Elective (diagnostic or therapeutic) splenectomy

INTRODUCTION — Therapeutic splenectomy may be performed to treat certain conditions such as hereditary hemolytic anemias, autoimmune cytopenias, or symptomatic splenomegaly. Less commonly, splenectomy is used to make a diagnosis when there is unexplained splenomegaly or a splenic mass. There are several considerations to address before, during, and after the procedure to ensure the best outcomes and the least morbidity.

This topic discusses the indications for elective splenectomy, preprocedure interventions, operative technique, and postprocedure risks.

Traumatic splenectomy, the approach to evaluating splenomegaly, and the care of the asplenic (or functionally asplenic) patient are discussed separately:

●Anatomy and function of the spleen – (See "Splenomegaly and other splenic disorders in adults", section on 'Properties of the normal spleen'.)

●Splenic trauma, operative management – (See "Surgical management of splenic injury in the adult trauma patient".)

●Splenic trauma, non-operative management – (See "Management of splenic injury in the adult trauma patient".)

●Splenomegaly evaluation (child) – (See "Approach to the child with an enlarged spleen".)

●Splenomegaly evaluation (adult) – (See "Splenomegaly and other splenic disorders in adults".)

●Asplenic patient, prevention of infections – (See "Prevention of infection in patients with impaired splenic function".)

●Asplenic patient, treatment of infections – (See "Clinical features, evaluation, and management of fever in patients with impaired splenic function" and "Evaluation and management of fever in children and adults with sickle cell disease".)

INDICATIONS

Overview of indications — The conditions for which splenectomy may be appropriate therapy, those for which it is rarely used, and those for which it is generally contraindicated are listed in the table (table 1). Close consultation between the primary care provider, the consulting hematologist or oncologist, and the surgeon is important to determine whether splenectomy is the best treatment option for the individual patient and what preoperative, intraoperative, and postoperative considerations apply.

Broadly, indications for splenectomy include conditions with significant hemolysis or thrombocytopenia that depends on splenic reticuloendothelial function and/or autoantibody production; malignant or infectious disorders predominantly localized to the spleen; removal en bloc with other organs for cancer surgery; and rare complications of other disorders in which there is massive splenomegaly and/or hypersplenism with cytopenias [1]. Details are presented in the following sections.

Inherited hemolytic anemias — Splenectomy is an accepted therapy for several inherited hemolytic anemias and may be used to reduce hemolysis and improve the hemoglobin level when less invasive medical therapies have been ineffective. It may also provide symptomatic relief when there is massive splenomegaly that interferes with normal functioning.

●Hereditary RBC membrane disorders – Hereditary spherocytosis, elliptocytosis, pyropoikilocytosis, and Southeast Asian ovalocytosis are hereditary hemolytic anemias caused by variants in genes that encode components of the red blood cell (RBC) membrane and cytoskeleton. Splenectomy may be used in selected individuals (often children) who remain transfusion dependent or severely symptomatic after one year of age, although it is preferable to delay the procedure until after six years of age due to the possible risk of sepsis. (See "Hereditary spherocytosis", section on 'Splenectomy' and "Hereditary elliptocytosis and related disorders", section on 'Role of splenectomy'.)

●Thalassemia – Thalassemias include inherited hemolytic anemias of varying severity that generally result from a decrease in alpha globin or beta globin production. Some individuals with severe disease (transfusion-dependent thalassemia, growth retardation, hypersplenism, splenic infarction) may be treated with splenectomy, although severe complications are becoming less common when individuals are treated with regular transfusions, chelation therapy, activin receptor ligand trap therapy [2]. Gene therapy will also likely have an impact on the natural history of this disease and thus may decrease the need for splenectomy [3]. (See "Management of thalassemia", section on 'Role of splenectomy'.)

●Sickle cell disease – Sickle cell disease causes splenic infarction early in life and is rarely associated with splenomegaly or hypersplenism. However, some patients may have life-threatening splenic sequestration resulting from massive pooling of red blood cells in the spleen causing rapidly developing splenomegaly, anemia, and hypovolemia. This is a serious complication and mortality is high. There have been no prospective controlled studies in patients with a history of splenic sequestration crisis evaluating the benefit of splenectomy versus chronic transfusion therapy versus observation to prevent further attacks [4]. Some experts recommend splenectomy after one or two episodes of splenic sequestration. (See "Overview of the management and prognosis of sickle cell disease", section on 'Splenic and hepatic sequestration'.)

