Haematology Flashcards
1
Q
pathogenesis of multiple myeloma
A
type of haematological malignacy
- caused by malignant proliferation of plasma cells within the bone marrow
- normal plasma cells are derived from B cells
- in myeloma plasma cells produce immunoglobulins of a single heavy and light chain= paraprotein
- although a small number of malignant plasma cells are present in the circulation, the majority are present in the bone marrow
- the malignant plasma cells produce cytokines, which stimulate osteoclasts and result in net bone reabsorption
2
Q
classification of myeloma
A
IgG
IgA
light chain only
3
Q
common incidence of rmyeloma
A
60-70yrs
it is uncommon
2% of all cancer dx
4
Q
MGUS
A
monoclonal gammopathy of insignificance
Monoclonal gammopathy of undetermined significance, or ‘MGUS’, is a benign (non-cancerous) condition. MGUS does not cause any symptoms and is usually diagnosed incidentally when tests are performed to investigate other problems. It does not require any treatment.
In MGUS, abnormal plasma cells in the bone marrow release an abnormal protein, known as paraprotein. MGUS is characterised by the presence of this abnormal protein in the blood and/or urine.
While most MGUS patients have a stable condition which has no effect on their general health, a small proportion of patients will go on to develop a cancer called myeloma.
5
Q
presenting features of MYELOMA
A
CRAB
hypercalcaemia-osteoclastic bone resorption
renal failure- due to the paraproteins
Anaemia- reduced RBC erythrocytes production
Bone pain-# spinal cord compression
others
- recurrent infections as reduced normal immunoglobulin production
- AKI
- symptoms of hypercalcaemia-stone bones moans groans thrones
- asymtpomatic
- amyloidosis
- carpal tunnel
- neuropathy
6
Q
what is the diagnostic criteria for myeloma
A
- need 2 of the following
1. increased malignant plasma cells in the bone marrow
2. serum and/or urinary protein
3. skeletal lytic lesions
7
Q
screening inx for myeloma
A
- serum protein electrophoresis- look for paraprotein in blood
- bence Jones proteinuria
need to do both tests as may have one of other
- if both positive need to do more testing
- if both negative unlikely to be MM so only do further testing if they present with very typical features
8
Q
confirm inx for myeloma
A
-bone marrow aspirate and trephine (need >10% clonal plasma cells) -skeletal survery (look for lytic lesions) -bloods check ca, ALKphos, anaemia
9
Q
morphology of myeloma plasma cells
A
eccentric nucleus surrounded by a paler area and blue cytoplasm
10
Q
if no evidence of myeloma related tissue impairment with diagnosis=
A
smouldering myeloma
11
Q
if patients have evidence of myeloma with tissue impairment
A
=multiple myeloma
12
Q
course time of multiple myeloma
A
- asymptomatic phase with either MGUS or smouldering myeloma
- once patients become symptomatic they have a certain level of serum light chains or bence jones proteins in urine that can be used to monitor their disease
- most patients respond well to initial therapy and will achieve reduction in paraprotein and some to the point of remission
- remission or plateau phase
6, relapse and remitting with gradually getting worse
13
Q
management of myeloma
supportive measures
A
- adequate hydration
- zoledronate for hypercalcaemia
- bone protection
- analgesia for bone pain
- transfusion for symptomatic anaemia
- treatment of bacterial infections- due to hypogammaglobulinaemia
- allopurinol prevent urate nephropathy
- plasmapheresis if necessary for hyperviscosity
14
Q
emergency complications for myeloma
A
-renal failure hypercalcaemia hyperviscosity hyperuricaemia spinal cord compression -radiotherapy and bisphosphonates
15
Q
asymptomatic multiple myeloma management
A
watchful waiting can be used
16
Q
<70 yr old myeloma with little or no significant co-morbidties management
A
intensive chemo approach
1. induction phase dexamethasone plus thalidomide plus PI proteasome inhibitor- borteozmib
2.consolidation phase
autologus peripheral blood stem cell transfer with melphalan
- maintenance phase
immunomodulatory drug thalidomide - relapse
thalidomide plus steroid
17
Q
non-intensive approach for multiple myeloma management
A
- induction
thalidomide+ dexamethasone + alkylator eg chlophosphamide/ mephalan
2.maintenace phase
thalidomide
3.relapse
bortezomib plus steroid
18
Q
how do intensive and non-intensive myeloma management differ
A
intensive= PBSCT and induction phase use proteasome inhibitor eg bortezomib
non-intensive= no consolidation phase so no PBSCT
19
Q
poor prognostic factors for multiple myeloma
A
high B2 microglobulin
low albumin at diagnosis
20
Q
acute leukaemias myeloid vs lymphoblastic age onset
A
myeloid= adults lymphoblastic= children
21
Q
definition of leukaemia
A
highly aggressive malignancy of haematopoietic stem cells or early progenitor cell
malignant blast cells accumulate in bone marrow and blood
22
Q
pathogenesis of leukaemia acute
A
- in acute leukaemia there is a proliferation of primitive stem cells with limited accompanying differentiation, leading to an accumulation of blasts, predominantly in the bone marrow, which causes bone marrow failure
- proliferation of cells that do not mature leading to primitive cells taking up bone marrow space at the cost of normal haematopoietic elements
- eventually this proliferation spills into blood and get blast cells in blood
23
Q
what conditions can the philadelphia chromosome be found in
A
CML
ALL
24
Q
risk factors for leukaemias
A
ionising radiation
radiotherapy
cytotoxic drugs
retroviruses- HTLV-1 adult T cell leukaemias
25
diagnosis findings for leukaemia
1. blood
- ANAEMIA with normal or raised MCV
- leukopaenia
- sometimes blast cells in the film
2. bone marrow aspirate and trephine
- hypercellular leukaemic blast cells
- auer rods in cytoplasm indicates it is myeloblastic
3. immune phenotype
- determine antigens on cells
- ALL blasts are positive for CD19 and CD20
4. cytogenic analysis= trisomy 8 can be seen
26
division of ALL
t or b precursor
27
AML types
- geentic abnormalities
- myelodysplasia changes
- neoplasms theray related
- myeloid sarcoma
28
presentation of acute myeloid leukaemia
adult
anaemia= pallor, breathlessness, angina, lassitude, palpitations,
neutropaenia= low neutrophils so get infections- sore throat, resp, perianal, skin, mouth ulcers, fever
thrombocytopaenia= bleeding mucosal - blood blisters, purpura, bleeding gums, prolonged menstrual bleeding, nose bleeds
tissue infiltrates= gum hypertrophy
bone pain
29
presentation of acute lymphoblastic leukaemia
child
-bone marrow failure
anaemia= pallor, breathlessness, angina, lassitude, palpitations,
neutropaenia= low neutrophils so get infections- sore throat, resp, perianal, skin, mouth ulcers, fever
thrombocytopaenia= bleeding mucosal - blood blisters, purpura, bleeding gums, prolonged menstrual bleeding, nose bleeds
tissue infiltrates= lymphadenopathy, testicular swelling, hepatomegaly, splenomegaly
-bone pain
30
management conservaitve acute leukaemias
- if very elderly or serious co-morbidities
- supportive treatment
- can give low intensity cytosine arabinoside
31
immediate management of acute leukaemias
- treat existing infection
- correct anaemia with transfusion
- control bleeding with platelet transufsion
- central venous catheter insertion
- assess tumour lysis risk and start prevention- allopurinol or rasburicase
32
stages of acute leukaemias management
- remission induction -combination chemotherapy after which patient can be in bone marrow hypoplasia and need intensive support
- remission consolidation- in remission but residual disease attacked by therapy
- remission maintenance- consolidate
33
chemotherapy for acute leukaemias ALL
- intensive
- remission induction: vinicristine, prednisolone, L asparaginase-pegaspargase +danorubicin
- consolidation= danorubicin, methotrexate
- maintenance= mercatopurine, methotrexate, vinicristine and prednisolone
- intrathecal methotrexate for CNS prophylaxis
- stem cell transplant
34
ALL what prophylactic treatment can be given for CNS
cranial irradiation
| intrathecal methotrexate
35
AML chemotherapy
induction: danuroubicin
consolidation: cytarabine, amsacrin
36
what should be given to acute leukaemias if philadelphia positive
IMATINIB
37
PROGNOSIS ALL in children
>90%
38
what is acute promyelocytic leukaemia
translocation in 15-17
treat with Vit A compound
assoc. to DIC
39
child with unexplained hepatosplenomegaly or unexplained petechiae should
refer urgently to specialist- ALL
40
child with pallor, persistent fatigue, unexplained fever
consider ALL
| need urgent FBC <48hrs
41
A 4-year-old girl presents with lethargy, dyspnoea, fever, and bruising. On examination she has hepatosplenomegaly. Chest x-ray shows a mediastinal mass and pleural effusion.
ALL
42
A 58-year-old man presents to his primary care physician with increasing tiredness, accompanied by bruising on his legs. He also complains of aching bones. He has no previous illnesses. On examination, he is pyrexial and pale, has bony tenderness over the sternum and tibia, and has petechiae on his legs. There are no palpable lymph nodes. He has crepitations at the left base. The liver and spleen are not palpable.
AML
43
A 45-year-old woman presents to the emergency department with nausea, vomiting, and confusion. She has a history of low back pain of 6 months' duration and increasing sciatic pain in the last 2 weeks. On physical examination, the patient is pale and dehydrated with bone tenderness in the lumbar region. Neurological examination reveals an upgoing plantar reflex on the left with intact power in all muscle groups and at all joints. Magnetic resonance imaging reveals an L5 compression fracture. This is associated with hypercalcaemia and renal insufficiency.
