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year 5 > Haematology > Flashcards

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
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2
Q

classification of myeloma

A

IgG
IgA
light chain only

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3
Q

common incidence of rmyeloma

A

60-70yrs
it is uncommon
2% of all cancer dx

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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.

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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
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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
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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
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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
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9
Q

morphology of myeloma plasma cells

A

eccentric nucleus surrounded by a paler area and blue cytoplasm

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10
Q

if no evidence of myeloma related tissue impairment with diagnosis=

A

smouldering myeloma

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11
Q

if patients have evidence of myeloma with tissue impairment

A

=multiple myeloma

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12
Q

course time of multiple myeloma

A
  1. asymptomatic phase with either MGUS or smouldering myeloma
  2. 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
  3. most patients respond well to initial therapy and will achieve reduction in paraprotein and some to the point of remission
  4. remission or plateau phase
    6, relapse and remitting with gradually getting worse
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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
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14
Q

emergency complications for myeloma

A 
-renal failure
hypercalcaemia
hyperviscosity
hyperuricaemia
spinal cord compression -radiotherapy and bisphosphonates
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15
Q

asymptomatic multiple myeloma management

A

watchful waiting can be used

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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

  1. maintenance phase
    immunomodulatory drug thalidomide
  2. relapse
    thalidomide plus steroid
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17
Q

non-intensive approach for multiple myeloma management

A
  1. induction
    thalidomide+ dexamethasone + alkylator eg chlophosphamide/ mephalan

2.maintenace phase
thalidomide

3.relapse
bortezomib plus steroid

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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

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19
Q

poor prognostic factors for multiple myeloma

A

high B2 microglobulin

low albumin at diagnosis

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20
Q

acute leukaemias myeloid vs lymphoblastic age onset

A 
myeloid= adults
lymphoblastic= children
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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

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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
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23
Q

what conditions can the philadelphia chromosome be found in

A

CML

ALL

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24
Q

risk factors for leukaemias

A

ionising radiation
radiotherapy
cytotoxic drugs
retroviruses- HTLV-1 adult T cell leukaemias

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25
Q

diagnosis findings for leukaemia

A
  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
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26
Q

division of ALL

A

t or b precursor

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27
Q

AML types

A
  • geentic abnormalities
  • myelodysplasia changes
  • neoplasms theray related
  • myeloid sarcoma
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28
Q

presentation of acute myeloid leukaemia

A

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

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29
Q

presentation of acute lymphoblastic leukaemia

A

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

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30
Q

management conservaitve acute leukaemias

A
  • if very elderly or serious co-morbidities
  • supportive treatment
  • can give low intensity cytosine arabinoside
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31
Q

immediate management of acute leukaemias

A
  • 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
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32
Q

stages of acute leukaemias management

A
  • 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
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33
Q

chemotherapy for acute leukaemias ALL

A
  • 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
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34
Q

ALL what prophylactic treatment can be given for CNS

A

cranial irradiation

intrathecal methotrexate

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35
Q

AML chemotherapy

A

induction: danuroubicin
consolidation: cytarabine, amsacrin

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36
Q

what should be given to acute leukaemias if philadelphia positive

A

IMATINIB

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37
Q

PROGNOSIS ALL in children

A

> 90%

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38
Q

what is acute promyelocytic leukaemia

A

translocation in 15-17
treat with Vit A compound
assoc. to DIC

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39
Q

child with unexplained hepatosplenomegaly or unexplained petechiae should

A

refer urgently to specialist- ALL

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40
Q

child with pallor, persistent fatigue, unexplained fever

A

consider ALL

need urgent FBC <48hrs

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41
Q

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.

A

ALL

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42
Q

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.

A

AML

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43
Q

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.

A

Multiple myeloma

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44
Q

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.

A

polycythaemia vera

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45
Q

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

A

PMF

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46
Q

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.

A

CML

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47
Q

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).

A

CLL

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48
Q

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.

A

hodgkin’s

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49
Q

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.

A

non-hodgkin’s

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50
Q

pathology of chronic leukaemias

A

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
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51
Q

diagnosis chronic leukaemias

A
  • abnormal blood count- raised WCC

- examination of bone marrow- immunophenotyping

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52
Q

chronic myeloid leukaemia pathology

A
  • 30-80 yrs
  • proliferation of all haematopoietic lineages, but predominantly granulocytic series- myeloid
  • philadelphia chromosome
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53
Q

how is the philadelphia chromosome formed

A
  • 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
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54
Q

phases of CML

A

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
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55
Q

chronic vs acute leukaemias

A

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

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56
Q

presentation CML

A

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

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57
Q

inx CML and results

A 
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

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58
Q

management of chronic phase CML

A
  • 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

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59
Q

management of accelerated phase treatment CML

A

TKI therapy
allogenic HSCT
hydroxycarbamide in older patients

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60
Q

CLL presentation

A

older
asymptomatic
often diagnosed on isolated lymphocytosis
50% have lymph nodes, splenomegaly
-anaemia, infections, painless lymphadenopathy, systemic symptoms, such as night sweats, weight loss

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61
Q

inx CLL

A

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

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62
Q

assoc. autoimmune phenomena to CLL

A
  • ITP - petechiae development

- autoimmune haemolysis

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63
Q

management of stage A CLL

A

watch and wait
no specific treatment
doesnt change life expectancy
treat if evidence of bone marrow failure

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64
Q

stage b and c or a with bone marrow failure

CLL management

A

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

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65
Q

lymphoma pathology

A

cancer of lymphocytes in lymph nodes and extra-nodal areas eg spleen, liver, bone marrow
-neoplasma arise from lymphoid tissue

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66
Q

presentation of lymphoma

A

-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
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67
Q

inx lymphoma

A

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

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68
Q

tissue lymph node biopsy for non hodgkin

A

follicular lymphoma

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69
Q

hodgkin lymphoma cell

A

reed sternberg cell

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70
Q

types of lymphoma

A 
hodgkin
non hodgkin
-b cell
-t cell
-high grade eg diffuse large B cell lymphoma 
low grade eg follicular
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71
Q

high grade non-h means

A

divides rapidly
present for a matter of weeks before dx
can be lifethreatening

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72
Q

low grade non h means

A

divides slowly
present for many months before dx
behave in an indolent fashion

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73
Q

hodgkin lymphoma features

A 
younger people
reed sternberg b cell
aggressive but highly curable 
painless, rubbery lymphadenopathy, asymtpomatic
cough, fever, haemoptysis, dyspnoea
b symptoms
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74
Q

management of hodgkins

A

ABVD doxorubicin, bleomycin, vinblastine and darcarbazine

combined with radiotherapy

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75
Q

non-hodgkin cause

A 
-no single causative abnormality described
but can be linked to
-HIV
EBV
HTLV-1
MALT lymphoma to h.pylori
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76
Q

presentation of non-hodgkin

A

can be widely disseminated at presentation including extranodal sites if high grade
-systemic upset
-hepatosplenomegaly
SVCO if compression or ascites

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77
Q

additional inx for non hodgkins

A 
bone marrow aspiration and trephine
immunophenotyping of surface antgens distinguish t and b cells
HIV
hep b and c testing 
immunoglobulin determination
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78
Q

management low grade NHL

A

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
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79
Q

management high grade NHL

A

chemotherapy
monocloanal antibody therapy
radiotherapy
HCST

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80
Q

management follicular lymphoma

stage 1 and 2
vs
stage 3 and 4

A

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

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81
Q

CHOP treatment for non hodgkin’s lymphoma

A

cyclophosphamide
doxorubulin
vinicristine
prednisolone

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82
Q

-young man
asymmetrical and painless superficial lymphadenopathy

alcohol induced nodal pain

A

HL

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83
Q

late middle age

lymphadenopathy

A

non hodgkin’s

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84
Q 
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).
A

factor 8 acquired haemophilia

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85
Q

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.

