Which defect in the hemoglobin chain responds to splenectomy: idiopathic thrombocytopenic purpura (ITP), hereditary spherocytosis, thalassemia, or glucose-6-phosphate dehydrogenase (G6PD) deficiency?

From the Guidelines

The correct answer is (b) hereditary spherocytosis, as it is a defect in the hemoglobin chain that responds to splenectomy, although other options like thalassemia may also respond in certain cases. However, based on the provided evidence, hereditary spherocytosis is not directly mentioned as responding to splenectomy in the given texts, but it is known to respond to splenectomy in clinical practice. On the other hand, thalassemia, specifically beta-thalassemia intermedia and hemoglobin H disease, may respond to splenectomy in certain cases, as the spleen becomes hyperactive and removes abnormal red blood cells prematurely, contributing to anemia [ 1 ]. Splenectomy can help reduce this destruction and improve red cell survival, though it's not a first-line treatment and is reserved for specific cases with significant hypersplenism. The procedure works by removing the primary site of red cell destruction, thereby reducing hemolysis and improving anemia. However, splenectomy carries risks including increased susceptibility to infections and thrombotic complications, so the decision must be carefully considered [ 1 ]. Other conditions listed, like idiopathic thrombocytopenic purpura (ITP), have different pathophysiologies and management approaches, and splenectomy is considered in certain cases of ITP, but with careful consideration of the risks and benefits [ 1 ]. Some key points to consider when evaluating the response to splenectomy include:

• The underlying condition and its pathophysiology
• The severity of the disease and the impact on quality of life
• The potential risks and benefits of splenectomy
• The availability of alternative treatment options It is essential to weigh these factors carefully and consider the most recent and highest-quality evidence when making decisions about splenectomy in clinical practice.

From the Research

Defect in the Hemoglobin Chain that Responds to Splenectomy

• The correct answer is (b) hereditary spherocytosis, as it is a defect in the red blood cell membrane that responds to splenectomy 2, 3, 4, 5, 6.
• Hereditary spherocytosis is a hematologic disorder characterized by hemolytic anemia and splenomegaly, and splenectomy is an effective therapeutic option for this condition 2, 3, 4, 5, 6.
• Splenectomy removes the primary site of red blood cell destruction, eliminating anemia and hyperbilirubinemia, and lowering the high reticulocyte number to nearly normal levels 2, 3, 4, 5, 6.
• The other options, (a) idiopathic thrombocytopenic purpura, (c) thalassemia, and (d) glucose-6-phosphate deficiency, are not directly related to hereditary spherocytosis or do not respond to splenectomy in the same way 2, 3, 4, 5, 6.

Hereditary Spherocytosis and Splenectomy

• Hereditary spherocytosis is a disease affecting the red blood cells membrane, and splenectomy is considered the standard surgical treatment in moderate and severe forms of the disease 4.
• Total splenectomy exposes the patient to a life-long risk of potentially lethal infections, and partial splenectomy is a feasible alternative that aims to retain splenic immunologic function while decreasing the rate of hemolysis 4, 6.
• The long-term outcomes of patients with total or subtotal splenectomy for congenital hemolytic anemia are still unclear, but the majority of studies show a qualitative resolution of anemia and reduction of transfusion rate 4.