Multiple myeloma
44
A 55-year-old man has had routine physical examinations for several years and has always been healthy, does not smoke, and has no history of pulmonary disease. His primary care physician has noted a gradually increasing haemoglobin level over the past few years (to a current level of 195 g/L [19.5 g/dL]), mild leukocytosis, and mild thrombocytosis. He has frequent episodes of facial flushing that are associated with slight headaches and a feeling of fullness in his head and neck. He has noted intermittent burning, stinging, and tingling sensations in his fingertips. He has recurrent, often severe, pruritus that is exacerbated by taking a hot bath. On examination, he has a red face and neck and the spleen is mildly enlarged.
polycythaemia vera
45
72 year old woman reports fatigue, night sweats, 4.5 kg (10 lb) weight loss, abdominal discomfort, and progressive dyspnoea on exertion. Laboratory results reveal a white blood cell count of 6.2 x 10^9/L (6200/microlitre) with an absolute neutrophil count of 2.2 x 10^9/L (2200/microlitre), an absolute lymphocyte count of 2.4 x 10^9/L (2400/microlitre), and 0.36 x 10^9/L monocytes (360 monocytes/microlitre); a haemoglobin (Hb) of 60 g/L (6 g/dL) with a mean corpuscular volume of 86; a platelet count of 96 x 10^9/L (96 x 10^3/microlitre) and a reticulocyte count of 0.6%, with an absolute reticulocyte count of 14.1 x 10^9/L (14.1 x 10^3/microlitre). The peripheral blood smear shows occasional teardrop-shaped red blood cells, and erythroblasts and myelocytes. on examination there is splenomegaly and they have a dry tap
PMF
46
A 54-year-old man presents to his primary care physician with a 2-month history of fever, malaise, and weight loss. He also reports frequent epistaxis, abdominal fullness, and early satiety. On examination, he is found to have splenomegaly.
CML
47
A 62-year-old man presents to his primary care physician for an annual physical. He denies any complaints such as fever or chills, weight loss, or fatigue. Of note, his blood tests show an elevated WBC count. The WBCs are predominantly lymphocytes, with a differential of 80% lymphocytes and an absolute lymphocyte count of 75 x 10⁹/L (75 x 10³/microlitre).
CLL
48
25 yr old male
painless unilateral neck lumps
6 month history
He denies recent upper respiratory tract infections, fevers, night sweats, or unintentional weight loss. He is otherwise healthy. Social history and family history are unremarkable. On examination he is afebrile with normal vital signs. Pertinent findings include a 3-cm, firm, round, non-tender, mobile mass in the mid-right neck. There is no other peripheral lymphadenopathy. Liver and spleen are not enlarged.
hodgkin's
49
A 56-year-old woman presents with a painless right neck lump that has been slowly enlarging for the last 2 years. She denies fevers, night sweats, or weight loss. Physical examination reveals bilateral cervical and axillary adenopathy and a palpable spleen.
non-hodgkin's
50
pathology of chronic leukaemias
in chronic leukaemias the malignant clone is able to differentiate resulting in an accumualtion of more mature cells aka from the myelocytic and lymphocytic progenitor cells
- myeloid relates to RBC, granulocytes, monocytes and platelets
- lymphocytes relates to T and B cells
51
diagnosis chronic leukaemias
- abnormal blood count- raised WCC
| - examination of bone marrow- immunophenotyping
52
chronic myeloid leukaemia pathology
- 30-80 yrs
- proliferation of all haematopoietic lineages, but predominantly granulocytic series- myeloid
- philadelphia chromosome
53
how is the philadelphia chromosome formed
- reciprocal translocation c22 and c9
- shortened chromosome 22 =philadelphia
- puts BCR and ABL next to each other which makes tyrosine kinase so get uncontrolled proliferation
54
phases of CML
chronic phase
-disease is responsive to treatment and easily controlled
accelerated phase
-disease control more difficult
blast crisis
- in which the disease transforms into an acute leukaemia either myeloblastic or lymphoblastic which is relatively refractory to treatment
- cause of death in majority of patients
- cant predict it
55
chronic vs acute leukaemias
chronic leukaemias is due to proliferation of myeloid or lymphocyte cells- over months to years - leukocytosis plus hepatosplenomegaly - marrow failure is later on
acute leukaemias is due to proliferation of blast- weeks days - with features of marrow failure
56
presentation CML
older patient
late middle age
abdominal discomfort and splenomegaly
most are asymptomatic
also can be hyperviscoisty- mucosal bleeding, SOB, visual changes, new neurology
thrombosis state
lethargy, weight loss, abdo discomfort, gout sweating
57
inx CML and results
```
like other myeloproliferative disorders
blood
-neutrophilia (baby neutrophils)
-normocytic normochromic anaemia
-thrombocytosis sometimes
-raised WCC
-LDH and urate can be high due to increased turnover
```
blood film
myeloblast to mature neutrophils present
predominant are neutrophils and myelocytes
also increase in eosinophils and basophils
- if it progresses to an accelerated phase then primitive cells increase
bone marrow
-confirm diagnosis
chromosome analysis for philadelphia chromosome
58
management of chronic phase CML
- give tyrosine kinase inhibitors= imatinib
- take bone marrow sample at 6 months to confirm
- 3 monthly blood BCR ABL mRNA aim to reduce levels
2nd line switch to a different TK inhibitor
3 rd line= allogenic HSCT haematopoietic stem cell transplant
59
management of accelerated phase treatment CML
TKI therapy
allogenic HSCT
hydroxycarbamide in older patients
60
CLL presentation
older
asymptomatic
often diagnosed on isolated lymphocytosis
50% have lymph nodes, splenomegaly
-anaemia, infections, painless lymphadenopathy, systemic symptoms, such as night sweats, weight loss
61
inx CLL
blood
- high lymphocyte count -isolated +++
blood film
-mature lymphocytosis >5
blood sample-flow cytometry
blood test-immunophenotyping
-monocloanal B cells express CD19 OR 23
t cell antigen CD5
bone marrow exam not essential but can be helpful
also can do coombs test and hypogammaglobulinaemia
62
assoc. autoimmune phenomena to CLL
- ITP - petechiae development
| - autoimmune haemolysis
63
management of stage A CLL
watch and wait
no specific treatment
doesnt change life expectancy
treat if evidence of bone marrow failure
64
stage b and c or a with bone marrow failure
CLL management
Fludarabine with alkylating agent cyclophosphamide and rituximab if <70 fit and TP53 mutation
if older and less fit use rituximab with bendamustine
new inhibitors of B cell receptor= ibrutinib for relapsed CLL and TP53 mutation
65
lymphoma pathology
cancer of lymphocytes in lymph nodes and extra-nodal areas eg spleen, liver, bone marrow
-neoplasma arise from lymphoid tissue
66
presentation of lymphoma
-swelling lymphadenopathy
>1cm, painless, persistent and not reactive
often in neck, axillaem inguinal, internal
- internal swelling can lead to mass effect eg compression on airways, bowel, ureter, SVCO, dysphagia
- organomegaly: hepatosplenomegaly
- constitutional B symptoms- fever, night sweats, weight loss
- marrow failure if diffuse marrow involvement
67
inx lymphoma
FBC
- only abnormal if lymphoma is also in the marrow (then can get marrow failure)
- anaemias
- raised ESR in hodgkin's
Tissue lymph node biopsy
CXR
PET-CT
staging
- CT staging CAP
bloods
- LDH marker of cell acitivty
- urate idea of risk of tumour lysis syndrome
Excision biopsy - a whole lymph node biopsy is the gold standard dx
68
tissue lymph node biopsy for non hodgkin
follicular lymphoma
69
hodgkin lymphoma cell
reed sternberg cell
70
types of lymphoma
```
hodgkin
non hodgkin
-b cell
-t cell
-high grade eg diffuse large B cell lymphoma
low grade eg follicular
```
71
high grade non-h means
divides rapidly
present for a matter of weeks before dx
can be lifethreatening
72
low grade non h means
divides slowly
present for many months before dx
behave in an indolent fashion
73
hodgkin lymphoma features
```
younger people
reed sternberg b cell
aggressive but highly curable
painless, rubbery lymphadenopathy, asymtpomatic
cough, fever, haemoptysis, dyspnoea
b symptoms
```
74
management of hodgkins
ABVD doxorubicin, bleomycin, vinblastine and darcarbazine
combined with radiotherapy
75
non-hodgkin cause
```
-no single causative abnormality described
but can be linked to
-HIV
EBV
HTLV-1
MALT lymphoma to h.pylori
```
76
presentation of non-hodgkin
can be widely disseminated at presentation including extranodal sites if high grade
-systemic upset
-hepatosplenomegaly
SVCO if compression or ascites
77
additional inx for non hodgkins
```
bone marrow aspiration and trephine
immunophenotyping of surface antgens distinguish t and b cells
HIV
hep b and c testing
immunoglobulin determination
```
78
management low grade NHL
majority preset with advanced stage disease and will run a relapsing and remitting course
-asymptomatic can be watchful waiting
```
options
-radiotherapy
chemotherapy
monoclonal antibody
kinase inhibitors
transplantation
```
79
management high grade NHL
chemotherapy
monocloanal antibody therapy
radiotherapy
HCST
80
management follicular lymphoma
stage 1 and 2
vs
stage 3 and 4
radiotherapy in stage 1/2
stage 3/4
-if symptomatic- chemotherapy using R-CVP
rituximab, cyclophosphamide, vinicristine, prednisolone
-asymptomatic watchful waiting or solely use rituximab
81
CHOP treatment for non hodgkin's lymphoma
cyclophosphamide
doxorubulin
vinicristine
prednisolone
82
-young man
asymmetrical and painless superficial lymphadenopathy
alcohol induced nodal pain
HL
83
late middle age
| lymphadenopathy
non hodgkin's
84
```
A 70-year-old coal miner presents with 3 weeks of haematuria and bruising. He is normally fit and well. He is on no medications. His results reveal:
Hb 9.0
WCC 11
Pl 255
PT 16 (normal)
APTT 58 (increased)
Thrombin time 20 (normal).