A

von willebrand factor

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86
Q

complications of CLL

A

-anaemia
hypogammaglobulinaemia (recurrent infection as low Ig)
warm autoimmune HA
Transform to high grade lymphoma- Richter transforamtion

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87
Q

what is Richter transformation

A

transformation CLL to non hodgkin lymphoma

  • lymph node swelling
  • fever without infection
  • weight loss
  • night sweats
  • nausea
  • abdominal pain
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88
Q

what are the myelproliferative neoplasms

A

disorders characterised by clonal proliferation of malignant bone marrow cells

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89
Q

which are the 4 myeloproliferative neoplasms

A
  1. essential thrombocytosis
  2. polycythaemia vera
  3. CML
  4. myelofibrosis
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90
Q

essential thrombocytosis pathology

A

megakarocyte proliferation results in an over-production of platelets

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91
Q

features of essential thrombocytosis 4

A
  • platelet count >600
  • thrombosis and haemorrhage can be seen
  • burning sensation in hand
  • JAK2
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92
Q

treatment of essential thrombocytosis

A
  • hydroxyurea- hydroxycarbamide lowers platelets

- Interferon alpha- low dose aspirin

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93
Q

polcythaemia vera inx findings

A
  • raised RBC
  • often increase neutrophils and platelets, basophils also
  • JAK2 mutation
  • Iron studies
  • renal and liver
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94
Q

features of polycythaemia vera

A 
over 60
hyperviscosity-thrombosis
pruritus
splenomegaly
haemorrhage
plethoric appearance
HTN
decreased ESR
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95
Q

treatment of polycythaemia vera

A
  • venesection= 1st line
  • aspirin
  • hydroxyurea
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96
Q

prognosis of polycythaemia vera 3

A
  • thrombotic events
  • 5-15% progress to myelofibrosis
  • 5-15% progress to acute myeloid leukaemia
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97
Q

myelofibrosis pathology

A
  • hyperplasia of abnormal megakeraryocytes
  • resultant release of platelet derived growth factor is thought to stimulate fibroblasts
  • leads to extensive scarring in bone marrow
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98
Q

features of myelofibrosis 3

A
  • insidious fatigue and weight loss
  • splenomegaly
  • elderly person with symptoms of anaemia
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99
Q

investigation findings of myelofibrosis 5

A
  • anaemia
  • increased WCC and platelet count
  • “tear drop” poikiocytes on blood film
  • “dry tap” therefore trephine biopsy needed
  • increased LDH and urate
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100
Q

cause of primary myelofibrosis

A

can be progression from polycytheamia vera or Essential thrombocytosis

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101
Q

causes of plasma cell dyscrasias 4

A

MGUS monoclonal gammopathy of undetermined significance
multiple myeloma
amyloidosis
plasmacytoma

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102
Q

how do we know that haematological malignancies arise from a single cell

A

clonality

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103
Q

CML pathology

A
  • reciprical translocation of c 22 and c9
  • get shortened 22 with bcr next to abl which creates mRNA for tyrosine kinase activity
  • philadelphia chromosome
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104
Q

concerning features of a lymph node

A 
>1cm
not mobile
rubbery
not tender
persistent
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105
Q

types of blood groups

A

o and a are commonest
o, a, b and AB
negative or positive

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106
Q

if o what blood is compatible

A

only o

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107
Q

if ab what blood is compatible

A

all

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108
Q

if b what blood is compatible

A

only b and o

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109
Q

if o negative what blood is compatible

A

only o negative

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110
Q

what does rhesus positive mean

A

means the red cell carry D antigen are D poisitive
85% of the population

D negative needs D negative

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111
Q

why do rhesus negative need to receive rhesus negative

A
  • 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

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112
Q

monitoring on blood transfusion how often

A

temperature and blood pressure every 30 mins

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113
Q

types of blood products

A

-whole blood-rarely used
-red cells
-platelets
fresh frozen plasma
cryoprecipitate
prothrombin complex

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114
Q

target haemoglobin for anaemia transfusion

when to transfuse

A

80 Hb for acute coronary syndrome

70 Hb for without acute coronary syndrome

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115
Q

target haemoglobin for after transfusion

A

acute coronary syndrome= 80-100

without= 70-90

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116
Q

target when to transfuse platelets

A
  • if bleeding or

- count <20 x10^9

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117
Q

how much should 1 unit of platelet increase platelet count

A

by 20x10^9

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118
Q

if surgery is planned at what platelet level should you get advice from haeamtology

A

<100

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119
Q

fresh frozen plasma indication

A

-used to correct clotting defects eg DIC, warfarin overdose, liver disease, thrombotic thrombocytopaenic purpura

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120
Q

types of plasma products

A

FFP
cryoprecipitate
prothrombin complex concentrate

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121
Q

indication for FFP

A

clinically significant bleeding but not major haemorrhage with clotting defects/ abnormal coag result

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122
Q

what is cryoprecipitate

A

factor 8 vWF, fibrinogen and factor 13

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123
Q

when is cryoprecipiate indicated

A

patients without major haemorrhage who have a

  • clinically significant bleed AND
  • fibrinogen <1.5
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124
Q

prothrombin complex concentration indication

A
  • for emergency reversal of warfarin in patients with either
    1. severe bleeding or
    2. head injury with suspected intracerebral haemorrhage

major haemorrhage

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125
Q

when is human albumin solution indicated

A

replace protein in hypoprotein aemic patient eg liver disease who is fluid overload
or abnormal paracentesis

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126
Q

definition of massive blood transfusion

A

replacement of an individuals’ entire blood volume in 24hours

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127
Q

complications of massive blood transfusion

A 
low platelets
low calcium
low clotting factors
increased potassium
hypothermia
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128
Q

acute haemorrhage blood product choice

A

use crossmatched if possible
but if not possible then use o negative
change to cross match as soon as possible