●Pyruvate kinase deficiency – Pyruvate kinase deficiency, which causes a reduced ATP production in RBCs, is a rare congenital nonspherocytic hemolytic anemia. Splenectomy is often performed in individuals with severe or transfusion-dependent anemia. If possible, surgery is delayed until after age 5 years to decrease the risk of sepsis [5]. A registry study in individuals with PK deficiency demonstrated an increase in hemoglobin of 1.6 g/dL after splenectomy, which is often less than what is seen in other inherited hemolytic anemias treated with splenectomy [6]. (See "Overview of the management and prognosis of sickle cell disease", section on 'Splenic and hepatic sequestration' and "Pyruvate kinase deficiency".)

Autoimmune cytopenias — In autoimmune cytopenias, the spleen may be the primary site of cell destruction due to reticuloendothelial macrophages, or it may be the source of the clone of cells producing the autoantibodies responsible for targeting the cells for destruction.

●ITP – Splenectomy is sometimes used in individuals (typically adults) with immune thrombocytopenia (ITP for whom initial therapies are ineffective in raising the platelet count to a safe range (eg, >30,000/microL) to reduce the risk of serious bleeding. Benefits and risks compared with other therapeutic options are presented separately. (See "Second-line and subsequent therapies for immune thrombocytopenia (ITP) in adults".)

●AIHA – Splenectomy is sometimes used in individuals with autoimmune hemolytic anemia (AIHA) for whom initial therapies are ineffective in improving the hemoglobin level and reducing hemolysis. Benefits and risks compared with other therapeutic options are presented separately. (See "Warm autoimmune hemolytic anemia (AIHA) in adults", section on 'Initial management'.)

●Felty syndrome – Splenectomy is rarely used for autoimmune neutropenia. An exception is Felty syndrome (splenomegaly and autoimmune neutropenia in individuals with rheumatoid arthritis), for whom splenectomy may be used if there are recurrent or severe infections and other interventions have been ineffective. (See "Role of splenectomy for Felty syndrome".)

SMZL — Splenic marginal zone lymphoma (SMZL) is a B-cell non-Hodgkin lymphoma that typically presents in adulthood with splenomegaly and lymphocytosis. Splenectomy may be used for symptom control in some individuals who are symptomatic from localized splenic involvement. (See "Splenic marginal zone lymphoma", section on 'Splenectomy'.)

Splenic abscess — Formation of an abscess in the spleen is a rare complication of other infections, typically seeded hematogenously (eg, from bacterial endocarditis). Management typically involves antibiotics and splenectomy, although experience with image-guided percutaneous drainage has been developing. (See "Splenomegaly and other splenic disorders in adults", section on 'Abscess and infarction' and "Splenomegaly and other splenic disorders in adults", section on 'Imaging and biopsy'.)

Splenic or portal vein thrombosis with bleeding varices — Splenic or portal vein thrombosis can be complicated by bleeding gastric varices. Some individuals are treated with splenectomy if they have repeated bleeding episodes. (See "Chronic portal vein thrombosis in adults: Clinical manifestations, diagnosis, and management", section on 'Management of complications'.)

Cancer surgery — Surgical excision or tumor debulking may include splenectomy (intentional or accidental), especially for the following cancer types:

●Ovarian cancer – (See "Cancer of the ovary, fallopian tube, and peritoneum: Surgical cytoreduction", section on 'Splenectomy'.)

●Colon cancer involving the splenic flexure – (See "Overview of colon resection", section on 'Anticipated splenectomy'.)

●Gastric cancer with direct splenic extension – (See "Surgical management of invasive gastric cancer", section on 'Extent of lymph node dissection'.)

●Pancreatic cancer that requires distal (or total) pancreatectomy – (See "Overview of surgery in the treatment of exocrine pancreatic cancer and prognosis" and "Total pancreatectomy", section on 'Standard technique'.)