```
factor 8 acquired haemophilia
85
A 33-year-old female is admitted for varicose vein surgery. She is fit and well. After the procedure she is persistently bleeding. She is known to have menorrhagia. Investigations show a prolonged bleeding time and increased APTT. She has a normal PT and platelet count.
von willebrand factor
86
complications of CLL
-anaemia
hypogammaglobulinaemia (recurrent infection as low Ig)
warm autoimmune HA
Transform to high grade lymphoma- Richter transforamtion
87
what is Richter transformation
transformation CLL to non hodgkin lymphoma
- lymph node swelling
- fever without infection
- weight loss
- night sweats
- nausea
- abdominal pain
88
what are the myelproliferative neoplasms
disorders characterised by clonal proliferation of malignant bone marrow cells
89
which are the 4 myeloproliferative neoplasms
1. essential thrombocytosis
2. polycythaemia vera
3. CML
4. myelofibrosis
90
essential thrombocytosis pathology
megakarocyte proliferation results in an over-production of platelets
91
features of essential thrombocytosis 4
- platelet count >600
- thrombosis and haemorrhage can be seen
- burning sensation in hand
- JAK2
92
treatment of essential thrombocytosis
- hydroxyurea- hydroxycarbamide lowers platelets
| - Interferon alpha- low dose aspirin
93
polcythaemia vera inx findings
- raised RBC
- often increase neutrophils and platelets, basophils also
- JAK2 mutation
- Iron studies
- renal and liver
94
features of polycythaemia vera
```
over 60
hyperviscosity-thrombosis
pruritus
splenomegaly
haemorrhage
plethoric appearance
HTN
decreased ESR
```
95
treatment of polycythaemia vera
- venesection= 1st line
- aspirin
- hydroxyurea
96
prognosis of polycythaemia vera 3
- thrombotic events
- 5-15% progress to myelofibrosis
- 5-15% progress to acute myeloid leukaemia
97
myelofibrosis pathology
- hyperplasia of abnormal megakeraryocytes
- resultant release of platelet derived growth factor is thought to stimulate fibroblasts
- leads to extensive scarring in bone marrow
98
features of myelofibrosis 3
- insidious fatigue and weight loss
- splenomegaly
- elderly person with symptoms of anaemia
99
investigation findings of myelofibrosis 5
- anaemia
- increased WCC and platelet count
- "tear drop" poikiocytes on blood film
- "dry tap" therefore trephine biopsy needed
- increased LDH and urate
100
cause of primary myelofibrosis
can be progression from polycytheamia vera or Essential thrombocytosis
101
causes of plasma cell dyscrasias 4
MGUS monoclonal gammopathy of undetermined significance
multiple myeloma
amyloidosis
plasmacytoma
102
how do we know that haematological malignancies arise from a single cell
clonality
103
CML pathology
- reciprical translocation of c 22 and c9
- get shortened 22 with bcr next to abl which creates mRNA for tyrosine kinase activity
- philadelphia chromosome
104
concerning features of a lymph node
```
>1cm
not mobile
rubbery
not tender
persistent
```
105
types of blood groups
o and a are commonest
o, a, b and AB
negative or positive
106
if o what blood is compatible
only o
107
if ab what blood is compatible
all
108
if b what blood is compatible
only b and o
109
if o negative what blood is compatible
only o negative
110
what does rhesus positive mean
means the red cell carry D antigen are D poisitive
85% of the population
D negative needs D negative
111
why do rhesus negative need to receive rhesus negative
- rhesus negative can make anti-D if they are exposed to D positive cells through transfusion or pregnancy
- can cause haemolysis on future exposure
so rhesus o negative can only receive o negative hence its given in rhesus scenarios
112
monitoring on blood transfusion how often
temperature and blood pressure every 30 mins
113
types of blood products
-whole blood-rarely used
-red cells
-platelets
fresh frozen plasma
cryoprecipitate
prothrombin complex
114
target haemoglobin for anaemia transfusion
| when to transfuse
80 Hb for acute coronary syndrome
| 70 Hb for without acute coronary syndrome
115
target haemoglobin for after transfusion
acute coronary syndrome= 80-100
| without= 70-90
116
target when to transfuse platelets
- if bleeding or
| - count <20 x10^9
117
how much should 1 unit of platelet increase platelet count
by 20x10^9
118
if surgery is planned at what platelet level should you get advice from haeamtology
<100
119
fresh frozen plasma indication
-used to correct clotting defects eg DIC, warfarin overdose, liver disease, thrombotic thrombocytopaenic purpura
120
types of plasma products
FFP
cryoprecipitate
prothrombin complex concentrate
121
indication for FFP
clinically significant bleeding but not major haemorrhage with clotting defects/ abnormal coag result
122
what is cryoprecipitate
factor 8 vWF, fibrinogen and factor 13
123
when is cryoprecipiate indicated
patients without major haemorrhage who have a
- clinically significant bleed AND
- fibrinogen <1.5
124
prothrombin complex concentration indication
- for emergency reversal of warfarin in patients with either
1. severe bleeding or
2. head injury with suspected intracerebral haemorrhage
major haemorrhage
125
when is human albumin solution indicated
replace protein in hypoprotein aemic patient eg liver disease who is fluid overload
or abnormal paracentesis
126
definition of massive blood transfusion
replacement of an individuals' entire blood volume in 24hours
127
complications of massive blood transfusion
```
low platelets
low calcium
low clotting factors
increased potassium
hypothermia
```
128
acute haemorrhage blood product choice
use crossmatched if possible
but if not possible then use o negative
change to cross match as soon as possible
129
cautions for transfusing a patient with heart failure
give each unit over 4 hours with furosemide with alternate units
check for increased JVP and basal lung crackles
consider CVP line
130
how is autologous blood transfusion done
-patients have their own blood stored pre-op for later use
give
- EPO to increase yield
- intraoperative cell salvage with re-transfusion
131
definition of mild febrile reaction to blood transfusion
defined as a temperature of 38 and a rise of between 1-2 degrees from pre-transfusion values
but no other symptoms or signs
132
how is the risk of febrile reactions to blood transfusions now reduced
leuco-depletion
133
pathology of febrile reactions
reaction to white cells or cytokines released from white cells
patient already has antibodies to the WCC
or due to cytokines
134
definition of mild allergic reaction
-transient flushing urticaria or rash, sometimes with a respiratory wheeze
135
pathogen of allergic transfusion reactions
due to an allergen in the donor's plasma
| occurs more frequently in patients with other allergies and atopy
136
what products tend to cause allergic transfusion reactions
plasma or platelets transfusion
137
what products tend to cause febrile transfusion reactions
red cells and platelets
not usually plasma
138
definition of moderate febrile transfusion reaction
rise of temperature of 2 degrees or more, or fever of 39degrees or rigors, chills and other inflammatory symptoms/ signs such as myalgia or nausea
139
definition of moderate allergic reaction
-wheeze or angioedema with or without flushing/ urticaria
| but without resp compromise or hypotension
140
definition of severe transfusion reaction febrile
reaction that fulfills the criteria of moderate and requires immediate medical attention or leads to admission or prolongs hospital stay
141
definition of severe allergic reactions
causing respiratory compromise or circulatory compromise which requires urgent medical intervention
or
anaphylaxis
142
acute transfusion reactions <24 hrs
```
acute haemolytic
allergic/ anaphylaxis
infection
febrile non haemolytic
respiraotry - TACO/ TRALI
```
143
delayed transfusion reaction >24hrs
delayed haemolytic
infection
transfusion vs graft host disease
post transufion pupura
144
responding to a transfusion reaction
1. stop transfusion and call senior
2. treat the patient symptomatically -antih-paracetamol
3. if collapsed call for help and A to E
4. inx the cause: set of observations, check right blood and right patient, check bag, culture, x-ray
5. seek haematologist help if doesnt settle
6. keep the patient informed
7. report locally and externally SHOT reported
145
acute haemolytic reaction pathology
ABO mismatch
RBC antibody IgM reacts with RBC causing
-intravascular haemolysis= RBC directly
-extravascular haemolysis= destroys spleen
antibodies cause the RBC to agglutinate and complement too RBC
146
cause of acute haemolytic reaction
usually due to failure of identification and checks
147
acute haemolytic transfusion reaction clinical presentation
```
-agitated
chills
abdominal pain- loin pain
dizzy
blood pressure collapse
can get fever
dark urine
renal failure
DIC -bleeding
```
148
What is TRALI
transfusion related acute lung injury
149
presentation of TRALI
- severe breathlessness and hypoxia within 6 hours of transfusion
- consider in patient not responding to pulmonary oedema treatment
150
pathology of TRALI
- transfusion of donor anti-white cell antibodies reacting with the patient's white cells
- white cells stick in the pulmonary capillaries and cause lung damage
- white cell antibodies occur in female donors because of previous pregnancy
151
how is TRALI rates reduced
- for FPP or platelets either come from a male donor or from a female donor who has been screened for anti white cell antibodies
- mostly male donors
152
inx findings TRALI
-pulmonary bilateral infiltrates
153
management of TRALI
- supportive therapy
154
TACO is
transufsion associated circualtory overload
-pulmonary oedema
usually at end of transfusion
155
reducing risk of TACO
- give only one unit at a time and reassess
- calculate dose by body weight
- monitor vital signs closely
- measure fluid balance
- reduce rate of transfusion
- give diuretics with transfusion
156
presentation of TACO
acute respiratory distress due to acute pulmonary oedema occurring during or within a few hours of transfusion
157
who is at risk of TACO
-very old and very young
renal impairment
heart failure
158
what is the commonest cause of transfusion related death