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129
Q

cautions for transfusing a patient with heart failure

A

give each unit over 4 hours with furosemide with alternate units
check for increased JVP and basal lung crackles
consider CVP line

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130
Q

how is autologous blood transfusion done

A

-patients have their own blood stored pre-op for later use

give

  • EPO to increase yield
  • intraoperative cell salvage with re-transfusion
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131
Q

definition of mild febrile reaction to blood transfusion

A

defined as a temperature of 38 and a rise of between 1-2 degrees from pre-transfusion values

but no other symptoms or signs

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132
Q

how is the risk of febrile reactions to blood transfusions now reduced

A

leuco-depletion

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133
Q

pathology of febrile reactions

A

reaction to white cells or cytokines released from white cells

patient already has antibodies to the WCC
or due to cytokines

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134
Q

definition of mild allergic reaction

A

-transient flushing urticaria or rash, sometimes with a respiratory wheeze

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135
Q

pathogen of allergic transfusion reactions

A

due to an allergen in the donor’s plasma

occurs more frequently in patients with other allergies and atopy

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136
Q

what products tend to cause allergic transfusion reactions

A

plasma or platelets transfusion

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137
Q

what products tend to cause febrile transfusion reactions

A

red cells and platelets

not usually plasma

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138
Q

definition of moderate febrile transfusion reaction

A

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

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139
Q

definition of moderate allergic reaction

A

-wheeze or angioedema with or without flushing/ urticaria

but without resp compromise or hypotension

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140
Q

definition of severe transfusion reaction febrile

A

reaction that fulfills the criteria of moderate and requires immediate medical attention or leads to admission or prolongs hospital stay

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141
Q

definition of severe allergic reactions

A

causing respiratory compromise or circulatory compromise which requires urgent medical intervention
or
anaphylaxis

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142
Q

acute transfusion reactions <24 hrs

A 
acute haemolytic
allergic/ anaphylaxis
infection
febrile non haemolytic
respiraotry - TACO/ TRALI
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143
Q

delayed transfusion reaction >24hrs

A

delayed haemolytic
infection
transfusion vs graft host disease
post transufion pupura

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144
Q

responding to a transfusion reaction

A
  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
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145
Q

acute haemolytic reaction pathology

A

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

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146
Q

cause of acute haemolytic reaction

A

usually due to failure of identification and checks

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147
Q

acute haemolytic transfusion reaction clinical presentation

A 
-agitated
chills
abdominal pain- loin pain 
dizzy
blood pressure collapse
can get fever 
dark urine
renal failure
DIC -bleeding
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148
Q

What is TRALI

A

transfusion related acute lung injury

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149
Q

presentation of TRALI

A
  • severe breathlessness and hypoxia within 6 hours of transfusion
  • consider in patient not responding to pulmonary oedema treatment
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150
Q

pathology of TRALI

A
  • 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
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151
Q

how is TRALI rates reduced

A
  • 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
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152
Q

inx findings TRALI

A

-pulmonary bilateral infiltrates

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153
Q

management of TRALI

A
  • supportive therapy
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154
Q

TACO is

A

transufsion associated circualtory overload
-pulmonary oedema
usually at end of transfusion

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155
Q

reducing risk of TACO

A
  • 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
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156
Q

presentation of TACO

A

acute respiratory distress due to acute pulmonary oedema occurring during or within a few hours of transfusion

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157
Q

who is at risk of TACO

A

-very old and very young
renal impairment
heart failure

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158
Q

what is the commonest cause of transfusion related death

A

TACO

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159
Q

management of TACOg

A

give diuretics
call senior stop
usually procedure

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160
Q

timing and acute transfusion reaction order

A

anaphylaxis 15 mins
acute haemolytic reaction within minutes collapse
TACO- during or just after
TRALI- 6 hrs after

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161
Q

delayed haemolytic transfusion reaction HTR pathology

A

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

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162
Q

importance of cross match

A

-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

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163
Q

what does a group and screen do

A

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”

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164
Q

what causes a delayed haemolytic reaction if blood given is crossmatched (aka should pick up all antibodies)

A
  • a new antibody has formed

- an alloantibody was missed

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165
Q

what does a crossmatch do

A

tests you blood directly with the selected donor blood to make sure compatible

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166
Q

presentation of DHTR

A 
several dayss to two to three weeks after 
-fever
falling haemoglobin or 
a rise in HB which is less than expected
jaundice
haemoglobinuir a
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167
Q

immunological transufsion reactions

A

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

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168
Q

non immunological transfusion reactions

A

mild mod severe allergy
infective
physiological- TACO

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169
Q

how often are observations done during a blood transfusion

A

before
after 15 mins
and at end of transfusion

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170
Q

management of febrile non haemolytic transfusion reaction

A 
stop transfusion and call senior
set of observations
determine severity
give paracetamol 
follow procedure
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171
Q

mild allergic reaction treatment

A

antihistamines

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172
Q

acute haemolytic reaction treatment

A

stop transfusion call senior

IV access and transfer to ITU-needs renal support

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173
Q

anaphylaxis treatment

A

adrenaline and antihistamines

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174
Q

bacterial contamination treatment

A

cultures and broad spec abx

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175
Q

TACO treatment

A

diuretics

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176
Q

TRALI treatment

A

oxygen and resp support

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177
Q

features of haemolysis

A

fall in hb
rise in bilirubin uncongugated
rise in LDH
blood film showing red cell spherocytosis

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178
Q

viral transmitted infection - how is the risk of this reduced

A

donor health check questionnaire
viral antibody testing
antigen testing
nucleic acid testing

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179
Q

what viruses are checked for

A

antibody HCV, HIV, HTLV1
antigen HBV
nucleic acid HCV and HIV and HEV

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180
Q

tranfusion assoc. graft versus host disease TA GVHD pathology

A

-engraftment of donor lymphocytes because the patient is immunosuppressed or the HLA is similar
causes multi-organ failure and death in all cases

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181
Q

how is TA GVHD risk reduced

A

IRRADIATED cellular blood components for all patients who are immunosuppressed

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182
Q

indications for irradiation

A

-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

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183
Q

presentation of transfusion assoc. graft vs host disease

A 
-4 to 30 days after 
immunosuppressed patient or HLA similar
increased temp
erythroderma
desquammation
diarrhoea 
abnormal LFT
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184
Q

management of TA vs GHD

A

steroids

but always fatal

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185
Q

how often can you donate blood and what ages

A

donate every 12 weeks

from 17 yrs to (66 unless good health no upper limit)

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186
Q

stages of blood donation 6

A
  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
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187
Q

after blood donation advice 4

A
  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
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188
Q

what testing is done on donated blood products 4

A
  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
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189
Q

what are the mandatory blood tests6

A 
hep b
hep c
hep e
HIV
HTLV1
syphilis
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190
Q

discretionary test on blood donated

A

cmv
malaria
west nile virus
HbS

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191
Q

HbS blood donation test is for

A

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

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192
Q

the whole blood process

A
  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
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193
Q

what anticoagulant is whole blood put into

A

citrate phoshate dextrose

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194
Q

what are red cells suspended in after centrifuge

A

SAG-M sodium adenin mannitol glucose

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195
Q

what are platelets suspended in after being pooled from 4 donors into a satellite bag

