BCSH Guidelines Flashcards

(124 cards)

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
DLBCL 2024 1st line >60yrs, Stage I/II, IPI 0
2x R-CHOP iPET2 If CMR give 2x R-CHOP If
26
DLBCL 2024 1st line <60yrs, Stage I/II, IPI 1
4x R-CHOP + RT or 6x R-CHOP
27
DLBCL 2024 1st line <80yrs, Stage I/II, IPI >1
6x R-Pola-CHP +/- RT for baseline bulk EOT PET-CT
28
DLBCL 2024 When to incorporate RT 1st line
Reduced intensity chemo EN disease Bulky disease (>7.5cm)
29
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
30
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
31
DLBCL 2024 1st line treatment elderly (+/- cardiac problems)
R-miniCHOP (50% cyclo, doxo 25mg/m^2, vinc 1mg) R-GCVP for cardiac patient
32
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
33
AA 2024 Camitta criteria
NSAA BM cellularity <25% SAA NSAA + 2/3 Retic <60 Plat <20 Neut <0.5 VSAA SAA + neut <0.2
34
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
35
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
36
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
37
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
38
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
39
HIT 2023 Pregnancy
Fondaparinux but beware long half life (42 hours before regional technique)
40
MF 2023 Indications ruxolitinib
Any grade with splenomegaly or symptoms
41
MF 2023 Infectious risk mitigation ruxolitinib
Baseline HIV, HepB, HepC Individualised plan TB risk Individualised herpes virus plan
42
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)
43
MF 2023 Ruxo dosing for platelet ranges
>200 : 20mg BD 100-200 : 15mg BD 75-99 : 10mg BD 50-74 : 5mg BD
44
MF 2023 Options for platelets <50
Start danazol then add ruxo if platelets respond Off SpC license - low dose ruxo with monitoring
45
MF 2023 Fedratinib indication
Disease related splenomegaly Or those resistant to ruxo
46
MF 2023 Momelotinib indication
Consider 1st line for MF with splenomegaly and anaemia Not routinely available in UK
47
MF 2023 Role for EPO
Alone or with ruxo if low EPO level
48
MF 2023 Role for danazol
Alone or with ruxo if EPO doesn't work
49
MF 2023 Role for chelation
Consider in iron overloaded patients being worked up for alloSCT
50
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)
51
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
52
MZL 2023 Non-gastric MALT management
Antiviral for hep C Antibiotics for eyes Antibiotics if evidence chronic infection RT otherwise
53
MZL 2023 Management advanced EMZL/NMZL
R-Chlorambucil
54
MZL 2023 Management SMZL
R monotherapy
55
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
56
MCL 2023 Localised disease management
Need full staging workup Consider local RT
57
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
58
MCL 2023 1st line in transplant ineligible
R-chemo R maintenance R-miniCHOP or similar for frail
59
MCL 2023 Management indolent MCL
W+W if low volume nodal or isolated spleen/BM/blood
60
MCL 2023 Management relapse
Ibrutinib unless used 1st line Ibrutinib good for CNS relapse
61
MCL 2023 Indications for brexu-cel
Relapsed post CD20 + BTKi Lack of early response to BTKi in 2nd line
62
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
63
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
64
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
65
Pregnant MHV 2023 Pre-pregnancy management
Counsel before valve insertion if CBA Counsel as soon as become CBA
66
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
67
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
68
MGUS 2023 Workup
IgG, IgG, IgM AND SPEP Immunofixation (more sensitive than SPEP) blood and urine Serum freelite FBC Renal function Corrected calcium
69
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
70
MGUS 2023 Indications for BMAT + imaging
Int-high or high
71
MGUS 2023 Follow up
All patients: repeat bloods in 6 months Thereafter yearly follow up Don’t discharge int-high or high
72
ATR 2023 Mandatory training
Recommended for all staff in clinical or laboratory areas involved in transfusion
73
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
74
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
75
ATR 2023 Management recurrent fever