TACO
159
management of TACOg
give diuretics
call senior stop
usually procedure
160
timing and acute transfusion reaction order
anaphylaxis 15 mins
acute haemolytic reaction within minutes collapse
TACO- during or just after
TRALI- 6 hrs after
161
delayed haemolytic transfusion reaction HTR pathology
rare type of reaction
- usually seen in patients who have developed red cell antibodies in the past from transfusion or pregnancy
- ALLOMUNISATION: formation of IgG red cell antibodies that form in response to a previous transfusion or pregnancy
- If the patient has another transfusion with RBCS expressing the same "foreign" antigen the immune antibodies cause RBC destruction
- the antigen antibody complex does not destroy the red cell until it reaches the spleen or other macrophages within the reticulo endothelial system = EXTRAVASCULAR HAEMOLYSIS
-SORT OF RED CELL ANTIBODIES THAT CAUSE A DHTR are anti d, anti c, anti k, anti Fy and anti Jk
162
importance of cross match
-if a red cell antibody is identified, blood that is negative for that antigen is selected for crossmatching- if the antibody screen misses the antibody it can be picked up by the crossmatch
163
what does a group and screen do
it tells you the blood group and the antibody screen
but can miss specific antibodies as it only expresses all the red cell antigens that are "clinically significant"
164
what causes a delayed haemolytic reaction if blood given is crossmatched (aka should pick up all antibodies)
- a new antibody has formed
| - an alloantibody was missed
165
what does a crossmatch do
tests you blood directly with the selected donor blood to make sure compatible
166
presentation of DHTR
```
several dayss to two to three weeks after
-fever
falling haemoglobin or
a rise in HB which is less than expected
jaundice
haemoglobinuir a
```
167
immunological transufsion reactions
acute haemolytic- naturally occurring IgM antibodies to specific groups
delayed haemolytic- from previous transfusion/ pregnancy
TRALI- donor white blood cell antibodies
febrile non haemolytic- patient white blood cell antibodies
graft vs host
PTP
168
non immunological transfusion reactions
mild mod severe allergy
infective
physiological- TACO
169
how often are observations done during a blood transfusion
before
after 15 mins
and at end of transfusion
170
management of febrile non haemolytic transfusion reaction
```
stop transfusion and call senior
set of observations
determine severity
give paracetamol
follow procedure
```
171
mild allergic reaction treatment
antihistamines
172
acute haemolytic reaction treatment
stop transfusion call senior
| IV access and transfer to ITU-needs renal support
173
anaphylaxis treatment
adrenaline and antihistamines
174
bacterial contamination treatment
cultures and broad spec abx
175
TACO treatment
diuretics
176
TRALI treatment
oxygen and resp support
177
features of haemolysis
fall in hb
rise in bilirubin uncongugated
rise in LDH
blood film showing red cell spherocytosis
178
viral transmitted infection - how is the risk of this reduced
donor health check questionnaire
viral antibody testing
antigen testing
nucleic acid testing
179
what viruses are checked for
antibody HCV, HIV, HTLV1
antigen HBV
nucleic acid HCV and HIV and HEV
180
tranfusion assoc. graft versus host disease TA GVHD pathology
-engraftment of donor lymphocytes because the patient is immunosuppressed or the HLA is similar
causes multi-organ failure and death in all cases
181
how is TA GVHD risk reduced
IRRADIATED cellular blood components for all patients who are immunosuppressed
182
indications for irradiation
-immunosuppressed
severe T cell immunodeficiency
Hodgkins
HLA similar- blood from 1st or 2nd degree relatives or HLA matched platelets
other components: granulocytes, blood for intrauterine transfusions
treatments immunosuppressed- meds and stem cell transplant
183
presentation of transfusion assoc. graft vs host disease
```
-4 to 30 days after
immunosuppressed patient or HLA similar
increased temp
erythroderma
desquammation
diarrhoea
abnormal LFT
```
184
management of TA vs GHD
steroids
| but always fatal
185
how often can you donate blood and what ages
donate every 12 weeks
| from 17 yrs to (66 unless good health no upper limit)
186
stages of blood donation 6
1. make sure fit and well and something to eat
2. donor health check quesion about health and recent travel
3. give consent
4. finger prick test make sure not anaemic
5. lie back clean and needle inserted
6. blood giving takes 5-15 minutes
187
after blood donation advice 4
1. leave dressing on arm for 2 hours
2. rest reover and dont do strenuous exercise for a few hours
3. donors are asked to call up donor care line for up to 14 days post donation if feel unwell/ doubts
4. immediate medical attention advice
188
what testing is done on donated blood products 4
1. first 30 ml blood is diverted into a pouch to reduce risk of bacterial contamination
2. donation checked for ABO and D group
3. mandatory viral testing
4. discretionary test depending on travel hx and ethnic orgin
189
what are the mandatory blood tests6
```
hep b
hep c
hep e
HIV
HTLV1
syphilis
```
190
discretionary test on blood donated
cmv
malaria
west nile virus
HbS
191
HbS blood donation test is for
sickle cell patients
-needs to be done for blood for a sickle cell patient
as sickle cell patients should NOT receive blood from carriers for sickle cell disease
192
the whole blood process
1. whole blood is collected into an anticoagulant- CPD
2. unique 14 digit number given
3. leucocyte depletion by filtration remove WC
4. centrifuge to separate out the remaining components
5. red cells re-suspended in SAG-M
6. platelets made by pulling from four donors into a satellite bag and then re-suspended in PAS
7. FFP made by plasma into satellite bags -but all plasma from female donors is discarded due to risk of white cell antibodies from pregnancy/ transfusion TRALI
193
what anticoagulant is whole blood put into
citrate phoshate dextrose
194
what are red cells suspended in after centrifuge
SAG-M sodium adenin mannitol glucose
195
what are platelets suspended in after being pooled from 4 donors into a satellite bag
PAS platelet additive solution with a bit of plasma to reduce risk of allergic reactions
196
what is a RAD sure label
used to label if irradiated or not
| -done by a chemical in the label which turns black on irradiation if the blood undergoes irradiation
197
UK specification for leucocyte depletion
-LD whole blood takes place by filtration within 24hrs from donation but always by D2
some white blood cells may still be present hence need for irradiation
198
what steps means that CJD does not need to be tested for in blood donations
- patients who have received a blood transfusion since 1980 cannot donate blood again
- leucocyte depletion
- UK plasma not used for fractionation
- imported FFP for all patients born after 1996
199
shelf life red blood cells
35 days
200
storage temperature for red blood cells and where
2-6 degrees
| only in blood fridge never any other
201
how long if out of the blood fridge till red blood cells have to be discarded
30minutes
202
how long does a red blood cell transfusion have to be completed in
2-4hours must be completed RBC in
203
platelets how long shelf life
5-7 days
204
how long should platelets be transfused over
30-60 minutes
205
storage of platelets
gently agitated room temperature in an incubator at room temperature
NOT IN FRIDGE AS BECOME ACTIVATED and will be destroyed when transfused
206
how long can plasma be kept
3 years
207
where is plasma stored
freezer so need to thaw for 20-40 mins before use
208
how quickly does plasma have to be transfused
transfuse over 30 -60 mins
within 4 hours because clotting factors start to deteriorate
if put back in a blood fridge after thawing the clotting factors deteriorate more slowly and thawed FFP can be issued for transfusion up to 24 hours
209
platelet triggers
prophylaxis
surger
critical sites
- prophylaxis= 10
- surgery need platelets >50
- critical sites eg brain need to keep it >100
210
what needs to be matched for RBC transfusion
ABO group
and
crossmatch needs blood without antigens that patient has antibodies too
211
what needs to be matched for platelets
just Rhesus negative
| but better if ABO compatible
212
indication for platelets
to treat or prevent bleeding in patients with a low platelet count
213
indication for FFP
-to treat or prevent bleeding in patients with multiple clotting factor deficiencies
eg DIC, liver disease
mostly in major haemorrhage with 1:1 with RBC
214
comaptibility needed for FFP
- same ABO group where possible
- High titre anti a- and b negative
- no need to match for rhesus
- Group AB is universal donor
215
types of FFP
standard FFP- one male donor
methylene blue treated-one male donor-
for those born after 1996 , non UK source
Solvent detergent treated
pooled from 1500 donors- octapias
216
reversal of warfarin blood product
prothrombin complex- beriplex
217
IV immunoglobulin indication
made from plasma and is used to replace immunoglobulins in immunodeficiency
218
other blood products made from fractionation-
| not made from UK people risk of CJD variants
anti D immunoglobulin
albumin
prothrombin complex concentrate
IV immunoglobulin
219
risk of giving RBC transfusion to a patient with severe B12 deficiency
can cause heart failure
220
requestation procedure for blood transfusion 7
1. decision to transfuse
2. requesting procedure
3. informing the patient-requesting activity
4. requesting procedure- case study
5. pre-transfusion testing
6. group and save, crossmatching
7. blood availability
221
points to consider in decision to transfuse
- risk factors for transfusion
- hx of transfusion
- special requirements eg irradiated
222
what type of consent is needed for tranfusion
just informed
| DONT NEED SIGNED CONSENT
223
requesting procedure for a blood transfusion needs
```
-minimum patient ID data set
reason for request
type
pre-transfusion test
urgency
location patient
blood group antibodies
previous transfusion hx
special requirements
date and time
```
224
pre-transfusion testing what is done