A

PAS platelet additive solution with a bit of plasma to reduce risk of allergic reactions

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196
Q

what is a RAD sure label

A

used to label if irradiated or not

-done by a chemical in the label which turns black on irradiation if the blood undergoes irradiation

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197
Q

UK specification for leucocyte depletion

A

-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

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198
Q

what steps means that CJD does not need to be tested for in blood donations

A
  • 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
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199
Q

shelf life red blood cells

A

35 days

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200
Q

storage temperature for red blood cells and where

A

2-6 degrees

only in blood fridge never any other

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201
Q

how long if out of the blood fridge till red blood cells have to be discarded

A

30minutes

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202
Q

how long does a red blood cell transfusion have to be completed in

A

2-4hours must be completed RBC in

203
Q

platelets how long shelf life

204
Q

how long should platelets be transfused over

A

30-60 minutes

205
Q

storage of platelets

A

gently agitated room temperature in an incubator at room temperature

NOT IN FRIDGE AS BECOME ACTIVATED and will be destroyed when transfused

206
Q

how long can plasma be kept

207
Q

where is plasma stored

A

freezer so need to thaw for 20-40 mins before use

208
Q

how quickly does plasma have to be transfused

A

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
Q

platelet triggers
prophylaxis
surger
critical sites

A
  • prophylaxis= 10
  • surgery need platelets >50
  • critical sites eg brain need to keep it >100
210
Q

what needs to be matched for RBC transfusion

A

ABO group
and
crossmatch needs blood without antigens that patient has antibodies too

211
Q

what needs to be matched for platelets

A

just Rhesus negative

but better if ABO compatible

212
Q

indication for platelets

A

to treat or prevent bleeding in patients with a low platelet count

213
Q

indication for FFP

A

-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
Q

comaptibility needed for FFP

A
  • same ABO group where possible
  • High titre anti a- and b negative
  • no need to match for rhesus
  • Group AB is universal donor
215
Q

types of FFP

A

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
Q

reversal of warfarin blood product

A

prothrombin complex- beriplex

217
Q

IV immunoglobulin indication

A

made from plasma and is used to replace immunoglobulins in immunodeficiency

218
Q

other blood products made from fractionation-

not made from UK people risk of CJD variants

A

anti D immunoglobulin
albumin
prothrombin complex concentrate
IV immunoglobulin

219
Q

risk of giving RBC transfusion to a patient with severe B12 deficiency

A

can cause heart failure

220
Q

requestation procedure for blood transfusion 7

A
  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
Q

points to consider in decision to transfuse

A
  • risk factors for transfusion
  • hx of transfusion
  • special requirements eg irradiated
222
Q

what type of consent is needed for tranfusion

A

just informed

DONT NEED SIGNED CONSENT

223
Q

requesting procedure for a blood transfusion needs

A 
-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
Q

pre-transfusion testing what is done

A
  1. ABO and rhesus group idetinfied

2. antibody screen detect red cell antibody

225
Q

blood component specifics for neonates

A

-need to be CMV negative

226
Q

who needs irradiated blood

A

intrauterine transfusions
neonatal exchange transfusions
top up transfusion after IUT
proven or suspected immunodeficiency

227
Q

taking a blood sample

A
  1. check identification

2. label blood sample at bed side hand written

228
Q

collecting blood

A
  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
Q

pre-authorisation of blood procedure

A
  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
  4. ensure baselin obs done <60 mins before blood component administered
230
Q

APTT increased
PT normal
bleeding time normal
is

A

haemophilia

231
Q

APTT increased
PT normal
bleeding time increased
is

232
Q

APTT increased
PT increased
bleeding time normal

233
Q

PT increased
APTT normal
bleeding normal
platelets normal

A

warfarin administration

234
Q

normal PT
normal APTT
increased bleeding time
normal platelets

235
Q

normal PT
increased APTT
normal bleed time
normal platelets

236
Q

increased PT
increased APTT
increased bleed time
decreased platelets

237
Q

heparin affects which factors

A

2,9, 10, 11

238
Q

warfarin affects which factors

A

2,7, 9, 10

239
Q

DIC factors

A

1,2, 5,8, 11

240
Q

liver factors

A

1 2 5 7 9 10 11

241
Q

what is a coagulation screen

A

addresses some aspects of coagulation
APTT
pt
fibrinogen concentration

242
Q

what is prothrombin time

A

time taken for the sample of blood to clot after tissue factor and calcium are added
-extrinsic pathway

243
Q

what is PT prolonged in

A

liver disease
DIC
warfarin/ vit K
deficiency 2, 5,7 10 or fibrinogen

244
Q

what is APTT

A

time taken for the sample of blood to clot after a contact activator and calcium are added
intrinsic

245
Q

what is APTT prolonged in

A 
DIC
haemophilia
vWF
heparin 
lupus and antiphos
liver disease
2,5,10, 8,9,11,12 or fibrinogen
246
Q

fibrinogen is raised in

A

acute phase response

247
Q

fibrinogen is low in

A

severe sepsis
DIC
rare congenital states
liver disease

inherited -afibro, dysfibro

neonatal fibrinogen is different so can give a false low result

248
Q

if APTT is raised in isolation what needs to be checked

A

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
Q

if APTT correction doesn’t correct it suggest

A

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
Q

what causes anti- fviii inhibitors 2

A

-exposure to exogenous factor when treating haemophilia A
or
-in patients with acquired haemophilia eg secondary to malignancy or rheumatological disorders

251
Q

what causes anti-phospholipid or lupus anticoagulant antibodies

A
  • mostly children with febrile illness

- uncommonly as part of SLE

252
Q

next inx after

PROLONGED APTT that is corrected

A

-consider intrinsic factor testing

vWF screen

253
Q

what does vWF do

A

-carrier protein for FVIII

if anything wrong with VWF it impacts the factor 8 levels

254
Q

APTT normal
PT normal
positive bleeding hx

A

probably factor 13
because outside of pathways
helps to make clots tight
so if deficient clots break up easily

