Haematological emergencies

Question 1

List some causes of neutropenia

Answer 1

decreased production - ie bone marrow issue:

• acute leukaemia
• myelodysplastic syndrome
• marrow infiltration
• B12/folate deficiency
• medications eg methotrexate, carbimazole, clozapine

iatrogenic:
- chemotherapy

Question 2

What are the clinical features of neutropenic sepsis?

Answer 2

fever
rigors
malaise
less common: low BP, cardiovascular collapse and death

Question 3

When should you suspect neutropenic sepsis?

Answer 3

if someone has had chemo in the last 3 months 
presents with fever
chills/rigors
malaise
isolated hypotension

Question 4

What are the top three things you must do if you suspect neutropenic sepsis?

Answer 4

1. see the pt immediately
2. put a cannula in
3. give antibiotics in sheffield this is Tazocin and gentamycin

Question 5

What are the clinical features of a sickle cell crisis?

Answer 5

gradual onset over 2-3 days of pain
eventually the pain is unbearable and can be in their arms, hands, legs, back, ribs, chest, abdomen
pts often have a typical site of pain

Question 6

What is the cause of a sickle cell crisis?

Answer 6

can be precipitated by dehydration, infection, stress, alcohol, cold
the RBCs deform and become sickle shaped which can leads to vaso-occlusive events eg acute chest syndrome due to occlusion of the pulmonary vasculature, splenic sequestration and shock, aplastic crises and haemolytic crises, bone crises are due to bone infarcts due to vaso-occlusion in the bone vessels

Question 7

What are the worrying symptoms of sickle cell crises?

Answer 7

chest pain - as could be chest crises eg PE or ACS
severe abdominal pain
neurological symptoms as at high risk of stroke

Question 8

What are the three main points of management when it comes to sickle cell crises?

Answer 8

1. see the pt
2. control the pain eg paracetamol, ibuprofen or morphine
3. put a cannula in an d start fluids - to prevent dehydration and put them on O2 if they have low oxygen sats

Question 9

What is acute chest syndrome?

Answer 9

the pt has a vaso-occlusive crisis in their lungs, causing infarction of the lungs and so hypoxia, which then generates more sickles and these cause further lung infarction and the cycle starts again

Question 10

What are the clinical features of acute chest syndrome?

Answer 10

new signs on CXR
hypoxia
chest signs
eg may look like they have pneumonia but it is a chest crisis

Question 11

How would you manage a pt with sickle cell crisis?

Answer 11

1. see the pt
2. give the pt O2/antibiotics
   3) call a haematologist (as the pt needs a red cell exchange to remove the sickles)

Question 12

What haematological emergency can be encountered with multiple myeloma pts?

Answer 12

spinal cord compression

Question 13

What are the symptoms of spinal cord compression?

Answer 13

back pain 
neuropatic pain
leg weakness/numbness
saddle anaesthesia
loss of sphincter control/urinary retention
incontinence

Question 14

What are the signs of spinal cord compression?

Answer 14

decreased power in the legs
typically decreased reflexes 
decreased sensation 
PR exam shows decreased anal tone 
very large residual bladder volume >200ml

Question 15

What 3 points of management should you do if you suspect that sb has spinal cord compression?

Answer 15

1. keep them in bed
2. dexamethasone 8mg twice a day
3. urgent MRI

Question 16

How would you treat a confirmed SCC in a myeloma pt?

Answer 16

radiotherapy
chemotherapy

NB: in pts without myeloma you may do surgery

Question 17

Which immunoglobulin will cause hyperviscosity syndrome in smaller amounts and why?

Answer 17

IgM as it is a pentamer

Question 18

What are some causes of hyperviscosity syndrome?

Answer 18

myeloma
acute leukaemia with hyperleukocytosis
Waldesnström’s macroglobulinaemia
polycythaemia vera

Question 19

What are the symptoms of hyperviscosity syndrome?

Answer 19

haedaches
neurological
blurred vision
fatigue
mucosal bleeding
confusion/ altered mental state/ decreased glasgow coma score
SOB, hypoxia - could be due to pulmonary oedema

Question 20

What would be seen on examination of a pt with hyperviscosity syndrome?

Answer 20

bruising 
epistaxis 
pulmonary oedema 
evidence of fluid overload 
decreased glasgow coma score 
confusion
ataxia
nystagmus
papillodema
peripheral oedema

Question 21

What 3 steps of management would you do for a pt who has hyperviscosity syndrome?

Answer 21

1. put in a cannula
2. give them fluids
3. call the haematologist

Question 22

What is tumour lysis syndrome?

Answer 22

chemo kills cancer cells, causing them to release their intracellular components
gives an acute rise in plasma uric acid, potassium, phosphate and hypocalcaemia
this can cause acute renal failure due to crystallisation in the kidney

Question 23

Which type of cancers is tumour lysis syndrome most likely to occur with?

Answer 23

rapidly dividing tumours eg acute leukaemia and Burkitt’s lymphoma
also people with a large tumour burden - more cells available to die
some solid tumours eg neuroblastoma

Question 24

what drug can be given to control hyperuricaemia and how does it work?

Answer 24

rasburicase - oxidises uric acid to allantoin

Question 25

What are the clinical features of tumour lysis syndrome?

Answer 25

Hyperkalaemia (first)
Hyperphosphataemia
Hyperuricaemia
Hypocalcaemia

finding clinical features on examination means it’s too late:
renal failure
seizures
sudden death

Question 26

Describe the management of tumour lysis syndrome

Answer 26

prevention is easier than treatment
those at high risk are given:
- vigorous IV fluids throughout first course of chemo and several days later
- rasburicase - completely eliminates uric acid from the circulation
- very close monitoring of tumour lysis bloods - ie U+Es
- close monitoring of urine output

Question 27

How do you manage a pt who has developed tumour lysis syndrome?

Answer 27

FLUIDS
rasburicase 
close monitoring of urine output
treat high potassium but don't treat low calcium 
dialysis if needed