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
MZL 2023
Diagnostic workup
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)
MZL 2023
Gastric MZL management
H pylori eradication for all
OGD 3-6 months after
ISRT for ongoing disease post eradication
12 month surveillance OGD in remission or asymptomatic
MZL 2023
Non-gastric MALT management
Antiviral for hep C
Antibiotics for eyes
Antibiotics if evidence chronic infection
RT otherwise
MZL 2023
Management advanced EMZL/NMZL
R-Chlorambucil
MZL 2023
Management SMZL
R monotherapy
MCL 2023
Diagnostic workup
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
MCL 2023
Localised disease management
Need full staging workup
Consider local RT
MCL 2023
1st line in young/fit
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
MCL 2023
1st line in transplant ineligible
R-chemo
R maintenance
R-miniCHOP or similar for frail
MCL 2023
Management indolent MCL
W+W if low volume nodal or isolated spleen/BM/blood
MCL 2023
Management relapse
Ibrutinib unless used 1st line
Ibrutinib good for CNS relapse
MCL 2023
Indications for brexu-cel
Relapsed post CD20 + BTKi
Lack of early response to BTKi in 2nd line
TTP 2023
Diagnosis + initial workup
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
TTP 2023
Initial management
Emergency
Time-ciritcal transfer to treatment centre
Intubate in local centre if needed
PLEX start within 4-8 hours
Do not give platelets
TTP 2023
Comprehensive management
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
Pregnant MHV 2023
Pre-pregnancy management
Counsel before valve insertion if CBA
Counsel as soon as become CBA
Pregnant MHV 2023
Antenatal management
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
Pregnant MHV 2023
Birth plan
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
MGUS 2023
Workup
IgG, IgG, IgM AND SPEP
Immunofixation (more sensitive than SPEP) blood and urine
Serum freelite
FBC
Renal function
Corrected calcium
MGUS 2023
Mayo risk criteria
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
MGUS 2023
Indications for BMAT + imaging
Int-high or high
MGUS 2023
Follow up
All patients: repeat bloods in 6 months
Thereafter yearly follow up
Don’t discharge int-high or high
ATR 2023
Mandatory training
Recommended for all staff in clinical or laboratory areas involved in transfusion
ATR 2023
Immediate management
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
ATR 2023
Investigations
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
ATR 2023
Management recurrent fever
Try prophylactic paracetamol or NSAID
If doesn’t work try washed components
ATR 2023
Recurrent mild allergy
Not for prophylaxis
Exclude other aetiologies
ATR 2023
Recurrent mod-severe allergy
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
ATR 2023
IgA deficiency
If history of anaphylaxis:
Washed components for elective transfusion
Do not delay emergency transfusion
If no history:
Standard components
Increased monitoring
ATR 2023
Reporting
All but mild febrile/allergic reactions via SABRE
Review within hospital transfusion team
BSH/BSIR 2023
HEMSTOP questionnaire
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
BSH/BSIR 2023
Low risk interventions
Venous
Superficial biopsy
GI tract
MSK
US drain
BSH/BSIR 2023
Med risk interventions
Low gauge arterial
Embolisation
Dialysis access
Tunnelled line
BSH/BSIR 2023
High risk interventions
High gauge arterial
Aortic
Tumour ablation
Renal biopsy/stent
TIPSS/TJ biopsy
Liver biopsy
BSH/BSIR 2023
Pre-procedure blood thresholds
Low risk: none
Med risk: Hb >70, plt >50, INR <2 on warfarin
High risk: Med risk but INR <1.5
BSH/BSIR 2023
Corrections for liver disease blood params
Fn >1.2g/L
Plts >50
HCT >0.25
BSH/BSIR 2023
Anticoag hold/restart times
See photo
BSH iron chelation Hb-opathy 2021
Complication IOL
Hypogonadotrophic hypogonadism
Hypothyroid/parathyroid
Diabetes
Cardiac siderosis - failure/arrythmia
HCC
BSH iron chelation Hb-opathy 2021
Surrogate marker for other complications
Liver iron concentration
BSH iron chelation Hb-opathy 2021
Threshold for risk of IOL
Transfusional: blood at least 1 unit per 3 months