Miscellaneous conditions — Wandering spleen is a rare condition in which there is laxity in the ligaments that hold the spleen in place. Individuals with this condition are at risk for complications including splenic torsion and/or infarction. Splenectomy is often recommended in these individuals [7]. Ideally, this is done on an elective basis, although patients present with an acute complication requiring emergency surgery [8]. (See "Splenomegaly and other splenic disorders in adults", section on 'Abscess and infarction'.)

Symptomatic or massive splenomegaly may also be treated with splenectomy. In these cases, the role of splenectomy depends on the underlying cause of splenomegaly and the risks and benefits of other available therapies.

Occasionally, individuals will have an enlarged spleen or a splenic mass without obvious cause despite extensive work-up, including bone marrow biopsy, peripheral blood flow cytometry, and imaging studies. Such patients could be considered for diagnostic splenectomy.

●Splenic mass – A series of 28 individuals who underwent splenectomy for a splenic mass (mostly solitary) found that approximately three-fourths had non-Hodgkin lymphoma and one-fourth had benign tumors (eg, inflammatory pseudotumor) [9]. (See "Splenomegaly and other splenic disorders in adults", section on 'Focal splenic lesions'.)

●Splenomegaly – A series of 1280 individuals who underwent splenectomy over a 10-year period from 1986 to 1995 reported that approximately 10 percent were done for diagnostic purposes [10]. The most common finding was lymphoma (often a primary splenic lymphoma) or leukemia (57 percent) followed by metastatic cancer (11 percent) or cyst/pseudocyst (9 percent). Less-common findings included vascular neoplasms, granulomatous lesions, infarction, hemorrhage, and extramedullary hematopoiesis. Other series have also reported lymphoma to be a common finding [11,12]. (See "Splenomegaly and other splenic disorders in adults", section on 'Evaluation (splenomegaly)'.)

CONDITIONS IN WHICH SPLENECTOMY IS GENERALLY CONTRAINDICATED — While there are no absolute contraindications to splenectomy, in certain conditions, splenectomy has been found either to be ineffective or to cause significant toxicity.

Splenectomy is rarely the treatment of choice but may be appropriate in selected individuals with the following:

●ABO or human leukocyte antigen (HLA) desensitization for kidney transplantation – (See "Kidney transplantation in adults: ABO-incompatible transplantation", section on 'Overview of desensitization'.)

●Chronic lymphocytic leukemia (CLL) – (See "Overview of the complications of chronic lymphocytic leukemia", section on 'Hypersplenism'.)

●Hairy cell leukemia (HCL) – (See "Treatment of hairy cell leukemia", section on 'Splenectomy'.)

●Hodgkin lymphoma (staging splenectomy is no longer standard of care) – (See "Pretreatment evaluation, staging, and treatment stratification of classic Hodgkin lymphoma".)

●Myeloproliferative neoplasms with massive splenomegaly, especially primary myelofibrosis (PMF) – (See "Myelofibrosis (MF): Management of primary MF and secondary MF", section on 'Splenectomy/splenic irradiation'.)

●Splenic infarction – (See "Splenomegaly and other splenic disorders in adults", section on 'Abscess and infarction'.)

●Splenic sequestration crisis in sickle cell disease – (See "Overview of the management and prognosis of sickle cell disease", section on 'Splenic and hepatic sequestration'.)

●Thrombotic thrombocytopenic purpura (TTP) – (See "Immune TTP: Treatment of clinical relapse", section on 'Clinical relapse'.)

Splenectomy is generally contraindicated in the following:

●ALPS – Autoimmune lymphoproliferative syndrome (ALPS) is a syndrome of immune dysregulation (lymphoproliferation, autoimmune cytopenias) that is typically inherited but may not present until adulthood. Splenectomy in these individuals leads to unacceptably high rates of sepsis and death due to encapsulated organisms. (See "Autoimmune lymphoproliferative syndrome (ALPS): Management and prognosis", section on 'Treatment of disease manifestations' and "Autoimmune lymphoproliferative syndrome (ALPS): Management and prognosis", section on 'Prevention of complications'.)