Try prophylactic paracetamol or NSAID If doesn’t work try washed components
76
ATR 2023 Recurrent mild allergy
Not for prophylaxis Exclude other aetiologies
77
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
78
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
79
ATR 2023 Reporting
All but mild febrile/allergic reactions via SABRE Review within hospital transfusion team
80
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
81
BSH/BSIR 2023 Low risk interventions
Venous Superficial biopsy GI tract MSK US drain
82
BSH/BSIR 2023 Med risk interventions
Low gauge arterial Embolisation Dialysis access Tunnelled line
83
BSH/BSIR 2023 High risk interventions
High gauge arterial Aortic Tumour ablation Renal biopsy/stent TIPSS/TJ biopsy Liver biopsy
84
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
85
BSH/BSIR 2023 Corrections for liver disease blood params
Fn >1.2g/L Plts >50 HCT >0.25
86
BSH/BSIR 2023 Anticoag hold/restart times
See photo
87
BSH iron chelation Hb-opathy 2021 Complication IOL
Hypogonadotrophic hypogonadism Hypothyroid/parathyroid Diabetes Cardiac siderosis - failure/arrythmia HCC
88
BSH iron chelation Hb-opathy 2021 Surrogate marker for other complications
Liver iron concentration
89
BSH iron chelation Hb-opathy 2021 Threshold for risk of IOL
Transfusional: blood at least 1 unit per 3 months Non-transfusional: NTDT, NTRIA
90
BSH iron chelation Hb-opathy 2021 Frequency serum ferritin
1-3 monthly
91
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
92
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
93
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
94
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
95
BSH iron chelation Hb-opathy 2021 Liver complications
Cirrhosis when LIC >7mg/g HCC
96
BSH iron chelation Hb-opathy 2021 Triggers for assessment
SCD: regular top ups or if rising ferritin NTDT: ferritin >800 NTRIA: ferritin >1000
97
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
98
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
99
BSH/SHOT anti-D pregnancy PSE types <12 weeks
Surgical managed abortion, miscarriage, ectopic or molar Medical abortion 10-12 weeks
100
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
101
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)
102
BSH/SHOT anti-D pregnancy RAADP
1500 IU anti-D at 28-30 weeks
103
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
104
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
105
BSH/SHOT anti-D pregnancy Threshold FMH for flow assessment
>2ml
106
BSH/SHOT anti-D pregnancy Dose anti-D
125 IU/ml (IM) 100 IU/ml (IV)
107
BSH/SHOT anti-D pregnancy Practical consideration anti-D
Blood derived product Consent Fever/headache Can cause allergic reaction
108
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
109
GTG65 Which abs have increased monitoring?
anti-c anti-D anti-K Previous history of HDFN (refer any)
110
GTG65 Frequency of cross matching if high risk of need for blood and allo-ab
Weekly!
111
GTG65 What to warn obs team about mother if allo-abs
Increased risk of needing blood Have blood ready
112
GTG65 What to send off on cord blood?
Hb DAT Bilirubin
113
GTG65 What to tell paeds team
Baby at risk for up to few weeks, monitor
114
CLL 2022 First line treatments unfit
Ven-O Acalabrutinib Ibru-ven Zanubrutinib Regardless of TP53 status
115
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
116
CLL 2022 Vaccinations at diagnosis
Prevenar then pneumovax Annual flu Covid
117
CLL 2022 Antimicrobials
Prophylaxis if IgG <4g/L IVIg if no response
118
BSH CNS prophylaxis 2020 Criteria for prophylaxis
CNS-IPI 4-6 3 or more EN sites Testes, renal/adrenal, intravascular
119
BSH CNS prophylaxis 2020 Sites to consider prophylaxis
Breast Uterus
120
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
121
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
122
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
123
Infection in hyposplenic 2024 Prophylactic antibiotics
Lifelong penicillin or macrolide Protects against pneumococcus if high risk Also carry supply of rescue antibiotics
124
Infection in hyposplenic 2024 Management of new infection
Hospitalise urgently Prompt IV antibiotics