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FRCPath Haematology Part 2 > BCSH Guidelines > Flashcards

BCSH Guidelines Flashcards

1
Q

VWD 2024

Initial panel of tests for VWD

A

FBC and film
PT, APTT, Clauss Fn
FVIII:C
VWF:Ag
VWF:Act (RCo, GP1bR, GP1bM)
Additional tests if Act:Ag <0.7

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2
Q

VWD 2024

Additional tests for VWD T2

A

FVIII:C (low —> T2N)
VWF:CB
Multimers
RIPA if T2B suspected
Genetics
VWF:FVIIIB only if genetics atypical

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3
Q

VWD 2024

Who gets DDAVP test

A

All but T2B and T3

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4
Q

VWD 2024

Diagnosis VWD T3

A

VWF:Ag <1 IU/dL
Confirmed by genetics

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5
Q

VWD 2024

Diagnosis VWD T1

A

VWF:Ag 1-30 IU/dL
VWF:Act 1-30 IU/dL
Act:Ag >0.7
Confirmed by genetics

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6
Q

VWD 2024

Diagnosis VWD T2N

A

VWF Act:Ag <0.7 (Ag might be normal)
FVIII:C <50

Sequence F8 gene and VWF
VWF:FVIIIB only if genetics atypical

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7
Q

VWD 2024

Diagnosis T2A

A

VWF:Ag >1 IU/dL
VWF:Act <30 IU/dL
Act:Ag <0.7

VWF:CB / VWF:Ag reduced
and/or
Multimers reduced

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8
Q

VWD 2024

Diagnosis T2M

A

VWF:Ag >1 IU/dL
VWF:Act <30 IU/dL
Act:Ag <0.7

VWF:CB / VWF:Ag normal
and/or
Multimers normal

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9
Q

VWD 2024

Diagnosis T2B

A

VWF:Ag >1 IU/dL
VWF:Act <30 IU/dL
Act:Ag <0.7
VWF:CB / VWF:Ag any

Genetics
and/or
RIPA - aggregation with low dose Ristocetin

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10
Q

Pre-procedure clotting 2024

How to assess bleeding risk pre-procedure

A

Not clotting screen/platelet count
Used structured questions eg HEMSTOP

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11
Q

Pre-procedure clotting 2024

In what circumstances do you do pre-op coag screen?

A

Procedure with a high risk of bleeding
And
Liver disease, malnutrition, antibiotics, risk of coagulopathy (e.g. sepsis/critical care)

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12
Q

Pre-procedure clotting 2024

Target Fn in unwell patients having procedure

A

> 1g/L

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13
Q

Pre-procedure clotting 2024

Threshold for platelet transfusion for tunnelled line

A

30

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14
Q

Pre-procedure clotting 2024

Threshold for TPO-RA in liver disease

A

<50 for high risk procedure

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15
Q

SMM 2024

Definition SMM

A

PP >30g/L (or BJP >500mg/day)
or
10-59% PCs in BM
and
No SLiM CRAB criteria

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16
Q

SMM 2024

SLiM CRAB criteria

A

S - >60% PCs in BM
Li - LC ratio >100
M - >1 MRI lesions >5mm
C - Hypercalcaemia
R - CrCl <40ml/min or creat >177
A - H <100
B - Lytic lesion >5mm

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17
Q

SMM 2024

When do renal biopsy

A

If using R criteria and SFLC <500mg/L

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18
Q

SMM 2024

3 tests to rule in/out amyloid

A

BNP
Trop
ACR

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19
Q

SMM 2024

IMWG risk SMM

A

Risk factors
PC >20%
PP >20g/L
SFLCr >20
CNA inc 14;14, 14;16, gain1q, -13/del13q

Low 0RF
Low-int 1RF
HIgh-int 2RF
High 3 RF

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20
Q

DBLCL 2024

Diagnostic workup DLBCL

A

Excision biopsy, or core if excision difficult
Sample analysed in SIHMDS and discuss in MDT
Baseline bloods inc LDH, HepB, HepC, HIV
PET-CT baseline or CT-NTAP if not possible
Contrast MRI brain if clinical suspicion CNS
Baseline ECG, consider echo
FISH for MYCr then BCL2 + BCL6 if positive
Determine COO

