Haem - mx Flashcards
Indications for RBC transfusion
- Catastrophic haemorrhage - major trauma, associated hypotension
- No safer alternative available - massive bleeding + plain fluids not sufficient
- Anaemic (iron/folate/B12 not appropriate)
- Hb <70g/L + signs of compromise (tachycardia, low BP, narrow pulse pressure, cold peripheries, syncope,dyspnoea)if no signs of compromise try to address underlying issue
- Hb <80g/L + pt >65 y/o + signs of compromise or Hx of IHD/severe respiratory disease
Indications for platelet transfusion
- Active bleeding +
- Plt <50 x 10^9/L (Normal 150-400 x 10^9/L)
- DIC
- Bone marrow failure (plt <10 x 10^9/L)
- If low platelets before surgery
- If cardiac bypass surgery needed + patient is on anti-platelets
Indications for FFP transfusion
* Fibrinogen <1g/L or * INR >1.5 * Reversal of warfarin if pt is bleeding * Bleeding from excessive anti-coagulation * Bleeding + abnormal coagulation test results TTP Isolated factor deficiencies Antithrombin III deficiency Immunodeficiencies
NOT to just replace volume loss
NOT to reverse warfarin if pt is not bleeding
What does a cryoprecipitate transfusion include?
Very concentrated form of fibrinogen
Fibronectin F8, F13
VWF
Indications for cryoprecipitate transfusion
- Massive bleeding + low fibrinogen
- Hypofibrinogenemia / afibrinogenemia
- DIC
- Reversal of anti-coagulation (FFP preferred)
- Haemophilia - emergency back up when factor concentrate not available
What does a prothrombin complex concentrate transfusion include?
Factors 2, 7, 9, 10
Indications for prothrombin complex concentrate transfusion
- Reversal of warfarin
- Reversal of other vitamin K antagonist anti-coagulants
- Pt with deficiency of one of the included factors (2, 7, 9, 10) (congenital, liver disease, haemophilia)
- Significant bleeding in people with coagulopathy
- Rapid anti-coagulation reversal before surgery (better than FFP)
- Contra-indicated in pt with DIC
Sickle cell anaemia - acute painful crisis mx
Fluids, Oxygen, analgesia
Saturate (Oxygen)
Abx if needed
Pain relief
Cannula (IVF)
Sickle cell anaemia - stroke mx
Manage as a stroke
Give an exchange blood transfusion
Sickle cell anaemia - sequestration crisis/recurrent episodes of splenic sequestration mx
Splenectomy
Sickle cell anaemia - chronic cholecystitis mx
Cholecystectomy
Sickle cell anaemia mx
Conservative
Trigger avoidance
Medical
Prophylactic abx
Vaccinations - Haemophilus Influenzae type B, Streptococcus pneumoniae, Meningococcus, Hep B
o Oral penicillin prophylaxis started at dx and continued until 5 years of age
Regular blood transfusions - Common prophylactic treatment to maintain HbS below 30%
Hydroxyurea (increases BM ability to make HbF which doesnt have the problematic β gene)
Surgical
MB stem cell transplant (curative)
Risks of blood transfusion
Over-transfusion (hyperviscosity, volume overload)
Transfusion reactions (acute, septic, febrile, and allergic)
Alloimmunisation to red cell antigens
Iron overload
Transfusion-transmitted diseases (hepatitis B and C, HIV, and other agents).
Which transfusion is used in DIC?