●Cold agglutinin disease and paroxysmal cold hemoglobinuria – Cold agglutinin disease is a type of autoimmune hemolytic anemia in which the autoantibodies bind at cold temperatures and fix complement. Splenectomy is unlikely to be effective because the primary site of RBC phagocytosis is the liver rather than the spleen. (See "Cold agglutinin disease", section on 'Management'.)

In paroxysmal cold hemoglobinuria, IgG autoantibodies bind at cold temperatures and fix complement, leading to intravascular hemolysis. Splenectomy has no role because intravascular hemolysis occurs independent of splenic function. (See "Paroxysmal cold hemoglobinuria", section on 'Treatment'.)

●Gaucher disease – Gaucher disease is the most common inherited lysosomal storage disease. The advent of enzyme replacement or substrate reduction therapies have made splenectomy unnecessary in the majority of patients. Splenectomy accelerates accumulation of Gaucher cells at other sites and can worsen bone disease, liver disease, pulmonary hypertension, and bone marrow involvement [13]. (See "Gaucher disease: Treatment", section on 'Splenectomy'.)

●Hereditary stomatocytosis or xerocytosis – Hereditary stomatocytosis and hereditary xerocytosis are inherited RBC disorders in which genetic variants lead to altered RBC membrane ion channels and hemolytic anemia. Splenectomy does not significantly reduce hemolysis and is associated with a much greater risk of vascular complications including thromboembolic events, vaso-occlusive episodes, and/or pulmonary hypertension. (See "Hereditary stomatocytosis (HSt) and hereditary xerocytosis (HX)", section on 'Management'.)

●Thrombocytopenia in hepatic cirrhosis – Cirrhosis can cause hypersplenism and thrombocytopenia, but there are other therapeutic alternatives, and splenectomy has a high complication rate. (see 'Alternatives to splenectomy' below) make splenectomy controversial in this setting. (See "Hemostatic abnormalities in patients with liver disease", section on 'Thrombocytopenia and platelet dysfunction' and "Hemostatic abnormalities in patients with liver disease", section on 'General approach to invasive procedures'.)

PREOPERATIVE CONSIDERATIONS — Ideally, surgery is planned with sufficient time to perform any needed preoperative testing, interventions (including preoperative vaccinations), and/or medication changes to ensure the best outcome.

Alternatives to splenectomy — Individuals considering splenectomy should have the opportunity to compare the benefits and risks with those of other alternatives.

●In some autoimmune conditions, rituximab, growth factor agonists (eg, TPO agonists) or immunosuppressive agents are often considered; the relative efficacy and complication rate for the specific disorder should be evaluated.(See "Second-line and subsequent therapies for immune thrombocytopenia (ITP) in adults", section on 'Choice of second-line therapy'.)

●If splenectomy is being considered for diagnostic purposes, non-invasive tests should be done such as genetic testing for Gaucher disease or bone marrow, flow cytometry, genetic testing, or imaging for hematologic malignancies or amyloidosis. (See "Splenomegaly and other splenic disorders in adults", section on 'Causes (splenomegaly)' and "Splenomegaly and other splenic disorders in adults", section on 'Evaluation (splenomegaly)'.)

●For isolated splenic lesions, it may be possible to perform a fine needle aspiration (FNA), reserving splenectomy for those in whom the FNA is non-diagnostic. In one case series, 88 of 130 FNA procedures provided a diagnosis [14]. Major bleeding can occur in up to 4 to 5 percent of individuals undergoing splenic biopsy and appears to occur in <2 percent of those undergoing FNA [15,16]. (See "Splenomegaly and other splenic disorders in adults", section on 'Imaging and biopsy'.)

●For thrombocytopenia due to liver disease in which a higher platelet count is needed for surgery, the platelet count can be raised using a thrombopoietin receptor agonist or platelet transfusions if there are time constraints [17]. However, the response to platelet transfusions is hampered by a blunted response and shorter half-life of the transfused platelets [18]. (See "Hemostatic abnormalities in patients with liver disease", section on 'Invasive procedures'.)

