changes: sensory loss and numbness

20
Q

li-fraumeni - what mutation?

A

germline mutations to p53 (tumour suppressor)

21
Q

BRCA 1 and 2 - risks of what cancers

A

• Chr 17 (BRCA 1) and Chr 13 (BRCA 2)
• Breast cancer (60%) risk.
• Risk of ovarian cancer (55% with BRCA 1 and 25% with BRCA 2).
BRCA2: prostate cancer in men

22
Q

lynch syndrome: inheritance, what cancers

A
  • AD
    • colon and endometrial cancer at young age
    • 80% of affected individuals will get colonic and/ or endometrial cancer
    • High risk individuals may be identified using the Amsterdam criteria

Amsterdam criteria

- Three or more family members with a confirmed diagnosis of colorectal cancer, one of whom is a first degree (parent, child, sibling) relative of the other two.
- Two successive affected generations.
- One or more colon cancers diagnosed under age 50 years.
- Familial adenomatous polyposis (FAP) has been excluded.
23
Q

gardners syndrome
what is it
features
what mutation

A

• AD familial colorectal polyposis
• Multiple colonic polyps
• Extra colonic diseases include: skull osteoma, thyroid cancer and epidermoid cysts
• Desmoid tumours are seen in 15%
• Mutation of APC gene located on chromosome 5
• Due to colonic polyps most patients will undergo colectomy to reduce risk of colorectal cancer
Now considered a variant of FAP

24
Q

ITP mx

A

Life or organ threatening bleeding: plt transfusion, IV methylpred + IVIg
Plts >30: observe
Asym and <30: oral pred

plt transfusion threshold is 30

25
Q

most common clotting disorder

A

factor V leiden (activated protein C resistance)

26
Q

philadelphia chromosome

  • what is it
  • good prognosis in?
  • bad prog in?
A

t(9;22) - good prognosis in CML, poor prognosis in AML + ALL

27
Q

paroxysmal nocturnal hamoglobinuria

  • dx?
  • features?
  • pathology?
A

· Gold standard dx: flow cytometry for CD59 and CD55

NOTES
Features
· haemolytic anaemia
· Can get pancytopenia (as RBC, WBC, plts or stem cells can be affected)
· haemoglobinuria: dark urine in AM (or throughout the day)
· VTE e.g. Budd-Chiari syndrome
· aplastic anaemia may develop in some patients

DETAILS
· acquired disorder
·&raquo_space;> haemolysis (mainly intravascular) of haematological cells.
· Theory: increased sensitivity of cell membranes to complement due to a lack of glycoprotein glycosyl-phosphatidylinositol (GPI)

28
Q 
what cancers are they ass with
- ebv
- htlv-1
- HIV 1
- helicobacter pylori
malaria
A

Viruses:
- EBV: Hodgkin’s and Burkitt’s lymphoma, nasopharyngeal carcinoma
- HTLV-1: Adult T-cell leukaemia/lymphoma
- HIV-1: High-grade B-cell lymphoma
Bacteria - Helicobacter pylori: gastric lymphoma (MALT)
Protozoa - malaria: Burkitt’s lymphoma

29
Q

neutropenic sepsis

  • most common bacteria?
  • mx?
A

Most common bac: gram +ve cocci (staph epidermis)
If likely to end up neutropenic: offer fluoroquinolone prophylaxis
If suspect, start abx immediately (don’t wait for WBC): Tazocin

30
Q

positive DAT/coombs means?
warm - what Ab, causes?
cold - what Ab, causes?

A

autoimmune haemolytic anaemia

warm: IgG
- SLE, lymphoma, CLL, methyldopa

cold: lymphoma, mycoplasma, EBV. IgM

31
Q

what type of haemolysis is haptoglobin low in?

A

intravascular

causes
mismatched blood transfusion
G6PD deficiency*
red cell fragmentation: heart valves, TTP, DIC, HUS
paroxysmal nocturnal haemoglobinuria
cold autoimmune haemolytic anaemia
32
Q

universal donor of FFP?

A

FFP: AB RhD negative blood
- produce neither anti-A or anti-B so compatible with all

RBC: O neg

33
Q

burkitt’s lymphoma

  • what cell?
  • gene , microscopy, mx?
A
  • C-myc gene translocation
    • Microscopy: starry sky
    • Mx: chemotx (risk of tumour lysis syndrome)

high-grade B-cell neoplasm

34
Q

cisplatin elec abnormality

A

low Mg

35
Q

anastrozole/letrozole - mechanism?

A

aromatase inhibitors that reduces peripheral oestrogen synthesis

36
Q

tamoxifen
- what does it do?
use?

A

SERM = selective oestrogen receptor modulator
- acts as an oestrogen receptor antagonist and partial agonist

use: mx of oestrogen receptor-positive breast cancer.

37
Q

initial mx of suspected neoplastic SC compression

A

high dose dex

then urgent whole spine MRI

38
Q

systemic sclerosis - most common cause of death

A

resp involvement and ultimately failure (due to ILD and pulm arterial hypertension)

39
Q 
acute intermittent porphyria 
- inheritance
- cause
- fts
dx
A

AD
Cause: defect in porphobilinogen deaminase (enz in biosynth of haem)
-&raquo_space; toxic accumulation of delta aminolaevulinic acid and porphobilinogen

PC: abdo + neuropsych syms in 20-40 year olds
- 5x more in women

PC: combo of abdo, neuro and psych symptoms:
	• abdo: abdominal pain, vomiting
	• motor neuropathy
	• psych: e.g. depression
	• hypertension and tachycardia common

DX
• urine turns deep red on standing
• raised urinary porphobilinogen (elevated between attacks and to a greater extent during acute attacks)
• assay of red cells for porphobilinogen deaminase
raised serum levels of delta aminolaevulinic acid and porphobilinogen

40
Q

when do you use cryoprecipitate in bleeding pt

A

low fibrinogen level

MRCP (16 decks)

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