1. ABO and rhesus group idetinfied
| 2. antibody screen detect red cell antibody
225
blood component specifics for neonates
-need to be CMV negative
226
who needs irradiated blood
intrauterine transfusions
neonatal exchange transfusions
top up transfusion after IUT
proven or suspected immunodeficiency
227
taking a blood sample
1. check identification
| 2. label blood sample at bed side hand written
228
collecting blood
1. ensure detail on blood collection form matches patient ID
2. locate and remove blood component from temp controlled storage. confirm correct one by matching lab generated label to minimum patient ID
3. check components and expiry
4. document removal
5. only collect ONE UNIT AT A TIME SO DONT MIX UP PATIENTS
6. ensure prompt delivery to clinical area
- often brought over by blood porters
229
pre-authorisation of blood procedure
1. check authorisation form/ transfusion record ensure component authorised and any specific requirements are noted
2. consent checked
3. check patient details on patient lab label to ID band
if interuppted must STOP and start again
5. ensure baselin obs done <60 mins before blood component administered
230
APTT increased
PT normal
bleeding time normal
is
haemophilia
231
APTT increased
PT normal
bleeding time increased
is
vWF
232
APTT increased
PT increased
bleeding time normal
vit K
233
PT increased
APTT normal
bleeding normal
platelets normal
warfarin administration
234
normal PT
normal APTT
increased bleeding time
normal platelets
aspirin
235
normal PT
increased APTT
normal bleed time
normal platelets
heparin
236
increased PT
increased APTT
increased bleed time
decreased platelets
DIC
237
heparin affects which factors
2,9, 10, 11
238
warfarin affects which factors
2,7, 9, 10
239
DIC factors
1,2, 5,8, 11
240
liver factors
1 2 5 7 9 10 11
241
what is a coagulation screen
addresses some aspects of coagulation
APTT
pt
fibrinogen concentration
242
what is prothrombin time
time taken for the sample of blood to clot after tissue factor and calcium are added
-extrinsic pathway
243
what is PT prolonged in
liver disease
DIC
warfarin/ vit K
deficiency 2, 5,7 10 or fibrinogen
244
what is APTT
time taken for the sample of blood to clot after a contact activator and calcium are added
intrinsic
245
what is APTT prolonged in
```
DIC
haemophilia
vWF
heparin
lupus and antiphos
liver disease
2,5,10, 8,9,11,12 or fibrinogen
```
246
fibrinogen is raised in
acute phase response
247
fibrinogen is low in
severe sepsis
DIC
rare congenital states
liver disease
inherited -afibro, dysfibro
neonatal fibrinogen is different so can give a false low result
248
if APTT is raised in isolation what needs to be checked
check a 50:50 APTT correction has been done
which is when normal serum is added to patient serum and test run again
so then should normalise due to factor addition
249
if APTT correction doesn't correct it suggest
something is inhibiting the test and preventing normalisation
aka an inhibitor or heparin contamination eg if taken from a hepairinsed line
inhibitors are
-antibody to factor specific eg FVIII
-non specific antibody that binds to phospholipid membranes= lupus anticoagulant - usually not pathogenic apart from in anti-phospholipid syndrome
250
what causes anti- fviii inhibitors 2
-exposure to exogenous factor when treating haemophilia A
or
-in patients with acquired haemophilia eg secondary to malignancy or rheumatological disorders
251
what causes anti-phospholipid or lupus anticoagulant antibodies
- mostly children with febrile illness
| - uncommonly as part of SLE
252
next inx after
| PROLONGED APTT that is corrected
-consider intrinsic factor testing
| vWF screen
253
what does vWF do
-carrier protein for FVIII
| if anything wrong with VWF it impacts the factor 8 levels
254
APTT normal
PT normal
positive bleeding hx
probably factor 13
because outside of pathways
helps to make clots tight
so if deficient clots break up easily
255
prolonged APTT and PT causes
```
DIC/ SEPSIS
vitamin K problems
-vit K deficiency
-vit K warfarin antagoniser
-liver disease
-malaborsorption
-abnormalities of cycle
```
medication
fibrinogen disorders
dilutional coagulopathy eg massive blood transfusion
isolated congenital factor deficiencies
-10, 5, 2
congenital multiple clotting factors
5 and 8 deficiencies
256
how does neonatal coagulation differ
-vit k dependent factors at half level till 6 months
-factors 5, 11, and antithrombin iii are all lower till 3-6months
-alpha 2 macroglobulin is raised
plasminogen low till 6 months
257
vit K dependent factors
2,7 9 10
258
```
13 yr old girl
menorrhagia
nosebleeds
easy bruising
prolonged APTT
normal PT
likely dx
```
von willebrand factor
259
```
36yr old man post-tonsillectomy bleeds
APTT prolonged and corrected
normal PT
vWF normal
likely dx
```
mild factor 8 haemophilia A undiagnosed
260
```
64 yr old man
pre-op coagulation
APTT prolonged and corrected
Normal PT
vwf normal
fXII deficiency
```
factor XII deficiency
| non clinical risk
261
child with rash
raised WCC
raised APTT corrected
raised PT
DIC
262
short APTT cause
due to acute phase response and high factor 8
263
thrombin time prolonged by
heparin
decreased fibrinogen
increased D-dimer
264
neutrophilia causes
infection and inflammation
drugs: steroids, GCSF
CML
265
neutropaenia causes
infection
drugs- cytotoxic, idiosyncratic
marrow failure
266
thrombocytosis causes
infection and inflammation
iron deficiency
myeloproliferative disorders
267
thrombocytopaenia causes
```
-drugs- cytotoxic, quinine
bleeding and DIC
immune- ITP
microangiopathy
marrow failure
```
268
lymphocytosis causes
viral infection
inflammation
lymphoid malignancy - CLL
269
lymphocytopaenia causes
viral infection
drugs- cytotoxic
ageing
marrow failure
270
eosinophils are
WCC
271
erythrocytes are
RBC
272
thrombocytosis definition
platelet count >2SD above population mean
>400
common incidental finding >40
273
reactive thrombocytosis is
proliferation of platelets caused by a response to growth factors released from an underlying malignancy or inflammatory condition NOT due to a primary haem disorder
274
clonal thrombocytosis is
underlying myeloproliferative disorder or myelodysplastic neoplasm
275
causes of reactive thrombocytosis
```
-iron deficiency and blood loss
infection
rheum
inflammation
surgery
malignancy
hyposplenism
```
276
symptoms of thrombocytosis
mostly asymptomatic
| but could have thrombotic or bleeding complications
277
initial inx and further INX for thrombocytosis
-ESR and CRP
-blood smear
iron status
occult malignancy
malignancy inx
->40 check CXR, pelvic USS women consider
FBC repeat 3 months from assessment
if persistent unexplained do haem assessment
haem-myeloproliferative
- bone marrow aspirate
- JAK mutation
278
management thrombocytosis
blood thinner
279
thrombocytopaenia symptoms
bleeding or bruises
epistaxis
menorrhagia
tiredness
280
drugs that cause thrombocytopaenia
H2 blockers
paroxetine
furosemide
metronidazole
281
causes of thrombocytopaenia
-revent viral infeciton
-drugs
chronic liver disease
ITP
hep c
hiv
h.pylori
bone marrow disorders eg leukaemia
inherited platelet disorders
TTP
HUS
acut folate deficiency
282
thrombocytopaenia with bleeding management
urgent referral
283
thrombocytopaenia of <30
urgent referral
284
30-50 platelets
semi urgent referral
285
>50-100 and impending surgery or on antiplatelets/ coagulants
semi-urgent referral
286
>50-100 and no impending surgeery
routine referral
287
>100 platelets
monitor
288
neutropaenia definition
<1.5
289
causes of neutropaenia
-benign ethnic neutropaenia
-drug induced or agranulocytosis
primary immune neutropaenia- children
viral eg glandular fever
primary haem malignancy although rare isolated
autoimmune eg SLE
haematinic deficiency also rare isolated
marrow infiltration eg mets
chronic idiopathic neutropaenia= dx of exclusion
HIV, hep c
290
causes of agranulocytosis
```
cytotoxics
-antibiotics eg nitrof, erythromycin
anticonvulsants
phenytoin
NSAID
gold
INFLIXIMAB
carbimazole and prophylthiouracil
spironalactone
beta blocker
closapine, olanzapine
omeprazole
allopurinol
```
291
management agranulocytosis
stop drug
| Granulocytic stimulating factor
292
inx for neutropaenia
```
persistently <1
blood film
chronic viral serology
monospot for EBV
haematinics B12 AND Folate
ANA and RF- SLE
antineutrophil antibodies- primary immune neutropaenia
```
293
refer neutropaenia if
suspiscion of progressive or serious disease
-with anaemia or thrombocytopaenia
more severe neutropaenia
persistently unexplained <1
294
polcytheamia
abnormal accumulation of RBC
295
haematocrit is
proportion of RBC
296
haemoglobin is
g/L
297
relative polcythaemia causes
dehydration
diuretics
realtive amount as plasma is reduced but RBC is same so proportion is higher
298
true polycythaemia causes
due to an excess production of RBC and too many red cells
- EPO from chronic low o2, COPD
- androgens
- malignancy polcythaemia vera
- renal cancer
- CKD over treatment
299
haematopoiesis is the
production of blood cells
300
what controls neutrophils production
GCSF granulocyte colony stimulating factor
301
how is blood cells made
from stem cells that divide into blast cells which are primitive and then undergo maturation
302
lymphocyte formation
either become T or B cells
mature and then circulate particularly in the lymp system
they then proliferate at sites where they find antigens
-T cells become cytotoxic at sites
B cells return to bone marrow to become plasma cells and secrete antibodies
303
where are blood cells broken down
liver and spleen
reticulo-endothelial system
-macrophages find cells at end of life and break down
hence
hyposplenism decreased break down
hypersplenism increased break down
304
red cell life span
120 days
305
platelet life span
7-10 days
306
neutrophils
8 hours
307
processes to halt bleeding
vasoconstriction
platelets
coagulation cascade
308
disorders of primary haemostasis- platelets
1. collagen -ehlers danlos
2. platelet function bernard soulier disease
3. platelet number: thrombocytopaenia
4. vWF
309
pattern of bleeding for primary haemostasis
```
-easy bruising
prolonged bleeding from cuts
menorrhagia
epistaxis
bleeding after trauma
```
310
