Red blood cell transfusions play an important role in the treatment of some acute illnesses in patients with sickle cell disease. For severe complications, timely transfusions may be life saving. Specific guidelines for the use of transfusions for individual complications are provided in the clinical care paths throughout this manual. In general, appropriate use of red cell transfusions requires attention to the following issues:

Indications for red cell transfusions include acute exacerbations of the patient's baseline anemia that require increased oxygen carrying capacity, acute life or organ-threatening vaso-occlusive episodes, and preparation for surgical or radiographic procedures that involve general anesthesia or the use of ionic contrast.

• Acute exacerbation of baseline anemia

1. Aplastic crisis

2. Splenic sequestration

3. Hepatic sequestration

4. Hyperhemolysis

• Severe vaso-occlusive events

1. Acute chest syndrome

2. Stroke

3. Severe infection

4. Acute multiorgan failure syndrome

• Preparation for procedures

1. General anesthesia and surgery

2. Radiographs with ionic contrast

Leukocyte-depleted, packed red blood cells are recommended. Where available, minor-antigen-matched, sickle-negative cells are preferred.

A simple transfusion of packed RBC is appropriate for most situations characterized by acute exacerbation of anemia. Partial exchange transfusion, generally by erythrocytapheresis, may be needed for severe life- threatening illness or in situations where a relatively high baseline hemoglobin precludes a simple transfusion that would risk hyperviscosity by increasing the hemoglobin level to > 10-11gm/dl.

Simple transfusion with 10cc/kg of packed RBC typically raises the hemoglobin about 2gm/dl. Patients with severe anemia that develops over several days (i.e. aplastic crisis) may be at risk for volume overload and congestive heart failure from rapid infusion of RBC. Thus, slow correction of the anemia (e.g. 4-5 cc/kg packed RBC over 4 hr, often with furosemide) or isovolemic partial exchange transfusion may be needed to prevent precipitation of heart failure.

Hyperviscosity

Because sickle red cells are poorly deformable, simple red cell transfusions that increase the hemoglobin levels to >10-11gm/dl may cause hyperviscosity in patients not receiving chronic transfusions and should be avoided.

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References