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ASPEN syndrome

  • adverse reaction after exchange transfusion in sickle cell disease
  • ASPEN (association of sickle cell disease, priapism, exchange transfusion, and neurologic events)
    • headache 
    • seizures
    • altered mental status
    • hemiparesis
    • above occur within 11 days of exchange transfusion


Indications for emergency transfusion/exchange transfusion in sickle cell disease

  • stroke
  • retinal artery occlusion
  • splenic sequesteration crisis
  • actue chest syndrome
  • aplastic crisis
  • priapism treated medically unless unsuccessful, then transfused


Indications for elective chronic transfusion in sickle cell

  • children with abnormal flow velocity by transcranial Doppler for stroke prevention
  • progressive renal or cardiopulmonary disease
  • complicated pregnancy
  • usual target HbS is <30% in children and < 50% in adults


Alloimmunization in multiply transfused sickle cell patients

  • with nonphenotypically matched blood, rate of alloimmunization per transfusion is 3%, overall rate of alloimmunization is between 19%-47%
  • most common alloantibodies
    • K
    • C
    • E
    • Fya
    • Jkb
  • with blood matched for Cc, D, Ee, Fya, and Jkb alloimmunization rate per transfusion is 0.5%


Class I hemorrhage

  • loss of <15% blood volume (<750 ml), usually asymptomatic or has mild tachy


Class II hemorrhage

  • loss of 15-30% of blood volume (750-1500 ml)
  • tachycardia, tachypnea, anxiety, clammy skin
  • only fluid resuscitation is required usually


Class III hemorrhage

  • loss of 30-40% of blood volume (1500-2000 ml)
  • hypotension
  • tachycardia
  • tachypnea
  • pallor
  • AMS
  • usually need transfusion


Class IV hemorrhage

  • loss of > 40% of blood volume (>2000 ml)
  • shock - thready pulse and risk of death
  • fluid resuscitation needed as well as transfusion


Principles of fluid resuscitation

  • fluid resuscitation comes first
    • red blood cells initiated once administration of fluid >30 ml/kg of body weight (~2 L)
  • all fluids have capacity to impair homeostasis, mainly through hemodilution


Emergency release

  • release of blood based on history of blood type is forbidden
  • release of blood based on forward type only is not good
  • physician must sign release stating the blood was not fully tested for compatibility within 24 hours (not required at time of release of blood)
  • blood product label must indicate that compatibility testing was not completed
  • consider giving Rh Ig if Rh+ blood given to woman of child-bearing age
  • when patient has known anti E or anti C, Rh- negative blood should be given


Principles of Rh Ig administration for Rh-incompatible blood products


- dose

- how do you administer

- contraindicated when?

  • RhIg should be given within 72 hours of transfusion
  • IV RhIg dosage: 90 IU/ 1 ml of transfused Rh+ RBCs /2ml transfused whole blood
  • administer entire dose of IV RhIg into vein over 3-5 minutes 
  • IV RhIg is discouraged for transfusion of quantity of Rh+ blood that excceds 20% of blood volume as this may cause severe hemolysis


Massive transfusion definition

  • transfused total blood volume (10-15 units of PRBC in a 70 kg patient)
  • 1/2 of patient's blood voume replaced within 3 hours
  • >4 units of RBCs are transfused within 4 hours


Complications of massive transfusion

  1. transfused blood does not immediately have the O2 carrying capacity of innate blood because of depletion of 2,3 DPG and ATP resulting in a shift of the O2 dissociation curve to the left (impaired release of O2)
  2. lower pH 
  3. increase K
  4. lower body temperature
  5. increase free Hgb
  6. coagulopathy 2/2 coagulation factors and platelet consumption, dysfunction, or dilution
    • thus the need for MTP in which platelets and plasma are also given with RBCs to prevent this