disorders of secondary haemostasis
1. haemophilia
2. warfarin and heparin
3. liver disease
4. DIC
311
pattern of bleeding in secondary haemostasis
bleeding is prolonged and can be delayed
| bleeding into deep sites eg muscles and joints
312
acquired coagulation disorders
```
more common than inherited
-liver disease
-bone marrow failure
-ITP
-drugs: warfarin, heparin, aspirin
-DIC
metabolic: uraemia renal failure
vit c scurvy
```
313
ITP cause
immune thrombocytopaenia
| caused by antiplatelet autoantibodies
314
presentation of ITP 2
acute
usually in children ,2 weeks after an infection
sudden self-limiting purpura
or chronic mostly in women
315
presentation of chronic ITP
-fluctuating course of bleeding, purpura, epistaxis and menorrhagia
no splenomegaly
316
inx findings for ITP
-increased megakaryocytes in marrow
| antiplatelet antibodies
317
treatment of ITP
if symptomatic or platelets <20
-prednisolone
-aim to keep >30
none if mild
318
liver disease factors
2,7 9 10 11
vit K dependent
also makes protein c and s and antithrombin
319
liver disease and 2 types of haemostasis problems with regards to bleeding
primary haemostasis
- thrombocytopaenia -splenomegaly
- platelet dysfunction-defective aggregation
secondary haemostasis
-synthesis of 2, 5, 7, 9, `10 11
-vit k dependent factors
reduced c and fibrinogen
320
presentation of liver disease bleeding problem
-can get both a bleeding or thrombosis picture
321
management of liver disease bleeding
- give FFP
- cryoprecipitate if low fibrinogen
- platelets if low
322
why can liver patients get thrombosis
-high vWF
-low ADAMTS13
high factor VIII
low plasminogen
high PAI
323
DIC pathology
- get loss of normal regulation of coagulation
- get coagulation all over the place and consumption of clotting factors and platelets so then cant clot where you need too
324
DIC causes
-obstetric complications: intrauterine death, placental abruption, amniotic fluid embolism
-sepsis
malignancy
trauma
vascular abnormalities
immunological reactions
-severe transfusion reactions
-transplant rejection
-reaction to toxin eg rec drug
325
clinical manifestations of DIC
- underlying cause
- petechial rash- non blanching
- bleeding
- microvascular thrombosis
- organ failure
326
lab features of DIC INX
```
prolonged APTT, PT, Bleeding time
low fibrinogen
low platelet count
increased fibrin degradation products eg D dimer
D dimer will be massively elevated
```
327
Rx approaches to DIC
- treat underlying cause
- platelet transfusion
- fresh frozen plasma and cryoprecipitate
- recombinant activated protein C
- rFVIIIa
- role of heparin controversial and unproven
328
function of vWF 2
1. ACTS as a carrier protein for factor viii
| 2. it binds to exposed collagen and is crucial for platelet adherence and activation
329
coagulation what defines
coagulation precisely refers to the mechanism leading to the conversion of soluble fibrinogen to insoluble fibrin
330
function of antithrombin, protein c and s
- group of physiological anticoagulant substances that naturally inhibit the coagulation system
- stop all the circulating fibrinogen clotting at once
331
what breaks down the fibrin clot
plasmin
332
what prolongs thrombin time
```
process of turning fibrinogen to fibrin
-low fibrinogen
abnormal fibrinogen
heparin
high level of FDP
```
333
Hereditary haemorrhagic telangiectasia pathology
dominant inherited conditions caused by mutations in the genes encoding for Endoglin and activin receptor like kinase
which are endothelial cell receptors for transforming growth factor beta TGF-B
means blood vessels dont form properly
334
presentation of HHT
- telangiectasia and small aneurysms on fingertips, face and tongue, nasal passages and lungs
- some develop larger pulmonary arteriovenous malformations PAVM that cause arterial hypoxaemia
- patients present with recurrent bleeds- epistaxis, iron deficiency
335
management HHT
-can be difficult due to multiple bleeds
| regular iron therapy
336
haemophilia a is due to
flip tip inversion in factor 8 gene on X chromosome
337
haemophilia b
mutation defeciency in factor 9
338
most common severe inherited bleeding disorder
haemophilis
339
pathology of haemophilia
x-linked recessive
340
presentation of haemophilia
```
depends on severity
often in early life
often after surgery or trauma
bleeds into joint and muscles
leads to crippling arthropathy and haematomas
```
341
diagnosis of haemophilia
-fhx- female carrier, antenatal
-clinical suspicion
-prolonged bleeding and re-bleeding
excessive bruising when they becom mobile
bleeding on vaccinations/ IM injections
unusual surgical bleed
342
coagulation results for haemophilia
```
prolonged APTT
normal PT
normal bleeding time
normal fibrinogen
decreased factor 8 or 9
```
343
severe haemophilia level and complications
-<1% factor or <0.01
recurrent spontaneous joint bleeds
chronic joint damage
spontaneous intracranial bleeds-rarer
344
moderate haemophilia level and complications
1-5% 0.01-0.05
bleeding with minor trauma
subcutaneous, intramuscular
345
mild haemophilia level and complications
6-49% 0.06 to 0.49
bleeding with surgery or dental work
bleeding with major trauma
so can present later on in life
346
normal haemophilia level
50-150%
| normal range
347
management of haemophilia
1.recombinant synthetic factor 8 or 9
given on demand for bleeding episodes
targeted prophylaxis prior to surgery or dental work
2. Fc fusion and pegylation-extends half life f8
3. antifibrinolytics
4. DDAVP in mild disease vasopressin
5. physio for joints
6. emicuzimab to activate factor 10- so brings together factor 9a and 10 which f8 usually does so can make a thrombus
7. avoid NSAID and IM injection
348
who gets primary prophylaxis for haemophilia
severe haemophilia
| to try and turn them into moderate phenotype
349
risk of haemophilia treatment
inhibitor formation
-anti factor 8 antibodies
increased bleeding unresponsive to factor 8 therapy
needs bypassing agents and immune tolerance induction
350
complications of haemophilia
-spontaneous bleeding in severe patients- joint bleeds
-increased bleeding with trauma
surgical, iatrogenic bleeding
351
haemophilia B management
- recombinant factor 9
- plasma derived factor 9
- do not automatically give tranexamic acid
352
von willebrand disease pathology
```
low vWF
-defective platelet adhesion
-vWF protects f8 from degradation
autosomal dominant mostly
variable phenotype usually mild
```
353
presentation of vWF
```
primary bleeding disorder
-mucosal bleeding
easy bruising
dental bleeding
epistaxis
menorrhagia
surgical and post-trauma bleeding
v.rare to get haemarthroses
```
354
vWF inx findings
increased APTT
normal PT
increased bleeding time
normal platelets
low vWF and factor 8
355
management vWF
- give desmopressin to increase vWF levels
- tranexamic acid for mucosal bleeding
- haemostasis with selected factor 8 concentrates if more severe
356
types of vwf
type 1=partial quantative
type 2a= problem with vWF release or half life
2b=problem vwf and collagen interaction
2n= lack of f8 binding - similar to mild haemophilia
3= total qualitative behaves like severe haemophilia A
357
thrombosis virwchow triad
endothelial injury
stasis
hypercoaguability
358
risk factors for thrombosis
```
acquired risk factors
surgery
active malignancy
pregnancy puerpuerium
oestrogen medication
antiphos antibodies
prolonged immobility
smoking
obesity
```
inherited risk factors
inherited thrombophilia
fhx
359
classify VTE
provoked
eg pregnancy, oestrogen, trauma, surgery <3months, bed rest, travel >6hrs, active malignancy
unprovoked
all others
360
inx for VTE
-blood test- polcythaemia vera, anaemic chronic disease
well's score 1 or less
-do a D dimer
Well's score 2 or more
-straight to USS
radiology inx
unprovoked- look for malignancy
thrombophilia screen- antiphos and hereditary
361
what elevates D dimer
pregnnacy
infection
inflammation
malignancy
362
DVT symptoms
calf swelling
localised pain
asymmetric oedema
prominent superfiical veins
363
management of DVT 1st line
first line= doac
364
management DVT with heparin induced thrombocytopaenia
fondaparinux
365
management of DVT with renal impairment
unfractionated heparin
366
management of DVT in pregnancy
LMWH
367
management of DVT and obese
LMWH
368
management of DVT and risk of bleeding
UFH
369
management of DVT and active cancer
given LMWH for heparin
370
management of DVT with active bleeding
IVC filters
371
management of DVT and breast feeding
warfarin
372
management after dvt
-unprovoked= warfarin for 6 months to life long
-provoked- warfarin for 3 months
unless active malignancy give LMWH
373
Unfractionated heparin
```
initial bolus then given by cont.IV
can also be SC
half life 45 mins
potentiate antithrombin
reticuloendothelial clearance
```
used for
- bleeding as can be reversed
- renal failure
sore
needs APTT monitoring
heparin induced thrombocytopaenia
374
reversal agent for UFH
protamine
375
LMWH
```
preferred option
potentiate antithrombin
SC once a day
renally cleared
half life 12 hours
```
used
pregnancy and obese and cancer
```
cons
not renally impaired <30
HIT
sore
needs monitoring if pregnant or obese or cancer
```
376
LMWH reversal
protamine reverses 60% of LMWH
377
side effects heparin
bleeding
HIT
osteoporosis
378
management of bleeding unfractionated heparin
stop infusion
| protamine sulphate
379
management of LMWH bleeding
withold next dose
| protamine sulphate
380
Heparin induced thrombocytopaenia
is an immune reaction- forms antibodies against complexes of platelet factor 4 in heparin
develops 5-10 days post-treatment
prothrombotic condition
381
management of HIT
stop non -heparin
| use fondaparinux
382
warfarin is
an oral anticoagulant
vitamin K antagonist 2,7,10, 9
half life days
need to give with heparin initially
383
monitoring warfarin
use INR
high INR more likely to bleed
low INR more likely to clot
use prothrombin time
384
interactions of warfarin
metabolised by the CYP450 system
so enzyme inhibitors incerase INR
-omeprazole erythromycin, cranberry juice
reduce INR
-rifampicin, anti-epileptics
385
complications of warfarin
```
haemorrhage
teratogenic
thrombotic complications if subtherapeutic
interactions with meds
interacts with alcohol
