Short‐ and long‐term risks of splenectomy for benign haematological disorders: should we revisit the indications?

Abstract

SummarySplenectomy has represented a key treatment option in the treatment of many benign haematological diseases, including immune thrombocytopenia (ITP) and disorders associated with ongoing haemolysis (thalassaemia major and intermedia, sickle cell disease, and hereditary or acquired haemolytic anaemias). Improvements in surgical techniques have reduced perioperative complications and mortality. Preventive measures (new protein conjugate vaccines, antibiotic prophylaxis, and increased vigilance) are thought to greatly reduce the risk of overwhelming post-splenectomy infection (OPSI), although their implementation is inconsistent. Nevertheless, there is increasing documentation of the short-and long-term risks of splenectomy, which vary according to the underlying indication. Splenectomized patients are at increased risk of venous thromboembolism, particularly within the splenoportal system. The long-term thromboembolic risk is higher in haematological disorders associated with ongoing haemolysis, particularly in thalassaemia intermedia, which has led to a more conservative approach. In comparison, patients with ITP appear to be at lower risk of adverse effects of splenectomy, which maintains its place as the potentially most curative and safe second-line treatment. However, a splenectomy-sparing approach is also emerging for ITP, and recent guidelines recommend that this procedure is deferred until 12 months from ITP diagnosis, to allow sufficient time for possible remission.

Is ITP a thrombophilic disorder?

“…Comprehensive reviews on vascular complications after splenectomy for hematological disorders in general [25], or limited to benign ones only [6] have been published. In this latter review by the author, the reader will also find an in-depth discussion on peri-surgical, acute deep vein thrombosis and on portal vein thrombosis.…”

“…Hemorrhages usually occur in patients with platelet count less than 10-20 3 10 9 /L, but one fourth of patients are asymptomatic at the time of diagnosis, which includes all cases with platelets less than 100 3 10 9 /L. In the long history of ITP, thrombosis was not considered part of the spectrum of ITP manifestations, apart from sparse reports in which splenectomy in ITP was associated with an increased risk of arterial or venous thromboembolism [5], although at a much lower rate than in patients splenectomized for other benign or malign hematological disorders and only slightly increased over the risk found in controls after appendectomy [6]. The issue of thrombosis received much greater attention by medical literature with the introduction of second-generation thrombopoietins, also called thrombopoietin-receptor agonists (TPO-ra) or thrombopoietin mimetics, romiplostim, and eltrombopag.…”

Splenectomy in non-traumatic diseases

“…They should also undergo antibiotimicrobial chemoprophylaxis for the rest of their lives. 1,2,44 The risk of overwhelming post-splenectomy infection (OPSI) is both more common and closer to the date of splenectomy. It is characterized by rapid evolution, hypotension, altered consciousness, or cardio-circulatory shock.…”

“…Therefore, it is the only organ capable of removing encapsulated bacteria. 2 It's exeresis, thus, causes profound changes on the organism's homeostasis, in particular on the immune system. Nevertheless, it is the curative treatment for spontaneous rupture, cysts, abscesses, and some malignancies.…”

Splenomegaly, hypersplenism, and hereditary disorders with splenomegaly

“…By age 15 years, nearly 90% had undergone splenectomy with a resultant elimination of transfusion requirement in most TI patients and with a reduction in the need for red cell transfusions in TM patients. However, these improvement came at the expense of many splenectomy-associated complications including sepsis from encapsulated organisms (although this risk has been lessened with new protein conjugate vaccines and antibiotic prophylaxis), pulmonary hypertension, altered cytokine patterns [129] and an increased risk of thrombotic complications due to altered endothelial function attributable to larger numbers of circulating platelets and immature red cells, increased numbers of platelet derived blood microparticles, enhanced platelet activation, increases in levels of Protein C and S, and persistent post-splenectomy hemolysis [130]. Consequently, total splenectomy is now generally discouraged for children and adolescents with TM so that among children born after 1980, only 12% have had total splenectomy prior to age 20 years [128].…”