Transfusion in SS, Therapeutic Apheresis

Question 1

Transfusion in Sickle Cell Disease:

-What is the endpoint goal

Answer 1

• Proportion of HbS <30%

* Not a target hemoglobin

Question 2

What is ASPEN syndrome?

Answer 2

Association of Sickle cell Disease

• Priapism
• Exchange transfusion
• Neurologic events

Question 3

How does ASPEN syndrome present?

*Onset

Answer 3

• Headaches
• Seizures
• AMS
• Hemiparesis

*<11 days of exchange transfusion

Question 4

What are indications for elective chronic transfusion in SS? (4)

Answer 4

• Children with abnormal flow velocity by transcranial Doppler (stroke prevention)
• Renal disease
• Cardiopulmonary disease
• Pregnancy (complicated)

Question 5

What are the target HbS for elective chronic transfusion in SS?

• Kids
• Adults

Answer 5

<30% in children

<50% in adults

Question 6

What are the most common alloantibodies in multiply transfused SS patients? (5)

Answer 6

• K
• C
• E
• Fya
• Jkb

Question 7

When a patient who requires emergency release RBCs, has a known anti-E or anti-C antibody, what blood should they be given?

Answer 7

Rh Neg (Group O)

Question 8

What are the main complications in Massive Transfusion? (5)

Answer 8

• Left-shifted O2 dissociation curve
• Lower pH
• Increase Potassium
• Lower body temperature
• Increase free Hemoglobin

Question 9

What are Category I Indications for Apheresis? (10)

Answer 9

• Guillain-Barre
• ANCA ass/ RPGN
• Goodpastures
• Chronic Inflammatory Demyelinating Polyneuropathy (CIPD)
• Cryoglobulinemia
• FSGS (renal)
• Hyperviscosity d/t IgM paraprotein
• Myasthenia Gravis
• Thrombotic Thrombocytopenic Purpura (TTP)
• SCD, acute stroke

Question 10

What are Category II Indications for Apheresis? (7)

Answer 10

• Renal transplantation
• Antiphospholipid syndrome
• MS (acute)
• SLE: cerebritis, alveolar hemorrhage
• Chronic focal encephalitis
• Mushroom poisoning
• Neuromyelitis optica (Devic syndrome)
• SCD: acute chest, stroke, iron overload (prevention)
• Thrombocytosis (symptomatic)

Question 11

What 3 replacement fluids can be used in therapeutic apheresis?

Answer 11

• Normal (0.9%) saline
• 5% albumin
• Allogeneic plasma (FFP or cryo poor FFP) in TTP

Question 12

What replacement fluid is used therapeutic apheresis for TTP?

Answer 12

Allogeneic plasma (FFP or cryo poor FFP)

Question 13

What medication should be discontinued for 24 hours prior to apheresis?

Answer 13

ACE Inhibitors

-Risk of severe Hypotension