Haeatological Emergencies Flashcards
List the haematological emergencies
Neutroepaenic sepsis
Spinal cord compression
Chest crises in SCD
TTP
Massive PE
Tranfusion Reaction
Major Haemorrhage
Causes of neutropaenia
Reactive - Sepsis, inflammation
Drugs - antispychotics, carbimazole
Liver failure
Ethnic - Duffy A and B neg –> largeer mariginated pool as more stick to Vascular endotheilium - but not a profound neutropaenia
Autoimmune
Bone marrow failure - aplastic anaemia, bone marrow failure
Only worried if below neutrophils below 1
How does carbimazole cause neutropaenia
causes agranulocytosis
desroys bone marrow precursors
LPS in cell wall
when destroyed by macrophages/complement/antibiotics its release triggers shock
Neutropaenic sepsis management
Broad spec penicillin within 1 hour
Aminoglycoside (gram +ve) or glycopeptide (gram-ve) in line with local policy
Don’t delay in post-chemo patients - Fever, blood cultures etc not required
Very urgent Abx needed
What occurs in chest crisis in SCD
sickling - due to cold, infection, hypoxia, drugs
occlude capillaries –> impairs gas exchange
worsens hypoxia –> worsen sickling
chest pain –> further hypoventilation –> further decreased O2 content
NO release –> VCAM –> worsens sickling
Management of ACS
High flow O2, CPAP, intubation, ECMO
* Mortality high once on ITU
Correct reversible causes - ABx, anticoagulation
Exchange transfusion
WHen should you suspect a chest crisis in an SCD patient
unexplained hypoxia, chest pain or reduced GCS
(PaO2 is a better indicator than pulse ox)
Always discuss with haematologist
What are the types of TTP
congenital - absence of ADAM-TS13
acquired - auto-antibody mediated decrease in ADAMTS-13
Presentation of TTP
**Pentad: **
Fever
Altered mental state
Thrombocytopenia - always present
Renal Impairement
Haemolytic anaemia
Acute onset
Only 10% present with all 5
What is the pathology behind TTP
ADAMTSs-13 normally cleaves vWF to make it the correct lenghth
if absent –> large vWF make platelets stick together and stick to RBC –> thrombocytopaenia and cleaving RBC
What is the most important test for TTP?
blood film
what does blood film with TTP look like
Note: MAHA also includes HUS, HELLP, TTP
Management of TTP
Immediate plasma exchange (if not available - transfuse FFP as this has ADAMTS-13)
Steroids, rituximab, aspirin
Congenital TTP - regular FFP trasnfusion (don’t need exchange as they don’t have antibody to destroy it)
Universally fatal without intervation
How is major haemorrage defined
Varied definitions - better to 2222 major haemorrhage than not