Cryoprecipitate (rich in fibrinogen)
Sickle cell trait management
- Adequate hydration
- Avoidance of excessive fluid loss
- Avoidance of severe heat
- Recurrent splenic sequestration is an indication for splenectomy
Vasocclusive/sickle cell crisis management (7)
• Avoid cold, fever, dehydration, stress • Analgesia o Paracetamol used to treat mild pain o NSAIDs used to treat mild to moderate pain – used with caution in patients with mild hepatic/renal impairment o Codeine to treat moderate pain
• Supportive care + correction of cause
o Oxygen
o IVF
o Deep breathing exercises
• Abx if there is evidence of infection
• Blood transfusion – indicated for life-threatening vaso-occlusive events, symptomatic anaemia, acute organ dysfunction
• Hydroxycarbamide (hydroxyurea)
o Can reduce the frequency of painful crises in sickle cell disease
o Given if there are 6+ episodes of vaso-occlusive crisis per year
• Folic acid – in severe haemolysis or pregnancy
Chronic sickle cell disease management
• Supportive care – prevention of complications
o Routine vaccinations - Haemophilus Influenzae type B, Streptococcus pneumoniae, Meningococcus, Hep B
o Oral penicillin prophylaxis started at dx and continued until 5 years of age
• Hydroxycarbamide (hydroxyurea)
o Considered in patients aged >2 years with sickle cell anaemia
o Increase in HbF
o Decreases the frequency of sickle cell crises, reduces transfusion requirements, decreases risk of acute chest syndrome
• Repeated blood transfusions
o Common prophylactic treatment to maintain HbS below 30%
o Required for severe anaemia or to reduce the proportion of HbS if there are lung or CNS complications, severe symptoms refractory to treatment (haemodynamic instability due to hypovolaemic shock)
o Iron overload is a common complication – chelation should be started in all children receiving regular blood transfusions (SC deferoxamine, oral deferasirox, oral deferiprone)
• Bone marrow transplantation
o 2nd line, considered in children with severe complications of sickle cell anaemia (e.g. stroke, recurrent acute chest syndrome) who are unresponsive to first-line therapies
• Stroke
o Transcranial Doppler US performed annually in children aged 2-16 years
o Regular blood transfusions considered in those with abnormal findings on transcranial Doppler US
Acute chest syndrome as a result of vasocclusive crisis in sickle cell disease management
- Oxygen, incentive spirometry, continuous positive airways pressure
- Analgesia
- Hydration
- Abx (macrolide + IV cephalosporin)
- Transfusion/exchange blood transfusion
- Hydroxycarbamide
Macrolides = azithromycin, erythromycin, claritromycin Cephalosporins = cefuroxime, ceftriaxone, cefaclor
Priapism as a result of vasocclusive crisis in sickle cell disease management
- Emergency
- Hydration + analgesia
- Minor episodes – bladder emptying, exercise, warm baths, analgesia
- Prolonged episode – aspiration + irrigation of corpora cavernosa with adrenaline or etilefrine
What is an acute transfusion reaction + mx?
• Acute transfusion reaction
o Immune system attacking foreign platelets, white cells, serum proteins
o Febrile patient + urticarial skin rash (erythema, hives, itching)
o Confirm blood transfusion, stop temporarily, give paracetamol + anti-histamines, resume transfusion at a lower rate
What is anaphylaxis + mx?
• Anaphylaxis
o Due to IgA deficient patients – they have anti-IgA antibodies attacking the IgA in the transfused blood
o Breathless + hypotensive patient
o Adrenaline (IM), chlorphenamine, corticosteroids, fluids
What is a haemolytic transfusion reaction + mx?
• Haemolytic transfusion reaction
o Mismatch of ABO alleles- immune destruction of transfused RBC attacked by the recipient’s antibodies
o Cold, feverish, nauseated, chest/flank pain, dark red urine from haemolysis
o Stop the blood transfusion, anticipate shock + DIC, use IV fluids + IV mannitol to flush out the products of haemolysis
What is a transfusion associated lung injury + mx?
• Transfusion associated lung injury (TRALI)
o Antibodies in the transfused blood cause the patient’s WBC to aggregate + clog up the pulmonary capillaries
o Breathlessness
o Respiratory support
What is a delayed haemolytic reaction + mx?
• Delayed haemolytic reaction
o Mismatch of alleles of non-ABO blood groups (e.g. Duffy Kell, Kidd, Rhesus factor)
o Malaise, jaundice, fever
o Supportive treatment, symptoms are self-limiting