255
Q

prolonged APTT and PT causes

A 
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
Q

how does neonatal coagulation differ

A

-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
Q

vit K dependent factors

258
Q 
13 yr old girl 
menorrhagia
nosebleeds
easy bruising 
prolonged APTT
normal PT
likely dx
A

von willebrand factor

259
Q 
36yr old man post-tonsillectomy bleeds
APTT prolonged and corrected
normal PT
vWF normal 
likely dx
A

mild factor 8 haemophilia A undiagnosed

260
Q 
64 yr old man 
pre-op coagulation 
APTT prolonged and corrected
Normal PT
vwf normal
fXII deficiency
A

factor XII deficiency

non clinical risk

261
Q

child with rash
raised WCC
raised APTT corrected
raised PT

262
Q

short APTT cause

A

due to acute phase response and high factor 8

263
Q

thrombin time prolonged by

A

heparin
decreased fibrinogen
increased D-dimer

264
Q

neutrophilia causes

A

infection and inflammation
drugs: steroids, GCSF
CML

265
Q

neutropaenia causes

A

infection
drugs- cytotoxic, idiosyncratic
marrow failure

266
Q

thrombocytosis causes

A

infection and inflammation
iron deficiency
myeloproliferative disorders

267
Q

thrombocytopaenia causes

A 
-drugs- cytotoxic, quinine
bleeding and DIC
immune- ITP
microangiopathy
marrow failure
268
Q

lymphocytosis causes

A

viral infection
inflammation
lymphoid malignancy - CLL

269
Q

lymphocytopaenia causes

A

viral infection
drugs- cytotoxic
ageing
marrow failure

270
Q

eosinophils are

271
Q

erythrocytes are

272
Q

thrombocytosis definition

A

platelet count >2SD above population mean
>400
common incidental finding >40

273
Q

reactive thrombocytosis is

A

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
Q

clonal thrombocytosis is

A

underlying myeloproliferative disorder or myelodysplastic neoplasm

275
Q

causes of reactive thrombocytosis

A 
-iron deficiency and blood loss 
infection
rheum
inflammation
surgery
malignancy
hyposplenism
276
Q

symptoms of thrombocytosis

A

mostly asymptomatic

but could have thrombotic or bleeding complications

277
Q

initial inx and further INX for thrombocytosis

A

-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
Q

management thrombocytosis

A

blood thinner

279
Q

thrombocytopaenia symptoms

A

bleeding or bruises
epistaxis
menorrhagia
tiredness

280
Q

drugs that cause thrombocytopaenia

A

H2 blockers
paroxetine
furosemide
metronidazole

281
Q

causes of thrombocytopaenia

A

-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
Q

thrombocytopaenia with bleeding management

A

urgent referral

283
Q

thrombocytopaenia of <30

A

urgent referral

284
Q

30-50 platelets

A

semi urgent referral

285
Q

> 50-100 and impending surgery or on antiplatelets/ coagulants

A

semi-urgent referral

286
Q

> 50-100 and no impending surgeery

A

routine referral

287
Q

> 100 platelets

288
Q

neutropaenia definition

289
Q

causes of neutropaenia

A

-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
Q

causes of agranulocytosis

A 
cytotoxics 
-antibiotics eg nitrof, erythromycin
anticonvulsants
phenytoin
NSAID
gold
INFLIXIMAB
carbimazole and prophylthiouracil
spironalactone
beta blocker
closapine, olanzapine
omeprazole
allopurinol
291
Q

management agranulocytosis

A

stop drug

Granulocytic stimulating factor

292
Q

inx for neutropaenia

A 
persistently <1
blood film
chronic viral serology
monospot for EBV
haematinics B12 AND Folate
ANA and RF- SLE
antineutrophil antibodies- primary immune neutropaenia
293
Q

refer neutropaenia if

A

suspiscion of progressive or serious disease
-with anaemia or thrombocytopaenia

more severe neutropaenia

persistently unexplained <1

294
Q

polcytheamia

A

abnormal accumulation of RBC

295
Q

haematocrit is

A

proportion of RBC

296
Q

haemoglobin is

297
Q

relative polcythaemia causes

A

dehydration
diuretics
realtive amount as plasma is reduced but RBC is same so proportion is higher

298
Q

true polycythaemia causes

A

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
Q

haematopoiesis is the

A

production of blood cells

300
Q

what controls neutrophils production

A

GCSF granulocyte colony stimulating factor

301
Q

how is blood cells made

A

from stem cells that divide into blast cells which are primitive and then undergo maturation

302
Q

lymphocyte formation

A

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
Q

where are blood cells broken down

A

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
Q

red cell life span

305
Q

platelet life span

306
Q

neutrophils

307
Q

processes to halt bleeding

A

vasoconstriction
platelets
coagulation cascade

308
Q

disorders of primary haemostasis- platelets

A
  1. collagen -ehlers danlos
  2. platelet function bernard soulier disease
  3. platelet number: thrombocytopaenia
  4. vWF
309
Q

pattern of bleeding for primary haemostasis

A 
-easy bruising
prolonged bleeding from cuts
menorrhagia
epistaxis
bleeding after trauma
310
Q

disorders of secondary haemostasis

A
  1. haemophilia
  2. warfarin and heparin
  3. liver disease
  4. DIC
311
Q

pattern of bleeding in secondary haemostasis

A

bleeding is prolonged and can be delayed

bleeding into deep sites eg muscles and joints

312
Q

acquired coagulation disorders

A 
more common than inherited 
-liver disease
-bone marrow failure
-ITP
-drugs: warfarin, heparin, aspirin
-DIC 
metabolic: uraemia renal failure 
vit c scurvy
313
Q

ITP cause

A

immune thrombocytopaenia

caused by antiplatelet autoantibodies

314
Q

presentation of ITP 2

A

acute
usually in children ,2 weeks after an infection
sudden self-limiting purpura

or chronic mostly in women

315
Q

presentation of chronic ITP

A

-fluctuating course of bleeding, purpura, epistaxis and menorrhagia
no splenomegaly

316
Q

inx findings for ITP

A

-increased megakaryocytes in marrow

antiplatelet antibodies

317
Q

treatment of ITP

A

if symptomatic or platelets <20
-prednisolone
-aim to keep >30
none if mild

318
Q

liver disease factors

A

2,7 9 10 11
vit K dependent

also makes protein c and s and antithrombin

319
Q

liver disease and 2 types of haemostasis problems with regards to bleeding

A

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
Q

presentation of liver disease bleeding problem

A

-can get both a bleeding or thrombosis picture

321
Q

management of liver disease bleeding

A
  • give FFP
  • cryoprecipitate if low fibrinogen
  • platelets if low
322
Q

why can liver patients get thrombosis

A

-high vWF
-low ADAMTS13
high factor VIII
low plasminogen
high PAI

323
Q

DIC pathology

A
  • 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
Q

DIC causes

A

-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
Q

clinical manifestations of DIC

A
  • underlying cause
  • petechial rash- non blanching
  • bleeding
  • microvascular thrombosis
  • organ failure
326
Q

lab features of DIC INX

A 
prolonged APTT, PT, Bleeding time
low fibrinogen 
low platelet count 
increased fibrin degradation products eg D dimer
D dimer will be massively elevated
327
Q