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21
Q

DLBCL 2024

IPI

A

Age >60
Stage III-IV
ECOG >1
LDH >ULN
>1 EN site

Out of 5

CNS-IPI
Same with extra point for kidneys/adrenal

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22
Q

DLBCL 2024

Primary prophylaxis

A

Aciclovir and septrin
Give all GCSF

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23
Q

DLBCL 2024

Supportive considerations for elderly

A

Bone health
Speciality referral e.g. cardiology

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24
Q

DLBCL 2024

1st line 18-60 Stage I/II, IPI 0, non-bulk

A

4x R-CHOP + 2x R

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25
Q

DLBCL 2024

1st line >60yrs, Stage I/II, IPI 0

A

2x R-CHOP
iPET2
If CMR give 2x R-CHOP
If <CMR give 4x R-CHOP + RT

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26
Q

DLBCL 2024

1st line <60yrs, Stage I/II, IPI 1

A

4x R-CHOP + RT

or

6x R-CHOP

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27
Q

DLBCL 2024

1st line <80yrs, Stage I/II, IPI >1

A

6x R-Pola-CHP +/- RT for baseline bulk
EOT PET-CT

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28
Q

DLBCL 2024

When to incorporate RT 1st line

A

Reduced intensity chemo
EN disease
Bulky disease (>7.5cm)

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29
Q

DLBCL 2024

1st line treatment for EN disease

A

Testes - 6x R-CHOP + CNS prophy + RT contralateral testis

Breast - 6x R-CHOP + RT + CNS prophy

Gastric - 6x R-CHOP + eradicate h. pylori

Intravascular - CNS directed protocol if evidence CNS disease

Leg type - 6x R-CHOP + RT

Bone - 6x R-CHOP + RT

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30
Q

DLBCL 2024

Advanced disease (Stage III/IV) treatment options

A

6x R-CHOP if IPI <2
6x R-Pola-CHP if IPI >1
Consider R-CODOX-M/R-IVAC for younger high risk
DA-EPOCH-R if double hit

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31
Q

DLBCL 2024

1st line treatment elderly (+/- cardiac problems)

A

R-miniCHOP (50% cyclo, doxo 25mg/m^2, vinc 1mg)
R-GCVP for cardiac patient

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32
Q

DLBCL 2024

EOT response

A

Do EOT PET-CT 6 weeks post chemo and BEFORE RT or HD-MTX
Review MDT
Deauville 1-3 = CMR
If <CMR do follow up scan or repeat biopsy

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33
Q

AA 2024

Camitta criteria

A

NSAA
BM cellularity <25%

SAA
NSAA + 2/3
Retic <60
Plat <20
Neut <0.5

VSAA
SAA + neut <0.2

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34
Q

AA 2024

Camitta stratified treatment AA

A

NSAA
CSA + horse ATG if transfusion dependent, bleeding or recurrent infections

SAA/VSAA
If MSD and <40 or 40-50 and fit —> upfront HSCT
CSA + horse ATG + eltrombopag if no MSD
CSA + horse ATG + eltrombopag if MSD and >40

ATG - patient must be stable, ideally afebrile, plt >20, prophylactic antibiotics, given in experiences centre

MUD/alternative
If young/fit upfront
In others if failed CSA-ATG

Syngeneic
Consider in all

Pregnant
Supportive transfusion
Can use CSA if needed

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35
Q

AA 2024

Camitta stratified treatment AA

A

NSAA
CSA + horse ATG if transfusion dependent, bleeding or recurrent infections

SAA/VSAA
If MSD and <40 or 40-50 and fit —> upfront HSCT
CSA + horse ATG + eltrombopag if no MSD
CSA + horse ATG + eltrombopag if MSD and >40

ATG - patient must be stable, ideally afebrile, plt >20, prophylactic antibiotics, given in experiences centre

MUD/alternative
If young/fit upfront
In others if failed CSA-ATG

Syngeneic
Consider in all

Pregnant
Supportive transfusion
Can use CSA if needed

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36
Q

HIT 2023

When and how to test

A

4Ts 4 or more
or
ECMO or critically ill

Test with screen e.g. lat-flow, ELISA
Follow up e.g. chemiluminescent or functional assay at specialist centre

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37
Q

HIT 2023

Management

A

3 months full anticoag if clot
1 month full if no clot or until platelets recover