Non-transfusional:
NTDT, NTRIA
BSH iron chelation Hb-opathy 2021
Frequency serum ferritin
1-3 monthly
BSH iron chelation Hb-opathy 2021
Frequency MRI cardiac T2* + LVEF
Baseline by age 8
2-yearly if T2* >20ms
Annual if T2* 10-20ms
6 monthly if T2* <10ms
BSH iron chelation Hb-opathy 2021
Frequency Liver R2 (ferriscan) or T2*
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
BSH iron chelation Hb-opathy 2021
Endocrine monitoring
6 monthly height and weight
Annual pubertal status, OGTT, TFT, cortisol, gonad function
Annual Vit D from age 2
BSH iron chelation Hb-opathy 2021
Non-endocrine components annual review
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
BSH iron chelation Hb-opathy 2021
Liver complications
Cirrhosis when LIC >7mg/g
HCC
BSH iron chelation Hb-opathy 2021
Triggers for assessment
SCD: regular top ups or if rising ferritin
NTDT: ferritin >800
NTRIA: ferritin >1000
BSH iron chelation Hb-opathy 2021
Thresholds for chelation
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
BSH iron chelation Hb-opathy 2021
Chelation options
Age <6
SC desferrioxamine
Age >6
Deferasirox-FCT (tablet)
2nd line for any
Desferrioxamine + deferipone
Deferasirox + desferrioxamine
Deferasirox + deferipone
BSH/SHOT anti-D pregnancy
PSE types <12 weeks
Surgical managed abortion, miscarriage, ectopic or molar
Medical abortion 10-12 weeks
BSH/SHOT anti-D pregnancy
PSE management before 20 weeks (includes PSE pre-12 weeks)
500 IU anti-D within 72 hours
Kleihauer not needed
BSH/SHOT anti-D pregnancy
Management continual uterine bleeding
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)
BSH/SHOT anti-D pregnancy
RAADP
1500 IU anti-D at 28-30 weeks
BSH/SHOT anti-D pregnancy
Delivery (or intrauterine death >20 weeks)
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
BSH/SHOT anti-D pregnancy
Delivery (or intrauterine death >20 weeks)
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
BSH/SHOT anti-D pregnancy
Threshold FMH for flow assessment
> 2ml
BSH/SHOT anti-D pregnancy
Dose anti-D
125 IU/ml (IM)
100 IU/ml (IV)
BSH/SHOT anti-D pregnancy
Practical consideration anti-D
Blood derived product
Consent
Fever/headache
Can cause allergic reaction
GTG65
Referral thresholds MoM, D, c, K, other
MoM - 1.5
anti-D - 4
anti-c - 7.5 (or lower if also anti-E)
anti-K - any
anti-other - 1/32
GTG65
Which abs have increased monitoring?
anti-c
anti-D
anti-K
Previous history of HDFN (refer any)
GTG65
Frequency of cross matching if high risk of need for blood and allo-ab
Weekly!
GTG65
What to warn obs team about mother if allo-abs
Increased risk of needing blood
Have blood ready
GTG65
What to send off on cord blood?
Hb
DAT
Bilirubin
GTG65
What to tell paeds team
Baby at risk for up to few weeks, monitor
CLL 2022
First line treatments unfit
Ven-O
Acalabrutinib
Ibru-ven
Zanubrutinib
Regardless of TP53 status
CLL 2022
First line treatments fit
TP53 wt
FCR only if IGHV mutated
Ven-O
Ibru-Ven
TP53 mut
Acalabrutinib
Ven-O
Ibru-ven
Zanubrutinib
CLL 2022
Vaccinations at diagnosis
Prevenar then pneumovax
Annual flu
Covid
CLL 2022
Antimicrobials
Prophylaxis if IgG <4g/L
IVIg if no response
BSH CNS prophylaxis 2020
Criteria for prophylaxis
CNS-IPI 4-6
3 or more EN sites
Testes, renal/adrenal, intravascular
BSH CNS prophylaxis 2020
Sites to consider prophylaxis
Breast
Uterus
BSH CNS prophylaxis
How to deliver
2x cycles HD MTX >3g/m2
Intercalate or at end
Add IT if testicular
IT only if CrCl <50
Infection in hyposplenic 2024
General points
Written info to patient, record updated
Carry card, buy bracelet
Education re travel esp malaria
Vaccination up to date
Register of at-risk patients locally
Infection in hyposplenic 2024
Vaccinations
-Pneumococcus, 23 valent start at 2 then booster every 5 years
-Meningococcus
-Annual flu
-Hib as per childrens schedule, not for older
Infection in hyposplenic 2024
Prophylactic antibiotics
Lifelong penicillin or macrolide
Protects against pneumococcus if high risk
Also carry supply of rescue antibiotics
Infection in hyposplenic 2024
Management of new infection
Hospitalise urgently
Prompt IV antibiotics