poor INR control if liver dysfunction
itchy maculopapular rash
alopecia
```
soft tissue necrosis
-rare , mostly skin, breast and buttocks , thrombosis in venules
due to reduced protein c and s
386
warfarin INR 4.5-6.9 and low risk
reduce warfarin or withold dose
387
warfarin inr 4.5-6.9 high risk
give vitamin K
withold warfarin
check INR at 24 hrs
388
warfarin INR >7
vit K
withold warfarin
check INR at 24hrs
389
warfarin and bleeding minor
vitamin K
withold warfarin
chec INR
390
warfarin and major bleeding
vit K and beriplex or prothrombin complex concentrate
withold warfarin
immediate check PT and APTT
consider other factors contributing
391
warfarin and pregnancy effects
foetal warfarin syndrome
-highest 6-12 weeks
cartilage and bone development
risk of bleeding
foetal CNS abnormalities 2nd and 3rd trimester
foetal haemorrhagic risk 3rd trimester
392
warfarin interaction with other drugs
```
antibiotics
NSAID
amiodarone
antiepilepetics
azole
statins
H2 antagonists and PPI
-sick day rules
alcohol
```
393
DOAC 2 types
factor xa inhibitors - eg apixaban, rivaroxaban
| direct thrombin inhibitors = dabigatran
394
DOAC pros
no monitoring
no direct interactions
lower risk of bleeding
395
disadvantages of DOAC
cant use in renal impairment
not for metallic heart valves
still developing reversal
not for anti phos patients
396
DOAC reversal
currently in development
idarucizumab
397
fondaparinux
glyoprotein
anti thrombotic
SC given
used for heparin induced thrombocytopaenia
cant monitor and not reversible
398
argatroban
direct thrombin inhibitor
IV
heparin induced thrombocytopaenia
IV infusion difficult
not reversible
399
A 6-month-old boy with no previous medical problems presents with fever and painful swelling of the hands and feet. His parents are concerned because he has been inconsolable for 6 hours. The infant has been refusing bottles and has needed fewer nappy changes over the last 2 days
sickle cell
400
Very young children may present with jaundice, haemolysis, or splenic sequestration crisis. For children older than 4 months, presentation may include swelling of the joints, especially dactylitis, leukocytosis in the absence of infection, protuberant abdomen (often with umbilical hernia), cardiac systolic flow murmur, and maxillary hypertrophy with overbite.
sickle cell
401
FasA 45-year-old man without symptoms has a routine FBC done prior to donating blood for the first time. He is informed that the haemoglobin concentration is slightly reduced, with an increase in mean corpuscular haemoglobin concentration (MCHC). Spherocytes are seen on the smear, and the serum bilirubin (mainly unconjugated) is slightly elevated. On examination, he is noted to have an enlarged spleen, which is just palpable.
hereditary spherocytosis
402
anaemia michroc causes
iron deficiency anaemia
| haemoglobinopathies
403
normoc anaemia causes
```
chronic disease anaemia
bone marrow failure
haemodilution
sickle cell
haemolysis
EPO
```
404
Macrocytic anaemia
haematinics deficiency
| haemolysis
405
iron deficiency anaemia
low ferritin
high transferrin
low iron
high TIBC
406
chronic disease anaemia
high ferritin
low transferrin
low iron
low TIBC
407
ferritin
stores iron
in tissue and macrophage
can rise in acute phase response
408
serum iron
fluctuates with inflammation
409
transferrin
transports iron
raised in iron deficiency as body tries to increase movement
low in chronic disease
410
features of anaemia
```
kolionychia
fatigue dyspnoea pallor
faint palpitation
headache tinnitus
flow murmurs
cardiac enlargement
```
411
iron deficiency anaemia
cause
-blood loss, diet, malabsorption, menstruation, GI
inx
low iron
low ferritin
high transferrin
412
management of IDA
need to prove it first
- menstruating women dont need to
- everyone else gets upper GI endoscopy and colonoscopy
- consider cancer referral eg >60 and IDA =colorectal
1. ferrous sulfate
-until FBC normal and then for 3 months after
20g/l rise over 3 weeks
SE nausea, abdo discomfort, diarrhoea
if not tolerated
2. ferrous fumarate
3. ferrous gumarate
4. IV iron
413
steps of inx of IDA
1. iron studies
2. check coeliac screen TTG
3. pre menopausal women with no fhx of colorectal cancer or no symptoms then Iron replacement
- do an OGD on premenopasual women if upper GI symptoms and do colonoscopy if fhx of colorectal cancer
4. everyone else colonoscopy and OGD
414
chronic disease normocytic anaemia
iron gets stuck in stores
new red cells are hard to make
low transferrin
over time MCV starts to fall
415
causes chronic disease and anaemia
inflammation
infection
cancer
anaemia or renal disease
416
dx of chronic disease anaemia
inflamamtory markers
low transferrin
ferritin normal or raised in acute phase
417
management chronic disease anaemia
dont prescribe iron (unless also low ferritin)
| treat the cause
418
bone marrow failure
normocytic anaemia
419
megaloblastic meaning
hyersegmented neurophils
| seen with haematinic and haemolysis
420
causes of macrocytic anaemia
haematinic deficiency
haemolysis
```
normoblastic
alcohol
liver disease
hypothyroid
drugs
```
421
inx macrocyric anaemia
b12 and folate
blood film
lft tft
bone marrow
422
low folate if
```
poor diet
alcohol
elderly
malabsorb
drugs eg antifolate
```
423
causes low folate
1. diet deficiency
2. malabsorption eg coeliac disease
3. increased requirement pregnancy
4. anti folate drugs
424
causes low vit b12
1. pernicious anaemia
2. distal malabsorption eg crohn's
3. gastrectomy
4. diet
425
b12 absorption
needs intrinsic factor
426
features of haematinic deficiency
```
glossitis
anaemia
neuropsychiatric- irritable
paraesthesia
peripheral neuropathy
```
427
risk of low b12
```
subacute combined degeneration of spinal cord
-both LMN and UMn signs
extensor plantar
absent knee jerks
absent ankle jerks
```
428
causes of low b12
1. pernicious anaemia
| 2. ileal disease or absorption states eg crohn's
429
pernicious anaemia is
usually older patients
autoimmune disease with atrophic gastritis leads to lack of IF from parietal cells
antibodies to intrinsic factor in 50%
dietary b12 remains unbound and cant be absorbed
430
inx for pernicious anaemia
```
anaemic
macrocytosis
low b12
decreased reticulocytes
IF antibodies
```
431
management b12 deficiency
hydroxycobalamin injection
432
assoc, to pernicious anaemia
female
blue eyes
group a
autoimmune eg thyroid, vitiligo
433
management haematinic deficiency
B12 must be replaced first
never give folate without checking b12
risk of subacute combined cord
434
haemolysis
is shortened red cell survival
| normoctyci or macrocytic
435
causes haemolysis
```
sickle cell
thalassaemia
G6PD
hereditary spherocytosis
autoimmune haemolysis
DIC
eclampsia
malaria
```
436
CF haemolysis
jaundice
dark urine
anaemia
splenomegaly
437
autoimmune haemolysis anaemia
mediated by autoantibodies
| cold and warm
438
warm AHA
antibody IgG causes haemolysis best at body temperature and mostly at extravascular sites eg spleen
seen in lupus, lymphoma, CLL, methyldopa, penicillin
management
steroids
immune eg rituximab
splenectomy
439
cold AHA
usually IgM, best haemolysis at 4 degrees
intravascular
raynauds and acrocyanosis
causes
-neoplasia, lymphoma, ebv
responds less well to steroids
440
AHA findings
increased bilirubin
increased reticulocytes
increased LDH
decreased haptoglobin
441
aplastic anaemia
body stop producing enough new blood cells
normocytic anaemia
leucopaenia
thrombocytopaenia
```
causes
idiopathic
congenital
drugs eg phenytoin
infection- parvovirus, hepatitis
sickle cell crisis
```
442
G6PD presentation
```
rapid anaemia and jaundice attacks
neonatal jaundice
intravascular haemolysis
gallstones
splenomegaly
usually male
of african or asian descent
hx of recent exposure to drug or infection can precipitate a crisis
```
443
inx G6PD
heinz bodies
bite and blister cells on blood film
enzyme assay
444
drugs and g6pd
sulpha containing drugs
ciprofloxacin
antimalarials
445
hereditary spherocytosis
autosomal dominant
less deformable spherical RBC so trapped in spleen
extravascular haemolysis
signs
-neonatal jaundice
chronic symptoms and crisis
splenomegaly
WHITE ANSCESTRY
446
inx for hereditary spherocytosis
osmotic fragility
447
inx for haemolysis
```
macrocytic or normocytic anaemia
reticylocytes increased
bilirubinaemia unconjugated
LDH increased
decreased haptoglobin
liver enzymes
```
coomb's test direct for presence of antibodies- autoimmune
448
inx haemoglobinopathies
fhx
fbc
blood film (target cells and howell jolly)
HPLC electrophoresis
449
normal haemoglobin types
4 globin and 4 haem molecules
```
HbA= >95% 2 alpha and 2 beta
HbA2= 2-3% 2 alpha and 2 delta chains
HbF= 1% 2 alpha and 2 gamma
```
450
foetal haemoglobin
is mostly HbF 2 alpha and 2 gamm
| then switchese to HbA at 2-3 months of life
451
thalassaemia are
Group of genetic disorders with decreased production rates of either alpha or beta chains
autosomal recessive
452
inx thalassaemia
microcytic hypochromic anaemia
beta thalssaemia trait
mild anaemia, elevated HbA2, normal ferritin, microcytosis disproportionate to anaemia
alpha thalssaemia
mild anaemia, normal HLPC normal ferritin
diagnosis of exclusion
deficiency of alpha chains
453
management of thalsaemia
asymptomatic no treatment required
| avoid unnecessary iron supplemenetation
454
types beta t
beta thalassaemia
major
minor/ trait
intermedia
455
beta thal major
severe microcytic anaemia
usually point mutation chromosome11
both genes mutated no beta production so no HbA production
RBC haemolyse while still in marrow
```
CF
-severe anaemia by 3-6 months
jaundice and gallstones
failure to thrive
splenomegaly
bone changes- skull bossing dips
```
456
management beta t major
chronic transfusion- life long transfusions
risk alloantibody formation
transfusions result in iron overload so need chelation
bone marrow transplant
457
An 8-month-old boy of Mediterranean origin presents with pallor and abdominal distension, both of which are progressive. The perinatal history was uneventful, and the boy is noted to be pale, with poor feeding, decreased activity, and failure to thrive. Hepatosplenomegaly and mild bony abnormalities of the skull are noted (frontal and parietal bossing).
