BCSH Guidelines Flashcards
VWD 2024
Initial panel of tests for VWD
FBC and film
PT, APTT, Clauss Fn
FVIII:C
VWF:Ag
VWF:Act (RCo, GP1bR, GP1bM)
Additional tests if Act:Ag <0.7
VWD 2024
Additional tests for VWD T2
FVIII:C (low —> T2N)
VWF:CB
Multimers
RIPA if T2B suspected
Genetics
VWF:FVIIIB only if genetics atypical
VWD 2024
Who gets DDAVP test
All but T2B and T3
VWD 2024
Diagnosis VWD T3
VWF:Ag <1 IU/dL
Confirmed by genetics
VWD 2024
Diagnosis VWD T1
VWF:Ag 1-30 IU/dL
VWF:Act 1-30 IU/dL
Act:Ag >0.7
Confirmed by genetics
VWD 2024
Diagnosis VWD T2N
VWF Act:Ag <0.7 (Ag might be normal)
FVIII:C <50
Sequence F8 gene and VWF
VWF:FVIIIB only if genetics atypical
VWD 2024
Diagnosis T2A
VWF:Ag >1 IU/dL
VWF:Act <30 IU/dL
Act:Ag <0.7
VWF:CB / VWF:Ag reduced
and/or
Multimers reduced
VWD 2024
Diagnosis T2M
VWF:Ag >1 IU/dL
VWF:Act <30 IU/dL
Act:Ag <0.7
VWF:CB / VWF:Ag normal
and/or
Multimers normal
VWD 2024
Diagnosis T2B
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
Pre-procedure clotting 2024
How to assess bleeding risk pre-procedure
Not clotting screen/platelet count
Used structured questions eg HEMSTOP
Pre-procedure clotting 2024
In what circumstances do you do pre-op coag screen?
Procedure with a high risk of bleeding
And
Liver disease, malnutrition, antibiotics, risk of coagulopathy (e.g. sepsis/critical care)
Pre-procedure clotting 2024
Target Fn in unwell patients having procedure
> 1g/L
Pre-procedure clotting 2024
Threshold for platelet transfusion for tunnelled line
30
Pre-procedure clotting 2024
Threshold for TPO-RA in liver disease
<50 for high risk procedure
SMM 2024
Definition SMM
PP >30g/L (or BJP >500mg/day)
or
10-59% PCs in BM
and
No SLiM CRAB criteria
SMM 2024
SLiM CRAB criteria
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
SMM 2024
When do renal biopsy
If using R criteria and SFLC <500mg/L
SMM 2024
3 tests to rule in/out amyloid
BNP
Trop
ACR
SMM 2024
IMWG risk SMM
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
DBLCL 2024
Diagnostic workup DLBCL
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
DLBCL 2024
IPI
Age >60
Stage III-IV
ECOG >1
LDH >ULN
>1 EN site
Out of 5
CNS-IPI
Same with extra point for kidneys/adrenal
DLBCL 2024
Primary prophylaxis
Aciclovir and septrin
Give all GCSF
DLBCL 2024
Supportive considerations for elderly
Bone health
Speciality referral e.g. cardiology
DLBCL 2024
1st line 18-60 Stage I/II, IPI 0, non-bulk
4x R-CHOP + 2x R
DLBCL 2024
1st line >60yrs, Stage I/II, IPI 0
2x R-CHOP
iPET2
If CMR give 2x R-CHOP
If <CMR give 4x R-CHOP + RT
DLBCL 2024
1st line <60yrs, Stage I/II, IPI 1
4x R-CHOP + RT
or
6x R-CHOP
DLBCL 2024
1st line <80yrs, Stage I/II, IPI >1
6x R-Pola-CHP +/- RT for baseline bulk
EOT PET-CT
DLBCL 2024
When to incorporate RT 1st line
Reduced intensity chemo
EN disease
Bulky disease (>7.5cm)
DLBCL 2024
1st line treatment for EN disease
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
DLBCL 2024
Advanced disease (Stage III/IV) treatment options
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
DLBCL 2024
1st line treatment elderly (+/- cardiac problems)
R-miniCHOP (50% cyclo, doxo 25mg/m^2, vinc 1mg)
R-GCVP for cardiac patient
DLBCL 2024
EOT response
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
AA 2024
Camitta criteria
NSAA
BM cellularity <25%
SAA
NSAA + 2/3
Retic <60
Plat <20
Neut <0.5
VSAA
SAA + neut <0.2
AA 2024
Camitta stratified treatment AA
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
AA 2024
Camitta stratified treatment AA
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
HIT 2023
When and how to test
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
HIT 2023
Management
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
HIT 2023
Re-exposure
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
HIT 2023
Pregnancy
Fondaparinux but beware long half life (42 hours before regional technique)
MF 2023
Indications ruxolitinib
Any grade with splenomegaly or symptoms
MF 2023
Infectious risk mitigation ruxolitinib
Baseline HIV, HepB, HepC
Individualised plan TB risk
Individualised herpes virus plan
MF 2023
Non haem, non-infectious complication ruxolitinib
Non-melanoma skin cancer
Need individualised surveillance (i.e. those with hx skin cancer or those with actinic keratosis)
MF 2023
Ruxo dosing for platelet ranges
> 200 : 20mg BD
100-200 : 15mg BD
75-99 : 10mg BD
50-74 : 5mg BD
MF 2023
Options for platelets <50
Start danazol then add ruxo if platelets respond
Off SpC license - low dose ruxo with monitoring
MF 2023
Fedratinib indication
Disease related splenomegaly
Or those resistant to ruxo
MF 2023
Momelotinib indication
Consider 1st line for MF with splenomegaly and anaemia
Not routinely available in UK
MF 2023
Role for EPO
Alone or with ruxo if low EPO level
MF 2023
Role for danazol
Alone or with ruxo if EPO doesn’t work
MF 2023
Role for chelation
Consider in iron overloaded patients being worked up for alloSCT