Preoperative testing — Individuals undergoing splenectomy will have had a complete blood count (CBC) with platelet count and differential. Results should be reviewed to confirm that they are consistent with the underlying diagnosis for which splenectomy is being performed, as well as to identify any abnormalities that might need to be addressed prior to surgery such as severe thrombocytopenia.

Coagulation studies (prothrombin time [PT] and activated partial thromboplastin time [aPTT]) are typically appropriate.

Other testing is appropriate in selected individuals:

●In individuals with myelofibrosis, bone marrow aspirate and biopsy may be helpful in determining whether there is adequate hematopoietic tissue outside the spleen. (See "Myelofibrosis (MF): Management of primary MF and secondary MF", section on 'Splenectomy/splenic irradiation'.)

●In individuals with autoimmune hemolytic anemia (AIHA) or immune thrombocytopenia (ITP), serum protein electrophoresis may identify a monoclonal protein that impacts therapeutic decision making. (See "Laboratory methods for analyzing monoclonal proteins", section on 'Serum protein electrophoresis (SPEP)'.)

Routine preoperative testing related to surgical risk is individualized according to the patient's clinical status. (See "Preoperative medical evaluation of the healthy adult patient" and "Evaluation of perioperative pulmonary risk" and "Evaluation of cardiac risk prior to noncardiac surgery".)

There do not appear to be any studies demonstrating that preoperative imaging for an accessory spleen improves surgical approach or management. One study investigated the usefulness of preoperative CT scanning and found that accessory spleens were readily detected by the surgeon at laparoscopy, and the preoperative imaging did not enhance patient care [19]. (See 'Surgical approach' below.)

Vaccinations — Providing appropriate vaccinations prior to splenectomy is one of the most important preoperative interventions. Splenectomy increases the risk for serious, including life-threatening, infections, especially with encapsulated organisms such as Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis, and vaccination against these organisms may reduce the risk. Influenza vaccination and coronavirus disease 2019 (COVID-19) vaccination should also be provided.

Pre-splenectomy vaccinations are listed in the table (table 2). These should be administered at least two weeks prior to planned splenectomy when feasible. In addition, routine vaccinations for which the individual would be due should also be provided, as these are more likely to be effective in an individual with an intact spleen. The ideal timing of vaccination prior to splenectomy is approximately 10 to 12 weeks to allow completion of the full vaccine series ≥2 weeks prior to the procedure (some are given as two doses 8 weeks apart); in some cases, this may not be possible. A good immune response to most vaccines occurs within two weeks, and this timing may be reasonable in individuals with a more urgent need for splenectomy. However, even if the procedure is done urgently, vaccinations should be administered, as this may provide some degree of immunity. Important information about the selection of vaccine, dosing, and sequencing is presented in detail separately. (See "Prevention of infection in patients with impaired splenic function", section on 'Vaccinations'.)

For individuals who do not receive pre-splenectomy vaccinations for any reason, there is evidence that vaccination induces adequate antibody responses when given approximately 14 days after the procedure. Appropriate vaccinations that were not administered before splenectomy should be given. (See "Prevention of infection in patients with impaired splenic function", section on 'Timing of vaccination'.)

Annual influenza vaccination and COVID-19 vaccination can reduce mortality from secondary bacterial infection and should be administered. (See "Seasonal influenza in children: Prevention with vaccines" and "Seasonal influenza vaccination in adults" and "COVID-19: Vaccines".)

The risk of infection with the organisms listed above as well as others for which vaccinations are not available is discussed below. (See 'Infection' below.)

Optimizing hemoglobin and platelet count — In some cases, the individual undergoing splenectomy may have anemia or thrombocytopenia due to their underlying disorder. There is no absolute hemoglobin or platelet count target required before surgery, but in most cases, it is reasonable to use available therapies to optimize the hemoglobin or platelet count to reduce the risk of hemodynamic compromise or bleeding, respectively.

●Hemoglobin – The average estimated blood loss from splenectomy ranges from approximately 150 mL with laparoscopic procedures to 250 mL with open procedures, although it may be higher, especially if a laparoscopic procedure is converted to a laparotomy [20]. Blood transfusion was administered to 5 percent of the patients undergoing laparoscopy and approximately one-fourth of those undergoing laparotomy. Transfusion thresholds are discussed separately. (See "Red blood cell transfusion in infants and children: Indications" and "Indications and hemoglobin thresholds for RBC transfusion in adults".)