Argatroban infusion if unwell and kidneys OK
Biavlirudin of unwell and liver OK
Fondaparinux if not unwell
Convert to DOAC once fit

IVIg for autoimmune or VITT

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38
Q

HIT 2023

Re-exposure

A

Use alternative if possible
If need heparin eg bypass and antibody negative proceed using alternatives pre- and post-infusion
If still ab positive and need eg bypass use bivalirudin
PLEX + IVIg if above but can’t get bivalirudin
Agatroban or danaparoid for CVVHF

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39
Q

HIT 2023

Pregnancy

A

Fondaparinux but beware long half life (42 hours before regional technique)

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40
Q

MF 2023

Indications ruxolitinib

A

Any grade with splenomegaly or symptoms

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41
Q

MF 2023

Infectious risk mitigation ruxolitinib

A

Baseline HIV, HepB, HepC

Individualised plan TB risk

Individualised herpes virus plan

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42
Q

MF 2023

Non haem, non-infectious complication ruxolitinib

A

Non-melanoma skin cancer

Need individualised surveillance (i.e. those with hx skin cancer or those with actinic keratosis)

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43
Q

MF 2023

Ruxo dosing for platelet ranges

A

> 200 : 20mg BD
100-200 : 15mg BD
75-99 : 10mg BD
50-74 : 5mg BD

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44
Q

MF 2023

Options for platelets <50

A

Start danazol then add ruxo if platelets respond

Off SpC license - low dose ruxo with monitoring

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45
Q

MF 2023

Fedratinib indication

A

Disease related splenomegaly
Or those resistant to ruxo

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46
Q

MF 2023

Momelotinib indication

A

Consider 1st line for MF with splenomegaly and anaemia

Not routinely available in UK

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47
Q

MF 2023

Role for EPO

A

Alone or with ruxo if low EPO level

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48
Q

MF 2023

Role for danazol

A

Alone or with ruxo if EPO doesn’t work

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49
Q

MF 2023

Role for chelation

A

Consider in iron overloaded patients being worked up for alloSCT

50
Q

MZL 2023

Diagnostic workup

A

Biopsy for EMZL/NMZL
Blood flow/BM biopsy for SMZL

CT NTAP, PET only if HG transformation expected

BMAT for SMZL/NMZL if cytopenias, not needed for gastric EMZL

OGD for gastric EMZL

FISH for t(11;18)

51
Q

MZL 2023

Gastric MZL management

A

H pylori eradication for all

OGD 3-6 months after

ISRT for ongoing disease post eradication

12 month surveillance OGD in remission or asymptomatic

52
Q

MZL 2023

Non-gastric MALT management

A

Antiviral for hep C
Antibiotics for eyes
Antibiotics if evidence chronic infection

RT otherwise

53
Q

MZL 2023

Management advanced EMZL/NMZL

A

R-Chlorambucil

54
Q

MZL 2023

Management SMZL

A

R monotherapy

55
Q

MCL 2023

Diagnostic workup

A

Clinical assessment
Bloods inc LDH, hepB/C, HIV
Histology inc Ki67, CyclinD1 (t(11;14)), SOX11
TP53 sequencing
Frailty assessment
CTNTAP or PET
Consider BMAT for staging or if cytopenic (PET not sensitive to rule out)
LP + CNS imaging if CNS symptoms

56
Q

MCL 2023

Localised disease management

A

Need full staging workup
Consider local RT

57
Q

MCL 2023

1st line in young/fit

A

Rituximab + HD cytarabine containing regimen
AutoSCT in CR1
Maintenance R
If TP53mut then trial for consolidatoin
Add inrutinib to R-CHOP part + consolidation if accessible

58
Q

MCL 2023

1st line in transplant ineligible

A

R-chemo
R maintenance

R-miniCHOP or similar for frail

59
Q

MCL 2023

Management indolent MCL

A

W+W if low volume nodal or isolated spleen/BM/blood

60
Q

MCL 2023

Management relapse

A

Ibrutinib unless used 1st line
Ibrutinib good for CNS relapse

61
Q

MCL 2023

Indications for brexu-cel

A

Relapsed post CD20 + BTKi
Lack of early response to BTKi in 2nd line

62
Q

TTP 2023

Diagnosis + initial workup

A

Diagnosis is based on clinical and blood film findings

Coag screen normal

Send pre-treatment ADMATS13 assay

Do HepB/C, HIV and autoimmune screens

Do pregnancy test

ADAMTS13 <10 IU/dl is highly sensitive and specific

63
Q

TTP 2023

Initial management

A

Emergency
Time-ciritcal transfer to treatment centre
Intubate in local centre if needed
PLEX start within 4-8 hours
Do not give platelets