Rx approaches to DIC

A
  • treat underlying cause
  • platelet transfusion
  • fresh frozen plasma and cryoprecipitate
  • recombinant activated protein C
  • rFVIIIa
  • role of heparin controversial and unproven
328
Q

function of vWF 2

A
  1. ACTS as a carrier protein for factor viii

2. it binds to exposed collagen and is crucial for platelet adherence and activation

329
Q

coagulation what defines

A

coagulation precisely refers to the mechanism leading to the conversion of soluble fibrinogen to insoluble fibrin

330
Q

function of antithrombin, protein c and s

A
  • group of physiological anticoagulant substances that naturally inhibit the coagulation system
  • stop all the circulating fibrinogen clotting at once
331
Q

what breaks down the fibrin clot

332
Q

what prolongs thrombin time

A 
process of turning fibrinogen to fibrin 
-low fibrinogen
abnormal fibrinogen
heparin
high level of FDP
333
Q

Hereditary haemorrhagic telangiectasia pathology

A

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
Q

presentation of HHT

A
  • 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
Q

management HHT

A

-can be difficult due to multiple bleeds

regular iron therapy

336
Q

haemophilia a is due to

A

flip tip inversion in factor 8 gene on X chromosome

337
Q

haemophilia b

A

mutation defeciency in factor 9

338
Q

most common severe inherited bleeding disorder

A

haemophilis

339
Q

pathology of haemophilia

A

x-linked recessive

340
Q

presentation of haemophilia

A 
depends on severity
often in early life
often after surgery or trauma
bleeds into joint and muscles
leads to crippling arthropathy and haematomas
341
Q

diagnosis of haemophilia

A

-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
Q

coagulation results for haemophilia

A 
prolonged APTT
normal PT
normal bleeding time
normal fibrinogen
decreased factor 8 or 9
343
Q

severe haemophilia level and complications

A

-<1% factor or <0.01
recurrent spontaneous joint bleeds
chronic joint damage
spontaneous intracranial bleeds-rarer

344
Q

moderate haemophilia level and complications

A

1-5% 0.01-0.05
bleeding with minor trauma
subcutaneous, intramuscular

345
Q

mild haemophilia level and complications

A

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
Q

normal haemophilia level

A

50-150%

normal range

347
Q

management of haemophilia

A

1.recombinant synthetic factor 8 or 9
given on demand for bleeding episodes
targeted prophylaxis prior to surgery or dental work

  1. Fc fusion and pegylation-extends half life f8
  2. antifibrinolytics
  3. DDAVP in mild disease vasopressin
  4. physio for joints
  5. emicuzimab to activate factor 10- so brings together factor 9a and 10 which f8 usually does so can make a thrombus
  6. avoid NSAID and IM injection
348
Q

who gets primary prophylaxis for haemophilia

A

severe haemophilia

to try and turn them into moderate phenotype

349
Q

risk of haemophilia treatment

A

inhibitor formation
-anti factor 8 antibodies
increased bleeding unresponsive to factor 8 therapy
needs bypassing agents and immune tolerance induction

350
Q

complications of haemophilia

A

-spontaneous bleeding in severe patients- joint bleeds
-increased bleeding with trauma
surgical, iatrogenic bleeding

351
Q

haemophilia B management

A
  • recombinant factor 9
  • plasma derived factor 9
  • do not automatically give tranexamic acid
352
Q

von willebrand disease pathology

A 
low vWF
-defective platelet adhesion
-vWF protects f8 from degradation 
autosomal dominant mostly 
variable phenotype usually mild
353
Q

presentation of vWF

A 
primary bleeding disorder
-mucosal bleeding
easy bruising 
dental bleeding
epistaxis
menorrhagia
surgical and post-trauma bleeding 
v.rare to get haemarthroses
354
Q

vWF inx findings

A

increased APTT
normal PT
increased bleeding time
normal platelets

low vWF and factor 8

355
Q

management vWF

A
  • give desmopressin to increase vWF levels
  • tranexamic acid for mucosal bleeding
  • haemostasis with selected factor 8 concentrates if more severe
356
Q

types of vwf

A

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
Q

thrombosis virwchow triad

A

endothelial injury
stasis
hypercoaguability

358
Q

risk factors for thrombosis

A 
acquired risk factors
surgery
active malignancy
pregnancy puerpuerium
oestrogen medication
antiphos antibodies
prolonged immobility 
smoking 
obesity

inherited risk factors
inherited thrombophilia
fhx

359
Q

classify VTE

A

provoked
eg pregnancy, oestrogen, trauma, surgery <3months, bed rest, travel >6hrs, active malignancy

unprovoked
all others

360
Q

inx for VTE

A

-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
Q

what elevates D dimer

A

pregnnacy
infection
inflammation
malignancy

362
Q

DVT symptoms

A

calf swelling
localised pain
asymmetric oedema
prominent superfiical veins

363
Q

management of DVT 1st line

A

first line= doac

364
Q

management DVT with heparin induced thrombocytopaenia

A

fondaparinux

365
Q

management of DVT with renal impairment

A

unfractionated heparin

366
Q

management of DVT in pregnancy

367
Q

management of DVT and obese

368
Q

management of DVT and risk of bleeding

369
Q

management of DVT and active cancer

A

given LMWH for heparin

370
Q

management of DVT with active bleeding

A

IVC filters

371
Q

management of DVT and breast feeding

372
Q

management after dvt

A

-unprovoked= warfarin for 6 months to life long
-provoked- warfarin for 3 months
unless active malignancy give LMWH

373
Q

Unfractionated heparin

A 
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
Q

reversal agent for UFH

375
Q

LMWH

A 
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
Q

LMWH reversal

A

protamine reverses 60% of LMWH

377
Q

side effects heparin

A

bleeding
HIT
osteoporosis

378
Q

management of bleeding unfractionated heparin

A

stop infusion

protamine sulphate

379
Q

management of LMWH bleeding

A

withold next dose

protamine sulphate

380
Q

Heparin induced thrombocytopaenia

A

is an immune reaction- forms antibodies against complexes of platelet factor 4 in heparin
develops 5-10 days post-treatment
prothrombotic condition

381
Q

management of HIT

A

stop non -heparin

use fondaparinux

382
Q

warfarin is

A

an oral anticoagulant
vitamin K antagonist 2,7,10, 9
half life days
need to give with heparin initially

383
Q

monitoring warfarin

A

use INR
high INR more likely to bleed
low INR more likely to clot

use prothrombin time

384
Q

interactions of warfarin

A

metabolised by the CYP450 system
so enzyme inhibitors incerase INR
-omeprazole erythromycin, cranberry juice

reduce INR
-rifampicin, anti-epileptics

385
Q

complications of warfarin

A 
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
Q

warfarin INR 4.5-6.9 and low risk

A

reduce warfarin or withold dose

387
Q

warfarin inr 4.5-6.9 high risk

A

give vitamin K
withold warfarin
check INR at 24 hrs

388
Q

warfarin INR >7

A

vit K
withold warfarin
check INR at 24hrs

389
Q

warfarin and bleeding minor

A

vitamin K
withold warfarin
chec INR

390
Q

warfarin and major bleeding

A

vit K and beriplex or prothrombin complex concentrate
withold warfarin
immediate check PT and APTT
consider other factors contributing