●Platelet count – Ideally, the platelet count is above 50,000/microL. In patients with ITP, this may be achieved using appropriately timed intravenous immune globulin (IVIG) or a thrombopoietin receptor agonist. Platelet transfusion may be started after the spleen has been vascularly isolated (devascularized) so as not to sequester the transfused platelets in the spleen. (See "Second-line and subsequent therapies for immune thrombocytopenia (ITP) in adults", section on 'Pre-splenectomy considerations'.)

Other medication management — Medications should be reviewed prior to the procedure to determine if any need to be adjusted or discontinued. The following are examples related to medications the patient may be receiving:

●Glucocorticoids – If splenectomy is being considered, it is likely that corticosteroids were not effective or a high dose was required to maintain a safe platelet count. If patients are still on a high dose of corticosteroids prior to surgery, it is good practice to try and reduce that dose prior to surgery to minimize complications related to infection and wound healing. In one study that evaluated whether there was a connection between glucocorticoid use and increased risk of infections, preoperative glucocorticoids did not appear to alter the postoperative infection rate [21].

●Rituximab – There are several potential issues of which to be aware in patients undergoing splenectomy who have received or will receive rituximab:

•Rituximab is typically given as a once-weekly infusion for four weeks for autoimmune disorders such as ITP. If splenectomy is being performed, it is possible that the splenectomy was indicated because rituximab was ineffective, and it is reasonable to discontinue the infusions [16] once splenectomy has been planned. An exception might be an individual who may be developing a response and for whom it is decided to continue rituximab, assess the efficacy, and possibly cancel the splenectomy if a late response to rituximab occurs.

•Vaccination success rates are likely to be lower in individuals who have received rituximab. Pre-splenectomy vaccinations are prudent prior to starting rituximab if there is a possibility of splenectomy in the future. (See "Rituximab: Principles of use and adverse effects in rheumatoid arthritis", section on 'Immunization and response to vaccines'.)

•Rituximab can cause hypogammaglobulinemia. While this is usually mild, transient, and well tolerated, there are instances of persistent hypogammaglobulinemia with serious infectious complications. The infection rate could be worse following splenectomy. (See "Secondary immunodeficiency induced by biologic therapies", section on 'Rituximab'.)

●Ruxolitinib – Abrupt discontinuation of ruxolitinib has been associated with serious adverse events including relapse of disease symptoms and/or a sepsis-like syndrome, presumably related to a rebound increase in cytokines [22]. Therapy should be gradually tapered rather than abruptly discontinued. It might be preferable to do this preoperatively to avoid any potential increased risk for infectious complications. (See "Treatment of acute graft-versus-host disease", section on 'Ruxolitinib'.)

Other aspects of perioperative medication management are presented separately. (See "Perioperative medication management" and "Perioperative management of patients receiving anticoagulants".)

VTE prophylaxis — Venous thromboembolism (VTE) prophylaxis, in keeping with standard practice, is appropriate for all patients. Prospective and retrospective studies have demonstrated that splenectomy carries a higher postoperative VTE risk than other types of major abdominal surgery [23,24]. Several disorders for which splenectomy may be performed (autoimmune hemolytic anemia, immune thrombocytopenia, myelofibrosis, pancreatic cancer) carry a higher risk for VTE independent of surgical risk, making it challenging to determine whether the increased postoperative VTE risk is related to the underlying disorder, the surgery, or both. (See 'Venous thromboembolism' below.)

Regardless of the mechanism, close attention to VTE prophylaxis and evaluation of concerning symptoms for VTE are prudent. (See "Prevention of venous thromboembolic disease in adult nonorthopedic surgical patients".)

If splenectomy is being performed to treat ITP, it may be prudent to withhold prophylactic anticoagulation until the postoperative period.

Information about long-term VTE risk following splenectomy is discussed below. (See 'Venous thromboembolism' below.)