64
Q

TTP 2023

Comprehensive management

A

Start Caplcacizumab on confirmation for 30 days, continue if ADAMTS13 stays low
Methylpred 1g/day for 3 days
PLEX with octaplas, 1.5xPV exchange
Stop PLEX when plt >150
Start rituximab within 3 days
Thromboprophylaxis once plt >50

65
Q

Pregnant MHV 2023

Pre-pregnancy management

A

Counsel before valve insertion if CBA
Counsel as soon as become CBA

66
Q

Pregnant MHV 2023

Antenatal management

A

Tertiary centre with obs, cardio, thrombosis, cardiac surgery, neonatal and anaesthetics

Counsel about risks of low adherence i.e. valve thrombosis and associated morbidity

VKA best for valve but increased risk of pregnancy loss or neonatal morbidity

Convert VKA to LMWH once pregnant, ideally before 6/40

LMWH throughout with 2.5mg/kg/day enoxaparin (250IU/kg/day others) + aspirin 75mg (150mg for pre-eclampsia prophylaxis)

Higher dose LMWH because high rate thrombosis during transition

67
Q

Pregnant MHV 2023

Birth plan

A

Tertiary centre with obs, cardio, thrombosis, cardiac surgery, neonatal and anaesthetics

Individualised plan, documented + emergency plan

For VKA - convert to LMWH 2/52 before birth (if present in labour go for CS)

For LMWH - stop 24 hours before CS or when start labour

If prolonged LMWH disruption consider prophylactic dose

For aspirin - stop 3 days prior to delivery

Restart prophylactic LMWH for first 24-28 hours post delivery

VKA restart 7 days post delivery

Reassess plan for next pregnancy if relevant

68
Q

MGUS 2023

Workup

A

IgG, IgG, IgM AND SPEP
Immunofixation (more sensitive than SPEP) blood and urine
Serum freelite
FBC
Renal function
Corrected calcium

69
Q

MGUS 2023

Mayo risk criteria

A

M-protein >15g/L
Non IgG
Abnormal SFLCr

Low - 0 - 2% - 20yrs
Low-int - 1 - 10% 20 years
High-int - 2 - 18% 20 years
High - 3 - 27% 20 years

70
Q

MGUS 2023

Indications for BMAT + imaging

A

Int-high or high

71
Q

MGUS 2023

Follow up

A

All patients: repeat bloods in 6 months
Thereafter yearly follow up
Don’t discharge int-high or high

72
Q

ATR 2023

Mandatory training

A

Recommended for all staff in clinical or laboratory areas involved in transfusion

73
Q

ATR 2023

Immediate management

A

Stop transfusion, maintaining venous access
Double check ID band and label
Visually inspect unit
Assess patient A-E with obs

If temp <2deg and/or pruritus and/or rash: continue with supportive care
Fever: give paracetamol
Allergic type reaction: give antihistamine (not steroids)

Anaphylactic reaction:
Give IM adrenaline regardless of platelet count

Hypotension:
Consider if this is symptom of blood or indication for blood

Sustained temp >2deg with other symptoms:
Think haemolysis or infection

74
Q

ATR 2023

Investigations

A

FBC
Renal
Liver
CXR if resp symptoms

For moderate/severe:
Return blood
Repeat G+S with compatibility testing on unit (not needed for allergy symptoms alone)
Culture
DAT, LDH haptoglobin
If DAT positive/stronger do elution
Coag screen
Urine for haemoglobin
IgA levels
Consider withdraw associated components
Anti-HLA/HNA/HPA only for appropriate contexts
Consider mast cell tryptase

For resp symptoms not associated with allergy:
O2 sats, ABG
BNP (and request add-on to pre-transfusion)
Echo