beta thalassaemia major
458
sickle cell genes
```
point mutation beta chain
single nuclei GAG to GTG in beta globin chain
B to B2
autosomal recessive
HbAA normal
HbAS carrier
Hb SS sickle cell
```
459
pathology sickle cell
when hb is deoxygenated and crystalises should remain flexible and same shape
in sickle cell when deoxygenated it crystalises and forms a sickle shaped red blood cells - clump together
- so are damaged in small blood vessels
- haemolysis and block the vessels causing complicaitons
460
sickling (deoxygenated haemoglobin polymerisation and red cell sickling) is increased by
```
hypoxia
infection
acidosis
cold temp
low levels of HbF
(usually undetectable in adults, but higher levels are protective)
```
461
Clinical features of sickle cell
chronic haemolytic anaemia
jaundice
increased bilirubin
increased reticulocytes
462
inx sickle cell
guthrie test
| haemoglobin electrophoresis definitive dx
463
management sickle cell
- vaccinations- pneumoccoccal
- crises prevention and support
- intermittent transfusion to reduce HuS
- chelation
- hydroxycarbamide- increases HbF
- bone marrow transplant- curative
464
admission for sickle cell for
admit all in sickle cell crisis
| unless well adult or child with mild pain and normal temperature
465
sickle cell complications
```
acute vaso-occlusive bone pain
acute organ VOC
sequestration into liver or spleen
infection- hyposplenism
acute anaemia
painful vaso-occlusive crises
acute end organ damage
acute chest syndrome
```
466
types of anaemia in sickle cell
- splenic sequestration- RBC get trapped in spleen so spleen enlarges- increased reticulocytes
- aplastic crisis- parvovirus decreased reticulocytes
- haemolytic crisis
467
vaso-occlusive crisis
blockage or vaso-occlusion of small vessels
causes down stream ischaemia and infarction
trigger- cold, dehydrated, infection, hypoxia
```
CF
-dacytlitis in children
limb back pain
mesenteric ischaemia- abdo pain
CNS infarction- strokes seizures
priaprism
```
```
management
-depends on severity
analgesia at home if mild and no fever
aim for adequate pain control in 60 minutes
opiates
ensure hydrated
avoid hypoxia
examine inx cause
watch for chest symptoms
```
468
acute end organ damage sickle cell
mostly affects the lungs, brain, penis
chest crises
strokes
priaprism- impotence and infertility (alpha agonist)
469
acute chest syndrome sickle cell
pulmonary infiltrates
causes pain, fever, wheeze and cough
serious
main cause are fat embolism from bone marrow or an infection
management
oxygen
analgesia
bronchodialtors if wheezing
470
chronic complications sickle cell
chronic anaemia
chronic end organ damage- lung, kidney, heart spleen
transfusion related
iron overload
alloantibody formation
471
chronic end organ damage in sickle cell
spleen atrophies over first few yrs of life
renal failure
pulmonary hypertension
cerevrobascualr disease
472
general sickle cell problems
```
splenic infarction
infection due to hyposplenism
poor growth
chronic renal failure
gallstones
iron overload
retinal damage
lung damage- hypoxia- fibrosis
```
473
management of painful vaso-occlusive crisis
```
inx
-full obs
septic screen in indicated
bloods
electrophoresis in new patients only
```
other
CXR if chest signs
ABG if sats low
patients on DFO with diarrhoea/ pain should stop DFO and stool screen for yersinia
hydroxycarbamide check neutropaenia or thrombocytopaenia
mostly supportive- oxygen, hydrate, abx if indicated
if pain mild/mod = analgesia ladder
if pain mod/ severe or patient used analgesia ladder then go to morphine SC or oramorph and reassess 30 minutes
if pain persist add another dose 5-10
reassess and if still persist then consider alternative to morphine eg fentanyl and consult pain team
474
indication for transfusion in sickle cell crisis
severe chest crisis
suspected CNS event
multiorgan failure
475
organisms that affect immunocompromised
```
psuedomonas
stenotrophonomas maltophilia
pneumocysitis jiroveci
candidaemia
line assoc.
```
476
psuedomonas aeruginosa is
```
gram negative bacilli
aerobic
green blue colour
cut grass odour
oxidase positive gold
found in soil and moist environment
colonises human moist sites
```
477
diseases pseudomonas
```
catheter UTI
HAP
VAP
line infection and secondary bacteraemia
surgical site infection
```
478
rx pseudonomas
```
antipseudomonal
fluoroquinolones eg ciprofloxacin
piperacillin
gent
meropenem
```
479
stenotrophomonas maltophilia
aerobic gram negative bacilli
pale yellow on culture
ammonia odour
oxidase negative
480
risks stenotrophonomas
-found on lots of sites
medical devices, nebulisers, disinfectant and resistance
risk factors for invasvie disease
-prolonged neutropaenia
-carbapenem, cephalosporins, fluoroquinolones use
lines devices
causes
bacteraemia
resp infection
UTI
481
rx stenotrophonomas
trimethoprim co-trimox
levofloxacin
minocycline
482
pneumocystis jiroveci affects
-HIV CD4 <200 or AIDS
| immunocompromised with malignancies, bone marrow transplant
483
cf pneumocystis
```
progressive dyspnoea
fever
cough
pneumonia
tachycardia
tachypnoea
```
484
inx pneumocystis
CXR= bilateral diffuse infiltrates
| high resolution CT HRCT ground glass changes
485
diagnosis pneumocystis
induced sputum or bronchoscopic alveolar lavage and
immunofluorescent or PCR
cant culture
hypoxaemia
486
management pneumocystis
co-trimoxazole plus prednisolone
21 days HIV
14-21 days
487
candida
yeast infection
skin gi tract, gu tract
part of normal flora but when candidaemia in blood stream its significant
488
inx candidaemia
blood cultures
| from both lines and periphery
489
risk factors candidaemia
```
exposure to broad spec abx
immunocompromised
neutropaenia
indwelling IV catheters
TPN
GI or thoracic surgery
solid organ transplant
```
490
management candidaemia
antifungal treatment 14 days
repeat blood cultures every 48hrs until negative blood culture result
DURATION 14 DAYS FROM FIRST NEGATIVE BLOOD CULTURE
longer if endocarditis
```
remove all indwelling catheters
ECHO
opthalmology review
review souces
management risk factors eg TPN, reduce immunosuppression
```
491
antifungal for candida
triazoles
echinocandins eg caspofungin
polyenes eg amphotericin B
492
vancomycin resistant enterococci
gram positive bacteria
spread in healthcare
broad spectrum of infection
difficult to distinguish between carriage and infection
493
rf for VRE
```
broad spec abx
IV lines
catheter
haem malignancy
increased age
dm
surgery
long patient
ICU
```
494
treatment VRE
linezolid
daptomycin
from then on considered colonised and consider patient isolation
495
line assoc. infections
get into blood stream
| eg staph aureus and staph epidermidis are common
496
staph aureus rx
flucox 2 weeks
| unless MRSA
497
staph epidermidis
more common immunocompromised
normal skin flora
causes biofilms that grown on devices
498
peripheral venous cannula infections
local phlebitis to cellulitis
PVC insertion bundle management
review daily PVC maintenance
499
management central line infection
culture from line
remove line
duration of abx depends on organism
500
spleen functions
filtration
regulates inflammation
maturation of B and t cells
haemostasis
501
types of asplenia/ hyposplenia
acquired- surgical or splenic infarction
```
hyposplenia
sickle cell anaemia
GVHD
coeliac
HIV
alcoholic
```
502
bacterial organisms assoc. to sepsis post-splenectomy
encapsulated bacteria
-strep pneumoniae
HiB
N. meningitidis
unusual gram negative bacteria
bordetella
salmonella
paraistes
babesia microti
plasmodium falciparum
anaplasma
503
management of post-splenectomy
penicillin V life long
clarithromycin life long
vaccinations
pneumonooccal
meningococcal
influenza
vector avoidance
