haem emergencies

Question 1

what is neutropenic sepsis

Answer 1

sepsis which occurs with low neutrophill counts
common in those with haem cancers and those un dergoing cancer treatments

Question 2

Neutropenic sepsis clinical presentation

Answer 2

• neutrophil count <1 x 109/L
• fever >38
• clinical features of sepsis

Question 3

Neutropenic sepsis risk factors

Answer 3

Anticancer treatment

Haematological malignancy - Particularly acute leukaemias, myelodysplasia, aplastic anaemia

Drugs - E.g clozapine, carbimazole, methotrexate

Question 4

when Neutropenic sepsis suspected what action do you take

Answer 4

IV BROAD SPECTRUM ANTIBIOTICS WITHIN 1 HOUR + sepsis 6
Do not wait for results in at risk patients!!

Culture EVERYTHING
- Peripheral blood and each line lumen
- Urine
- Viral and bacterial throat swabs
- Sputum if expectorating
- Stool if diarrhoea
- Chest X Ray

Question 5

sepsis 6

Answer 5

GIVE 3 TAKE 3
1. Oxygen to maintain sats >94%
2. IV access + stat IV antibiotic
3. IV fluids to support BP

4. Send bloods inc Hb, lactate
5. Send blood cultures
6. Monitor urine output – consider catheter

Question 6

spinal cord compression presenting factors

Answer 6

• Back pain – often severe
• Leg weakness – typically bilateral
• Reduced sensation in legs
• Change in bowel habit
  – diarrhoea or constipation
• Change in urinary habit
  – often retention
• Saddle anaesthesia (cauda equina)

Question 7

name common haematological and non-haematological causes of SCC?

Answer 7

haematological:
- lymphoma (any type but common non-hodgkins)
- myeloma

non-haematological:
- other malignancy
- severe degenerative spinal disease
- disk protrusion
- spinal haematoma

high suspision in those with cancer

Question 8

what action to take when SSC is first suspected?

Answer 8

• urgent MRI whole spine within 24hrs
• dexamethasone 8mg BD

Question 9

what to do once its confirmed?

Answer 9

• surgery
• radiotherapy

if haematological:
- bone marrow biopsy - myeloma
- lymph node biopsy - lymphoma

Question 10

Hypercalcaemia treatment

Answer 10

• Aggressive IV hydration 2-3L/day
• IV bisphosphonate if Ca > 3 or not responding to fluids

Question 11

most likely haemotological causes in hypercalcaemia

Answer 11

myelomas
lymphomas

Question 12

tumur lysis syndrome definition

Answer 12

This is a potentially fatal complication of chemotherapy for malignancy (most commonly seen in patient’s with lymphoma or leukaemia).
- rapid breakdown of malignant cells
- occur sponatenously or within first week of steroids or chemo

Question 13

blood resuts of TLS

Answer 13

Hyperkalaemia
Hyperphosphatemia
Hyperuricaemia
Hypocalcaemia
severe acute kidney injury

Question 14

how to prevent TLS

Answer 14

• risk assess all patients prior to starting treatment
• hydrate patients
• Allopurinol for most patients – blocks uric acid production
• Rasburicase if high risk
  Breaks down existing uric acid

Question 15

TLS diagnostic criteria

Answer 15

Cairo & Bishop diagnostic criteria
need to have:
- high serum creatine
- arrythmia
seizure

Question 16

TLS treatment

Answer 16

IV fluids ++++
Rasburicase
Correct high K (not low Ca)
Intensive care for dialysis if not improving

Question 17

thrombotic thrombocytopenic purpura cause

Answer 17

TTP is caused by ADAMTS13 deficiency
Immune – antibody against ADAMTS13
Congenital (<10%)

Question 18

TTP pathology

Answer 18

ADAMTS13
Protein which breaks down vWF into smaller multimers
Deficiency leads to build up of large vWF chains
Block vessel lumens
Platelets adhere and aggregate into clots
Red cells can’t pass through – broken down into fragments

Question 19

what does TTP lead to

Answer 19

Microangiopathic haemolytic anaemia (MAHA)
=intravascular breakdown of red cells

Consumption of platelets
Leads to thrombocytopenia
Build up of clots in blood vessels

Thrombosis -> small vessels – purpura, AKI, neuro symptoms

Question 20

TTP findings

Answer 20

• low platelets and either MAHA or another feature
• confusion
• large vessel thrombosis
• red cell fragments on blood film
• thrombocytopaenia

Question 21

testing TTP

Answer 21

• red cell fragments on blood film
• thrombocytopaenia
• ADAMTS13 level confirms diagnosis
• after this is confirmed test for antibody to see if its immune or congenital

Question 22

TTP immediate treatment

Answer 22

PLASMA EXCHANGE IS LIFE-SAVING AND NEEDS TO BE STARTED WITHIN 4-6 HOURS OF SUSPECTED DIAGNOSIS - dont wait for ADAMTS13 results

• patient will need ICU bed and central venous access ASAP

Question 23

TTP other treatments

Answer 23

Plasma exchange:
- Aims to remove the antibody causing TTP
- Aims to replace some ADAMTS13
- Continued daily until improving

Other treatments:
- Steroids – immunosuppression
- Rituximab – immunosuppression
- Caplacizumab – antibody to vWF so that platelets can’t adhere and form clots