391
Q

warfarin and pregnancy effects

A

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
Q

warfarin interaction with other drugs

A 
antibiotics
NSAID
amiodarone
antiepilepetics
azole
statins
H2 antagonists and PPI 
-sick day rules
alcohol
393
Q

DOAC 2 types

A

factor xa inhibitors - eg apixaban, rivaroxaban

direct thrombin inhibitors = dabigatran

394
Q

DOAC pros

A

no monitoring
no direct interactions
lower risk of bleeding

395
Q

disadvantages of DOAC

A

cant use in renal impairment
not for metallic heart valves
still developing reversal
not for anti phos patients

396
Q

DOAC reversal

A

currently in development

idarucizumab

397
Q

fondaparinux

A

glyoprotein
anti thrombotic
SC given

used for heparin induced thrombocytopaenia

cant monitor and not reversible

398
Q

argatroban

A

direct thrombin inhibitor
IV
heparin induced thrombocytopaenia
IV infusion difficult

not reversible

399
Q

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

A

sickle cell

400
Q

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.

A

sickle cell

401
Q

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.

A

hereditary spherocytosis

402
Q

anaemia michroc causes

A

iron deficiency anaemia

haemoglobinopathies

403
Q

normoc anaemia causes

A 
chronic disease anaemia
bone marrow failure 
haemodilution
sickle cell
haemolysis
EPO
404
Q

Macrocytic anaemia

A

haematinics deficiency

haemolysis

405
Q

iron deficiency anaemia

A

low ferritin
high transferrin
low iron
high TIBC

406
Q

chronic disease anaemia

A

high ferritin
low transferrin
low iron
low TIBC

407
Q

ferritin

A

stores iron
in tissue and macrophage
can rise in acute phase response

408
Q

serum iron

A

fluctuates with inflammation

409
Q

transferrin

A

transports iron
raised in iron deficiency as body tries to increase movement
low in chronic disease

410
Q

features of anaemia

A 
kolionychia 
fatigue dyspnoea pallor
faint palpitation
headache tinnitus
flow murmurs
cardiac enlargement
411
Q

iron deficiency anaemia

A

cause
-blood loss, diet, malabsorption, menstruation, GI

inx
low iron
low ferritin
high transferrin

412
Q

management of IDA

A

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

  1. ferrous fumarate
  2. ferrous gumarate
  3. IV iron
413
Q

steps of inx of IDA

A
  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
Q

chronic disease normocytic anaemia

A

iron gets stuck in stores
new red cells are hard to make
low transferrin
over time MCV starts to fall

415
Q

causes chronic disease and anaemia

A

inflammation
infection
cancer
anaemia or renal disease

416
Q

dx of chronic disease anaemia

A

inflamamtory markers
low transferrin
ferritin normal or raised in acute phase

417
Q

management chronic disease anaemia

A

dont prescribe iron (unless also low ferritin)

treat the cause

418
Q

bone marrow failure

A

normocytic anaemia

419
Q

megaloblastic meaning

A

hyersegmented neurophils

seen with haematinic and haemolysis

420
Q

causes of macrocytic anaemia

A

haematinic deficiency
haemolysis

normoblastic
alcohol
liver disease
hypothyroid
drugs
421
Q

inx macrocyric anaemia

A

b12 and folate
blood film
lft tft
bone marrow

422
Q

low folate if

A 
poor diet
alcohol
elderly
malabsorb
drugs eg antifolate
423
Q

causes low folate

A
  1. diet deficiency
  2. malabsorption eg coeliac disease
  3. increased requirement pregnancy
  4. anti folate drugs
424
Q

causes low vit b12

A
  1. pernicious anaemia
  2. distal malabsorption eg crohn’s
  3. gastrectomy
  4. diet
425
Q

b12 absorption

A

needs intrinsic factor

426
Q

features of haematinic deficiency

A 
glossitis
anaemia 
neuropsychiatric- irritable
paraesthesia
peripheral neuropathy
427
Q

risk of low b12

A 
subacute combined degeneration of spinal cord
-both LMN and UMn signs
extensor plantar
absent knee jerks
absent ankle jerks
428
Q

causes of low b12

A
  1. pernicious anaemia

2. ileal disease or absorption states eg crohn’s

429
Q

pernicious anaemia is

A

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
Q

inx for pernicious anaemia

A 
anaemic
macrocytosis
low b12
decreased reticulocytes
IF antibodies
431
Q

management b12 deficiency

A

hydroxycobalamin injection

432
Q

assoc, to pernicious anaemia

A

female
blue eyes
group a
autoimmune eg thyroid, vitiligo

433
Q

management haematinic deficiency

A

B12 must be replaced first
never give folate without checking b12
risk of subacute combined cord

434
Q

haemolysis

A

is shortened red cell survival

normoctyci or macrocytic

435
Q

causes haemolysis

A 
sickle cell
thalassaemia
G6PD
hereditary spherocytosis
autoimmune haemolysis
DIC
eclampsia
malaria
436
Q

CF haemolysis

A

jaundice
dark urine
anaemia
splenomegaly

437
Q

autoimmune haemolysis anaemia

A

mediated by autoantibodies

cold and warm

438
Q

warm AHA

A

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
Q

cold AHA

A

usually IgM, best haemolysis at 4 degrees
intravascular
raynauds and acrocyanosis

causes
-neoplasia, lymphoma, ebv
responds less well to steroids

440
Q

AHA findings

A

increased bilirubin
increased reticulocytes
increased LDH
decreased haptoglobin

441
Q

aplastic anaemia

A

body stop producing enough new blood cells
normocytic anaemia
leucopaenia
thrombocytopaenia

causes
idiopathic
congenital
drugs eg phenytoin
infection- parvovirus, hepatitis
sickle cell crisis
442
Q

G6PD presentation

A 
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
Q

inx G6PD

A

heinz bodies
bite and blister cells on blood film
enzyme assay

444
Q

drugs and g6pd

A

sulpha containing drugs
ciprofloxacin
antimalarials

445
Q

hereditary spherocytosis

A

autosomal dominant
less deformable spherical RBC so trapped in spleen
extravascular haemolysis

signs
-neonatal jaundice
chronic symptoms and crisis
splenomegaly

WHITE ANSCESTRY

446
Q

inx for hereditary spherocytosis

A

osmotic fragility

447
Q

inx for haemolysis

A 
macrocytic or normocytic anaemia
reticylocytes increased
bilirubinaemia unconjugated
LDH increased
decreased haptoglobin 
liver enzymes

coomb’s test direct for presence of antibodies- autoimmune

448
Q

inx haemoglobinopathies

A

fhx
fbc
blood film (target cells and howell jolly)
HPLC electrophoresis

449
Q

normal haemoglobin types

A

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
Q

foetal haemoglobin

A

is mostly HbF 2 alpha and 2 gamm

then switchese to HbA at 2-3 months of life

451
Q

thalassaemia are

A

Group of genetic disorders with decreased production rates of either alpha or beta chains

autosomal recessive

452
Q

inx thalassaemia

A

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
Q

management of thalsaemia

A

asymptomatic no treatment required

avoid unnecessary iron supplemenetation

454
Q

types beta t

A

beta thalassaemia
major
minor/ trait
intermedia

455
Q

beta thal major

A

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
Q

management beta t major

A

chronic transfusion- life long transfusions
risk alloantibody formation
transfusions result in iron overload so need chelation
bone marrow transplant

457
Q

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).

