What is the best next step in managing a 5-year-old girl with sickle cell disease, presenting with lethargy, severe anemia (hemoglobin 5 g/dL), thrombocytopenia (platelets 70,000/mm3), and hypotension (blood pressure 80/40 mm Hg), currently on penicillin (antibiotic) and folic acid (vitamin B9)?

From the Guidelines

The best next step in management for this 5-year-old girl with sickle cell disease is packed red blood cell transfusion (option B). This patient is presenting with signs of acute splenic sequestration crisis, characterized by splenomegaly, severe anemia (hemoglobin 5 g/dL), thrombocytopenia, and hemodynamic compromise (tachycardia, hypotension). Packed red blood cell transfusion is urgently needed to restore blood volume, improve oxygen-carrying capacity, and reverse the shock-like state. The transfusion will help stabilize the patient's cardiovascular status and improve tissue perfusion. Splenic sequestration occurs when sickled red blood cells become trapped in the spleen, causing rapid splenic enlargement and circulatory collapse. While splenectomy may be considered for recurrent episodes, it is not the immediate intervention needed. Norepinephrine would not address the underlying anemia, tranexamic acid is used for bleeding disorders rather than sequestration, and whole blood transfusion is unnecessary when packed red blood cells can specifically address the critical anemia. After transfusion, the patient will require close monitoring for recurrence and potential complications, as highlighted in the management guidelines for sickle cell disease 1. It is also important to note that the patient's condition may be complicated by hyperhemolysis, a condition where transfused red blood cells are rapidly destroyed, leading to worsening anemia and potentially life-threatening complications 1. However, in this case, the immediate priority is to address the severe anemia and hemodynamic instability with packed red blood cell transfusion. The patient's care should be guided by the most recent and highest-quality evidence, including the American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support 1, and the guideline on the peri-operative management of patients with sickle cell disease: guideline from the Association of Anaesthetists 1.

Some key points to consider in the management of this patient include:

• The importance of prompt recognition and treatment of splenic sequestration crisis to prevent complications and improve outcomes 1
• The need for careful monitoring of the patient's hemoglobin level and transfusion requirements to avoid overtransfusion and potential complications 1
• The potential for hyperhemolysis and the need for extended matched red cell transfusions in some cases 1
• The importance of multidisciplinary care and collaboration between hematologists, anesthesiologists, and other specialists to optimize the patient's management and outcomes 1

From the Research

Patient Management

The patient is a 5-year-old girl with sickle cell disease, presenting with decreased energy and alertness, fatigue, and lethargy. Her laboratory results show a hemoglobin level of 5 g/dL, which is significantly below the normal range.

Transfusion Considerations

• The patient's low hemoglobin level indicates a need for transfusion to prevent tissue hypoxia 2.
• A restrictive transfusion strategy (trigger: Hb 7-8 g/dL) is as effective as a more liberal strategy (trigger: Hb about 10 g/dL) in certain patient groups 2, 3.
• The decision to transfuse should be guided by patient symptoms and preferences in conjunction with the patient's hemoglobin concentration 3.
• Red blood cell transfusion can improve anemia-related symptoms, such as fatigue and dyspnea, in the short term 4.

Best Next Step

• Given the patient's severe anemia (Hb 5 g/dL) and symptoms, packed red blood cell transfusion is the best next step in management 2, 3, 5.
• This is because transfusion can rapidly increase the patient's hemoglobin level and improve oxygen delivery to tissues.
• The other options, such as norepinephrine infusion, splenectomy, tranexamic acid, and whole blood transfusion, are not directly indicated for the management of severe anemia in this patient.

Key Considerations

• The patient's hemoglobin level is significantly below the threshold for transfusion, and transfusion is likely to improve her symptoms and oxygen delivery to tissues.
• The use of packed red blood cells is preferred over whole blood transfusion, as it allows for more targeted replacement of red blood cells and reduces the risk of transfusion-related complications 2, 6.