Haematological emergencies Flashcards

(51 cards)

1
Q

Does neutropenic sepsis always present with febrile neutropenia?

A

No

Patient can have neutropenia without fever

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the 2 defining features of neutropenic sepsis?

A

Absolute neutrophil count is 0.5 x 10(9)/L or lower

Temperature greater than 38 degrees Celsius OR any symptoms/signs of sepsis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the main reason why neutropenic sepsis is a life-threatening emergency?

A

Neutropenic patients already have weakened immune system and are more likely to contract bacterial infections which can become disseminated and fatal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Give 4 haematological risk factors for neutropenic sepsis?

A

Recent chemotherapy (usually within 14 days): Biggest risk factor

Immunotherapy with cytotoxic drugs

Stem cell transplant patient

Bone marrow disorders

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What treatment pathway is used for neutropenic sepsis?

A

Sepsis 6 care bundle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

How does a patient with neutropenic sepsis start the sepsis 6 care bundle treatment?

A

Call 999 ambulance for emergency transfer to hospital, ideally within 1 hour of neutropenic sepsis recognition

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is included in the sepsis 6 care bundle, to treat neutropenic sepsis?

A

3 diagnostic and 3 therapeutic steps delivered within 1 hour after initial recognition of sepsis

  1. Oxygen administration
  2. Two sets of blood cultures
  3. Venous blood lactate
  4. Fluid resuscitation
  5. Appropriate antibiotics
  6. Monitor observations
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How should oxygen be administered to treat neutropenic sepsis?

A

Give oxygen therapy to people with reduced oxygen saturation (below 92%) or with an increase in oxygen requirement over baseline, to maintain oxygen saturation above 94% unless contraindicated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How are the two blood cultures taken from the patient, in neutropenic sepsis treatment?

A

Paired cultures from a peripheral/central line in situ and periphery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the first line antibiotic for neutropenic sepsis and why?

A

Continuous IV piperacillin with tazobactam (tazocin)

Provides anti-pseudomonal cover

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What hormone drug can you administer to patients with neutropenic sepsis and why?

A

Recombinant G-CSF eg. Filgastrim

Can be used as treatment and prophylaxis to prevent neutropenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

If a patient with neutropenic sepsis is allergic to penicillin, which antibiotic should be administered instead of tazocin?

A

Second-line: IV Meropenem

Tazocin contains penicillin so cannot be used

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the 4 emergency sickle cell crises?

A

Vaso-occlusive crisis

Aplastic crisis

Sequestration

Acute chest syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is a vaso-occlusive crisis in SCD patients?

A

Sickled RBCs get stuck in microcirculation eg. capillaries and cause ischemic injury to organs and tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is an aplastic crisis in SCD patients?

A

Bone marrow suppression usually due to infection of parvovirus B19 which stops RBC production for a short time

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is acute splenic sequestration crisis in SCD patients?

A

Sickled RBCs block blood vessels exiting spleen, so spleen becomes engorged with blood

Causes severe anaemia and life-threatening circulatory collapse

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is acute chest syndrome in SCD patients?

A

Vaso-occlusive crisis in pulmonary vasculature

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the main symptom of sickle cell crises?

A

Sudden and intense pain throughout whole body

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the management for sickle cell crisis (HOP to it)?

A

H: Hemodilution (IVF, PRBCs)

O: Oxygen supplementation

P: Pain relief

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

In sickle cell crisis, how is hemodilution managed in 2 steps?

A

Exchange transfusion: Machine replaces sickled blood with donor PRBCs

Intravenous fluids: Slows sickling process

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

In sickle cell crisis, how is oxygen supplementation managed?

A

Provide oxygen if below 95% on room air

22
Q

In sickle cell crisis, how is pain relief managed?

A

Mild pain: Paracetamol/ibuprofen/naproxen

Moderate pain: Codeine, tramadol

Severe pain: Morphine, oxycodone

23
Q

What are the 3 defining signs of acute chest syndrome in SCD patients?

A

Fever

Respiratory symptoms

Pulmonary infiltrate (opacity) on chest x-ray

24
Q

What is TTP and why is it a haematological emergency?