75
Q

ATR 2023

Management recurrent fever

A

Try prophylactic paracetamol or NSAID
If doesn’t work try washed components

76
Q

ATR 2023

Recurrent mild allergy

A

Not for prophylaxis
Exclude other aetiologies

77
Q

ATR 2023

Recurrent mod-severe allergy

A

If react to apheresis platelets, try pooled in PAS
Antihistamine prophylaxis
Not for routine steroids
Washed units
Octaplas if allergy to FFP
Transfuse in suitable area

78
Q

ATR 2023

IgA deficiency

A

If history of anaphylaxis:
Washed components for elective transfusion
Do not delay emergency transfusion

If no history:
Standard components
Increased monitoring

79
Q

ATR 2023

Reporting

A

All but mild febrile/allergic reactions via SABRE
Review within hospital transfusion team

80
Q

BSH/BSIR 2023

HEMSTOP questionnaire

A

Seen doctor for bleeding?
2cm bruise without trauma?
Bled after dentist needing intervention?
Bleeding after surgery?
FHx bleeding disorder?
Seen doctor for heavy periods?
Prolonged PPH?

0-1: no coag screen (FBC for mod risk only)
2+: Coag screen + haem opinion

81
Q

BSH/BSIR 2023

Low risk interventions

A

Venous
Superficial biopsy
GI tract
MSK
US drain

82
Q

BSH/BSIR 2023

Med risk interventions

A

Low gauge arterial
Embolisation
Dialysis access
Tunnelled line

83
Q

BSH/BSIR 2023

High risk interventions

A

High gauge arterial
Aortic
Tumour ablation
Renal biopsy/stent
TIPSS/TJ biopsy
Liver biopsy

84
Q

BSH/BSIR 2023

Pre-procedure blood thresholds

A

Low risk: none

Med risk: Hb >70, plt >50, INR <2 on warfarin

High risk: Med risk but INR <1.5

85
Q

BSH/BSIR 2023

Corrections for liver disease blood params

A

Fn >1.2g/L
Plts >50
HCT >0.25

86
Q

BSH/BSIR 2023

Anticoag hold/restart times

A

See photo

87
Q

BSH iron chelation Hb-opathy 2021

Complication IOL

A

Hypogonadotrophic hypogonadism

Hypothyroid/parathyroid

Diabetes

Cardiac siderosis - failure/arrythmia

HCC

88
Q

BSH iron chelation Hb-opathy 2021

Surrogate marker for other complications

A

Liver iron concentration

89
Q

BSH iron chelation Hb-opathy 2021

Threshold for risk of IOL

A

Transfusional: blood at least 1 unit per 3 months

Non-transfusional:
NTDT, NTRIA

90
Q

BSH iron chelation Hb-opathy 2021

Frequency serum ferritin

A

1-3 monthly

91
Q

BSH iron chelation Hb-opathy 2021

Frequency MRI cardiac T2* + LVEF

A

Baseline by age 8

2-yearly if T2* >20ms
Annual if T2* 10-20ms
6 monthly if T2* <10ms

92
Q

BSH iron chelation Hb-opathy 2021

Frequency Liver R2 (ferriscan) or T2*

A

Baseline by age 8

T2* - do with cardiac

2-yearly if 7 mg/g
Annual if 7-15 mg/g
6 monthly if >15 mg/g

93
Q

BSH iron chelation Hb-opathy 2021

Endocrine monitoring

A

6 monthly height and weight

Annual pubertal status, OGTT, TFT, cortisol, gonad function

Annual Vit D from age 2

94
Q

BSH iron chelation Hb-opathy 2021

Non-endocrine components annual review

A

Calculate rate of iron loading based on transfusions

Seen by cardiology starting age 16
ECG + echo
Hepatitis serology
LFTS (more frequent)

Increase monitoring if poor chelation

95
Q

BSH iron chelation Hb-opathy 2021

Liver complications

A

Cirrhosis when LIC >7mg/g
HCC

96
Q

BSH iron chelation Hb-opathy 2021

Triggers for assessment

A

SCD: regular top ups or if rising ferritin

NTDT: ferritin >800

NTRIA: ferritin >1000

97
Q

BSH iron chelation Hb-opathy 2021

Thresholds for chelation

A

TDT: 10-12 units blood or ferritin >1000 (x2)