SURGICAL APPROACH

Open versus laparoscopic procedure — The spleen can be removed either using an open laparotomy or a laparoscopic approach. In institutions with the appropriate surgical expertise, laparoscopic splenectomy has become the standard operative technique for many indications [25]. However, the procedures have not been directly compared in a randomized trial, and some centers may perform open splenectomy depending on local expertise or anatomic considerations [1]. It also appears that successful laparoscopic surgery can be performed in individuals with large spleens [26].

Reasons to prefer laparoscopic splenectomy include the following:

●Lower surgical mortality – In a systematic review that included over 6000 splenectomies for immune thrombocytopenia, laparoscopic splenectomy was associated with a mortality of 0.2 percent (3 of 1301 patients), whereas laparotomy with an open procedure was associated with a mortality of 1 percent (48 of 4955 individuals) [27]. In a retrospective series involving 1781 splenectomies from the American College of Surgeons National Surgical Quality Improvement Program (NSQIP) database that evaluated operative mortality at 30 days, laparoscopic splenectomy had a lower odds of death than open splenectomy after adjusting for numerous potential confounding variables (odds ratio [OR], 0.39; 95% CI 0.18-0.84) [28]. Another study using NSQIP data also found reductions in operative morbidity, although the magnitude of the effect was smaller [29]. (See 'Operative morbidity and mortality' below.)

●Shorter hospitalization and faster recovery – One of the reviews from the NSQIP also evaluated length of hospital stay and found that this was approximately two days shorter with the laparoscopic procedures [29]. In a smaller report confined to 381 individuals who underwent splenectomy for diagnosis or treatment of a hematologic disorder, the median length of hospital stay was approximately three days with a laparoscopic approach and six days with laparotomy [20]. A 2021 meta-analysis (which included a small prospective randomized trial of 27 patients) demonstrated a decrease in hospital length of stay and decreased blood loss in patients treated with laparoscopic compared with open splenectomy [30].

●Reduced complications – Long-term complications such as infection and venous thromboembolism (VTE) appear similar with open and laparoscopic splenectomy. (See 'Postoperative risks' below.)

In the perioperative period, individuals undergoing laparoscopic rather than open splenectomy have been reported to receive fewer transfusions [20]. In a report from a high-volume center, the median blood loss was only 50 mL (interquartile range, 20 to 150 mL) [25]. However, it is unclear if the use of laparoscopy reduced the need for transfusions or if laparoscopic splenectomy is done less frequently in individuals with massive splenomegaly who are likely to have a primary bone marrow disorder such as leukemia, myelodysplastic syndrome, or myeloproliferative neoplasm. Such individuals tend to be older and are more likely to require transfusions than individuals with autoimmune cytopenias.

Settings in which an open procedure may be preferred include the following:

●Massive splenomegaly with concern about the ability to remove the spleen via a laparoscopic procedure. In a retrospective analysis comparing operative times with laparoscopic versus open splenectomy in 78 individuals with massive splenomegaly (spleen weight >1000 g on pathologic examination), operative times were longer for those undergoing laparoscopic procedures [31].

●Local expertise favoring an open procedure or lack of support or equipment for laparoscopy.

●Ability to search more thoroughly for an accessory spleen.

●Cancer surgery or adhesion of the spleen to adjacent organs requiring laparotomy.

In some cases, laparoscopic splenectomy may need to be converted to an open procedure. The likelihood of this happening has been estimated to be in the range of 3 to 10 percent [1,20,25]. Reasons for conversion included excess bleeding, technical problems, splenic size, and other abnormalities such as friability or infiltration of surrounding structures.

Partial splenectomy — Partial splenectomy involves removing a portion of the spleen and leaving a portion intact to allow some residual immune function. This procedure is generally reserved for children who require splenectomy when they are younger than six years of age. Following partial splenectomy, the spleen may regrow and a second procedure may be required. However, this may allow the patient to delay total splenectomy until they are considerably older and have a reduced risk of sepsis. (See "Hereditary spherocytosis", section on 'Operative techniques'.)