A

Clots form in microcirculation which blocks blood flow to heart, kidney, brain and other organs

25
What is the classic pentad of TTP symptoms? (FAT RN)
Fever Anaemic symptoms due to MAHA Thrombocytopenia: Purpura or bleeding Renal failure: Thirst, frequent urination Neurological symptoms
26
Which 5 investigations should be immediately done to confirm TTP?
FBC: Marked thrombocytopenia, haemolytic anaemia markers eg. reticulocytosis, low LDH, low haptoglobins Coagulation screen: Normal Peripheral blood film: Schistocytes, polychromasia, thrombocytopenia ADAMTS13 level: Very low Urinalysis and renal function tests
27
What is the mainstay emergency treatment of TTP, whether it is congenital or immune-mediated?
Plasma exchange: Donor plasma with normal ADAMS13 levels replaces patient's plasma which contains autoantibodies and no ADAMS13
28
Which 3 medications can either be administered to treat TTP?
Steroids eg. methylprednisolone: Reduces antibody level and immune response to ADAMS13 Rituximab: Stops ADAMS13 from being broken down Caplacizumab: Stops platelets from sticking to von Willebrand factor
29
Which supportive medicines are administered to treat TTP?
Folic acid Omeprazole Dalteparin (heparin injection)
30
What is Tumour Lysis Syndrome (TLS)?
Tumour cell breakdown (usually caused by cancer treatment) release contents into bloodstream
31
Give 4 common risk factors for TLS?
High-grade cancers eg. Burkitt's lymphoma, acute leukaemias Recent chemotherapy Renal issues Dehydration Conditions causing hyperuricemia
32
What is the classic tetrad of lab findings for TLS? (LUCK)
L: LDH low U: Hyperuricemia C: Hypocalcemia K: Hyperkalemia
33
What are some symptoms of TLS?
Urinary symptoms eg. haematuria, dysuria Hypocalcemia: Cramps, vomiting, altered mental status, spasms Hyperkalemia: Weakness, paralysis Arrythmias, sudden death
34
How do you manage TLS?
Hyperuricemia: Rasburicase, if condraindicated give allopurinol Hyperphosphatemia: IV fluids and monitor high urine output, if uncontrolled use dialysis Hypocalcemia: Cardiac monitoring unless symptomatic, then give calcium gluconate Hyperkalemia: Cardiac monitoring unless severe, then haemodialysis
35
What is Acute Promyelocytic Leukaemia (APML)?
Rare subtype of AML characterised by over proliferation of promyelocytes
36
Why is APML a haematological emergency?
Causes coagulopathy which can lead to CNS and pulmonary haemorrhaging
37
Why does APML cause coagulopathy in 2 ways?
APML blasts express tissue factor and secrete IL-1, which activate clotting cascade Cancer Procoagulant in present in APML cells and activates factor 10 directly
38
Give 5 risk factors of APML?
Age: Risk increases as age increases (more common in middle age than children) Hispanic Obesity Occupational exposure eg. Chemical agents Previous chemotherapy or radiotherapy
39
Give common signs and symptoms of APML?
Petechiae Excessive mucocutaneous bleeding Frequent infections and fever Fatigue Weight loss and appetite loss Pain in joints and bones Pallor
40
Which 5 lab tests should be done to investigate APML?
FBC: Pancytopenia Coagulation screen: High APTT and high PT D-dimer test: High Blood film FISH genetic testing
41
What is the characteristic finding of APML on a blood film?
Large, bi-lobed, hypergranular cells (buttock cells) with multiple auer rods
42
When APML is suspected, what genetic testing must be done for rapid diagnosis?
FISH: PML-RARA fusion gene Translocation between chromosomes 15 and 17 produces PML-RARA fusion gene RARA codes for retinoid acid receptor alpha, which normally allows promyelocytes to differentiate: Fusion gene prevents this
43
What is the emergency management of APML?
All-trans retinoic acid (ATRA) Arsenic trioxide
44
What is DIC?
Disseminated intravascular coagulation Hypercoagulable state causes microvascular and macrovascular clotting, which then depletes platelets and causes bleeding
45
What are the causes of DIC?
STOP Making Trouble S: Sepsis/snakebites T: Trauma (acute traumatic coagulopathy) O: Obstetric complications P: Pancreatitis or liver disease M: Malignancy T: Transfusions
46
What are the signs and symptoms of DIC?
Petechiae, purpura, bruising Uncontrollable bleeding from many sites Hypotensive Confusion SOB
47
What lab tests confirm DIC?
FBC: Thrombocytopenia Coagulation screen: Prolonged PT, APTT, TT Low fibrinogen level Elevated D-dimer Blood film: Schistocytes
48
What is the general treatment pathway of DIC?
Treat acute symptoms eg. major bleeding Treat underlying cause
49
How do you treat active bleeding in DIC?
No anticoagulants Transfuse platelets and fibrinogen or FFP
50
How do you treat DIC with no major bleeds?
Low dose anticoagulant eg. LMWH Transfuse platelets, fibrinogen or FFP after assessing levels
51
How do you treat DIC with over thromboembolism?
Therapeutic LMWH