NTDT: ferritin >800 or LIC >5mg/g

NTRIA: chelation or venesection if ferritin >500 or LIC > 5 mg/g

SCD: top up chelate as per TDT, RCEX - individualised plan

98
Q

BSH iron chelation Hb-opathy 2021

Chelation options

A

Age <6
SC desferrioxamine

Age >6
Deferasirox-FCT (tablet)

2nd line for any
Desferrioxamine + deferipone
Deferasirox + desferrioxamine
Deferasirox + deferipone

99
Q

BSH/SHOT anti-D pregnancy

PSE types <12 weeks

A

Surgical managed abortion, miscarriage, ectopic or molar

Medical abortion 10-12 weeks

100
Q

BSH/SHOT anti-D pregnancy

PSE management before 20 weeks (includes PSE pre-12 weeks)

A

500 IU anti-D within 72 hours
Kleihauer not needed

101
Q

BSH/SHOT anti-D pregnancy

Management continual uterine bleeding

A

500 IU anti-D every 6 weeks
Kleihauer every 2 weeks from 20 weeks onwards with extra anti-D as needed (then repeat after 72 hours)

102
Q

BSH/SHOT anti-D pregnancy

RAADP

A

1500 IU anti-D at 28-30 weeks

103
Q

BSH/SHOT anti-D pregnancy

Delivery (or intrauterine death >20 weeks)

A

Do Kleihauer test
Give 500 IU
If Kleihauer test indicates, give more anti-D
Repeat Kleihauer after 72 hours

If cell salvage used: give 1500 IU anti-D, do Kleihauer

104
Q

BSH/SHOT anti-D pregnancy

Delivery (or intrauterine death >20 weeks)

A

Do Kleihauer test
Give 500 IU
If Kleihauer test indicates, give more anti-D
Repeat Kleihauer after 72 hours

If cell salvage used: give 1500 IU anti-D, do Kleihauer

105
Q

BSH/SHOT anti-D pregnancy

Threshold FMH for flow assessment

A

> 2ml

106
Q

BSH/SHOT anti-D pregnancy

Dose anti-D

A

125 IU/ml (IM)
100 IU/ml (IV)

107
Q

BSH/SHOT anti-D pregnancy

Practical consideration anti-D

A

Blood derived product
Consent
Fever/headache
Can cause allergic reaction

108
Q

GTG65

Referral thresholds MoM, D, c, K, other

A

MoM - 1.5
anti-D - 4
anti-c - 7.5 (or lower if also anti-E)
anti-K - any
anti-other - 1/32

109
Q

GTG65

Which abs have increased monitoring?

A

anti-c
anti-D
anti-K
Previous history of HDFN (refer any)

110
Q

GTG65

Frequency of cross matching if high risk of need for blood and allo-ab

A

Weekly!

111
Q

GTG65

What to warn obs team about mother if allo-abs

A

Increased risk of needing blood
Have blood ready

112
Q

GTG65

What to send off on cord blood?

A

Hb
DAT
Bilirubin

113
Q

GTG65

What to tell paeds team

A

Baby at risk for up to few weeks, monitor

114
Q

CLL 2022

First line treatments unfit

A

Ven-O
Acalabrutinib
Ibru-ven
Zanubrutinib
Regardless of TP53 status

115
Q

CLL 2022

First line treatments fit

A

TP53 wt
FCR only if IGHV mutated
Ven-O
Ibru-Ven

TP53 mut
Acalabrutinib
Ven-O
Ibru-ven
Zanubrutinib

116
Q

CLL 2022

Vaccinations at diagnosis

A

Prevenar then pneumovax
Annual flu
Covid

117
Q

CLL 2022

Antimicrobials

A

Prophylaxis if IgG <4g/L
IVIg if no response

118
Q

BSH CNS prophylaxis 2020

Criteria for prophylaxis

A

CNS-IPI 4-6
3 or more EN sites
Testes, renal/adrenal, intravascular

119
Q

BSH CNS prophylaxis 2020

Sites to consider prophylaxis

A

Breast
Uterus

120
Q

BSH CNS prophylaxis

How to deliver

A

2x cycles HD MTX >3g/m2
Intercalate or at end
Add IT if testicular
IT only if CrCl <50

FRCPath Haematology Part 2 (11 decks)

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