What hematologic disease has the highest postoperative morbidity after splenectomy: idiopathic thrombocytopenic purpura (ITP), hereditary spherocytosis, myeloid dysplasia, sickle cell anemia, or thalassemia?

From the Guidelines

The answer is (d) sickle cell anemia. Patients with sickle cell anemia have the highest postoperative morbidity following splenectomy among the listed hematologic diseases. This increased risk stems from several factors unique to sickle cell disease, including acute chest syndrome, a life-threatening complication that can be triggered by surgery, anesthesia, and postoperative pain 1. Additionally, sickle cell patients have baseline vaso-occlusive tendencies that can worsen during the perioperative period due to dehydration, hypoxia, acidosis, and hypothermia. They also face higher risks of perioperative infections due to functional asplenia even before surgical removal. Some key points to consider in the management of these patients include:

• Careful perioperative management including adequate hydration, oxygenation, pain control, and prophylactic antibiotics is essential when performing splenectomy in sickle cell patients to minimize these complications.
• The risk of death due to certain events was 3- to 4-fold higher in patients who had undergone splenectomy versus those with intact spleens, including the risks of septicemia, pulmonary embolism, and non-Hodgkin lymphoma 1.
• Splenectomized patients need lifelong management to prevent sepsis, such as vaccinations and prophylactic antibiotics, as well as surveillance for relapse 1. In contrast, patients with idiopathic thrombocytopenic purpura and hereditary spherocytosis typically have excellent outcomes after splenectomy with lower complication rates. Patients with myeloid dysplasia and thalassemia have intermediate risk profiles, but still lower than sickle cell patients. The use of pharmaceutical second-line treatment options has significantly decreased the use of splenectomy in ITP 1, but splenectomy may still be preferred by some patients who desire independence from medications. Overall, careful consideration of the risks and benefits of splenectomy is necessary for each patient, taking into account their individual disease characteristics and medical history.

From the Research

Postoperative Morbidity after Splenectomy

The question of which hematologic disease has the highest postoperative morbidity after splenectomy can be addressed by examining the provided evidence.

• The study by 2 found that patients with myelofibrosis had the highest incidence of complications (50%) and the highest postoperative mortality (21%; p = 0.04) after splenectomy.
• However, myelofibrosis is not listed as an option in the question. Among the options provided, the study by 2 does not specifically mention the postoperative morbidity for idiopathic thrombocytopenic purpura, hereditary spherocytosis, myeloid dysplasia, sickle cell anemia, or thalassemia.
• The study by 3 reports on the outcomes of splenectomy for various hematological disorders but does not provide a direct comparison of postoperative morbidity among the specific conditions listed in the question.
• The study by 4 compares the outcomes of splenectomy for benign and malignant hematologic conditions and finds that patients with malignant disease had a higher rate of overall complications (27.2%) compared to patients with benign disease (14.1%).
• The study by 5 reports on the modern morbidity, mortality, and long-term outcomes associated with splenectomy for benign and malignant hematologic disorders but does not provide a direct comparison of postoperative morbidity among the specific conditions listed in the question.
• The study by 6 examines the splenic pathology in myelodysplasia and reports on the clinical correlation but does not provide information on the postoperative morbidity after splenectomy for this condition compared to the other options listed.

Comparison of Postoperative Morbidity

Based on the provided evidence, there is no direct comparison of postoperative morbidity after splenectomy among the specific conditions listed in the question. However, the study by 2 suggests that myelofibrosis, which is not an option in the question, has a high postoperative morbidity. Among the options provided, the evidence does not allow for a clear determination of which condition has the highest postoperative morbidity after splenectomy.

• Idiopathic thrombocytopenic purpura: no specific information available from the studies
• Hereditary spherocytosis: no specific information available from the studies
• Myeloid dysplasia: the study by 6 provides some information on the splenic pathology in myelodysplasia but does not compare the postoperative morbidity to the other conditions
• Sickle cell anemia: no specific information available from the studies
• Thalassemia: no specific information available from the studies

It is essential to consult additional research or clinical guidelines to determine the postoperative morbidity after splenectomy for these specific conditions.