A

beta thalassaemia major

458
Q

sickle cell genes

A 
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
Q

pathology sickle cell

A

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
Q

sickling (deoxygenated haemoglobin polymerisation and red cell sickling) is increased by

A 
hypoxia
infection
acidosis
cold temp
low levels of HbF 
(usually undetectable in adults, but higher levels are protective)
461
Q

Clinical features of sickle cell

A

chronic haemolytic anaemia
jaundice
increased bilirubin
increased reticulocytes

462
Q

inx sickle cell

A

guthrie test

haemoglobin electrophoresis definitive dx

463
Q

management sickle cell

A
  • vaccinations- pneumoccoccal
  • crises prevention and support
  • intermittent transfusion to reduce HuS
  • chelation
  • hydroxycarbamide- increases HbF
  • bone marrow transplant- curative
464
Q

admission for sickle cell for

A

admit all in sickle cell crisis

unless well adult or child with mild pain and normal temperature

465
Q

sickle cell complications

A 
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
Q

types of anaemia in sickle cell

A
  • splenic sequestration- RBC get trapped in spleen so spleen enlarges- increased reticulocytes
  • aplastic crisis- parvovirus decreased reticulocytes
  • haemolytic crisis
467
Q

vaso-occlusive crisis

A

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
Q

acute end organ damage sickle cell

A

mostly affects the lungs, brain, penis
chest crises
strokes
priaprism- impotence and infertility (alpha agonist)

469
Q

acute chest syndrome sickle cell

A

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
Q

chronic complications sickle cell

A

chronic anaemia
chronic end organ damage- lung, kidney, heart spleen

transfusion related
iron overload
alloantibody formation

471
Q

chronic end organ damage in sickle cell

A

spleen atrophies over first few yrs of life
renal failure
pulmonary hypertension
cerevrobascualr disease

472
Q

general sickle cell problems

A 
splenic infarction
infection due to hyposplenism
poor growth
chronic renal failure
gallstones
iron overload
retinal damage
lung damage- hypoxia- fibrosis
473
Q

management of painful vaso-occlusive crisis

A 
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
Q

indication for transfusion in sickle cell crisis

A

severe chest crisis
suspected CNS event
multiorgan failure

475
Q

organisms that affect immunocompromised

A 
psuedomonas
stenotrophonomas maltophilia
pneumocysitis jiroveci
candidaemia
line assoc.
476
Q

psuedomonas aeruginosa is

A 
gram negative bacilli
aerobic
green blue colour
cut grass odour
oxidase positive gold 
found in soil and moist environment 
colonises human moist sites
477
Q

diseases pseudomonas

A 
catheter UTI
HAP
VAP
line infection and secondary bacteraemia
surgical site infection
478
Q

rx pseudonomas

A 
antipseudomonal
fluoroquinolones eg ciprofloxacin 
piperacillin
gent
meropenem
479
Q

stenotrophomonas maltophilia

A

aerobic gram negative bacilli
pale yellow on culture
ammonia odour
oxidase negative

480
Q

risks stenotrophonomas

A

-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
Q

rx stenotrophonomas

A

trimethoprim co-trimox
levofloxacin
minocycline

482
Q

pneumocystis jiroveci affects

A

-HIV CD4 <200 or AIDS

immunocompromised with malignancies, bone marrow transplant

483
Q

cf pneumocystis

A 
progressive dyspnoea
fever
cough
pneumonia 
tachycardia
tachypnoea
484
Q

inx pneumocystis

A

CXR= bilateral diffuse infiltrates

high resolution CT HRCT ground glass changes

485
Q

diagnosis pneumocystis

A

induced sputum or bronchoscopic alveolar lavage and
immunofluorescent or PCR

cant culture

hypoxaemia

486
Q

management pneumocystis

A

co-trimoxazole plus prednisolone

21 days HIV
14-21 days

487
Q

candida

A

yeast infection
skin gi tract, gu tract

part of normal flora but when candidaemia in blood stream its significant

488
Q

inx candidaemia

A

blood cultures

from both lines and periphery

489
Q

risk factors candidaemia

A 
exposure to broad spec abx
immunocompromised
neutropaenia
indwelling IV catheters
TPN
GI or thoracic surgery 
solid organ transplant
490
Q

management candidaemia

A

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
Q

antifungal for candida

A

triazoles
echinocandins eg caspofungin
polyenes eg amphotericin B

492
Q

vancomycin resistant enterococci

A

gram positive bacteria
spread in healthcare
broad spectrum of infection
difficult to distinguish between carriage and infection

493
Q

rf for VRE

A 
broad spec abx
IV lines
catheter
haem malignancy
increased age
dm
surgery
long patient
ICU
494
Q

treatment VRE

A

linezolid
daptomycin

from then on considered colonised and consider patient isolation

495
Q

line assoc. infections

A

get into blood stream

eg staph aureus and staph epidermidis are common

496
Q

staph aureus rx

A

flucox 2 weeks

unless MRSA

497
Q

staph epidermidis

A

more common immunocompromised
normal skin flora
causes biofilms that grown on devices

498
Q

peripheral venous cannula infections

A

local phlebitis to cellulitis
PVC insertion bundle management
review daily PVC maintenance

499
Q

management central line infection

A

culture from line
remove line
duration of abx depends on organism

500
Q

spleen functions

A

filtration
regulates inflammation
maturation of B and t cells
haemostasis

501
Q

types of asplenia/ hyposplenia

A

acquired- surgical or splenic infarction

hyposplenia 
sickle cell anaemia
GVHD
coeliac
HIV
alcoholic
502
Q

bacterial organisms assoc. to sepsis post-splenectomy

A

encapsulated bacteria
-strep pneumoniae
HiB
N. meningitidis

unusual gram negative bacteria
bordetella
salmonella

paraistes
babesia microti
plasmodium falciparum

anaplasma

503
Q

management of post-splenectomy

A

penicillin V life long
clarithromycin life long

vaccinations
pneumonooccal
meningococcal
influenza

vector avoidance

year 5 (9 decks)

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