A systematic review from 2019 identified over 2000 reports of partial splenectomy over the course of approximately 60 years [32]. The majority were performed for hematologic disorders (over 1000), focal lesions such as nonparasitic or parasitic cysts or neoplasms (nearly 400), and portal hypertension (nearly 400). Most were done using an open laparotomy (81 percent); the remainder used a laparoscopic approach, and 27 were done robotically. Vascular dissection and parenchymal transection methods evolved over the years. Conversion to total splenectomy occurred intraoperatively in 14 individuals and postoperatively in 12; an additional 61 subsequently underwent elective conversion to total splenectomy to further treat their underlying hematologic condition. The mean length of hospital stay was six days, and overall complications were low. There were three deaths, one due to colonic perforation on postoperative day six and two from sepsis (one eight months after total splenectomy, and one 13 years after splenectomy). The long-term efficacy and safety of this procedure could not be evaluated in a systematic and thorough fashion due to the nature of the studies reviewed (retrospective and case reviews) and lack of data regarding detailed follow-up. It is noteworthy that many of the patients who required elective conversion to total splenectomy had hematologic indications for their partial splenectomy. A randomized trial might help to determine whether partial or total splenectomy is preferred in individuals with hematologic indications for splenectomy.

Partial splenectomy as a means of salvaging splenic function in individuals undergoing trauma surgery is discussed separately. (See "Surgical management of splenic injury in the adult trauma patient", section on 'Splenic salvage'.)

Simultaneous cholecystectomy — In some individuals, it may be reasonable to perform cholecystectomy at the same time as splenectomy. This is especially true for those with a history of pigment gallstones, which can develop as a complication of chronic hemolysis, such as with an inherited hemolytic anemia. (See 'Inherited hemolytic anemias' above.)

For individuals with inherited hemolytic anemias who do not have gallstones, splenectomy may reduce the risks of hemolysis and gallstone development, and prophylactic cholecystectomy may not be justified. (See "Hereditary spherocytosis", section on 'Splenectomy'.)

Accessory spleen — An accessory spleen is a separate collection of splenic tissue physically independent from the spleen, with an average size of approximately 1.5 cm in diameter [33]. The majority of accessory spleens are located in the splenic hilum, gastrosplenic ligament, splenorenal ligament, and greater omentum. They have also been located in the tail of the pancreas, where they might be mistaken for an intrapancreatic neoplasm. An accessory spleen is thought to form when the splenic tissue fails to fuse properly during embryogenesis. Splenosis arises from trauma, when a piece of the spleen breaks off and becomes lodged in the peritoneal cavity. (See "Splenomegaly and other splenic disorders in adults", section on 'Terminology'.)

A search for an accessory spleen is often done at the beginning of the procedure, especially in conditions for which splenectomy may be less effective therapeutically if the accessory spleen is not removed at the time of splenectomy, leading to recurrence of the underlying condition for which splenectomy was performed. Concerns about intra-abdominal hemorrhage from spontaneous or traumatic rupture of an accessory spleen have also been raised [33].

The prevalence of accessory spleens is not known. In a systematic review that included data on over 22,000 patients from imaging, operative, and cadaveric reports, the prevalence of an accessory spleen was 14.5 percent [33]. The prevalence was only slightly higher when restricted to individuals with immune thrombocytopenia (ITP; 16.7 percent); additionally, approximately one-fourth of the individuals with ITP who had an accessory spleen had more than one. The prevalence was slightly lower when restricted to healthy individuals (incidental finding) at 11.2 percent, although this may reflect bias in reporting.

An accessory spleen can be detected using various imaging modalities (eg, ultrasound, computed tomography, magnetic resonance imaging); however, we do not perform imaging specifically to search for an accessory spleen, as this has not been demonstrated to enhance management (see 'Preoperative testing' above). In other cases, it is identified during splenectomy when the surgeon explores the abdomen. Some individuals only discover the presence of an accessory spleen when evaluating the cause of refractory or relapse of their underlying condition.

POSTOPERATIVE RISKS — The major postoperative risks, infection and venous thromboembolism (VTE), appear to be increased following splenectomy regardless of the initial indication for splenectomy. In contrast, some of the less-common complications may be specific to certain underlying disorders.

Operative morbidity and mortality — The operative mortality of splenectomy depends on the patient's clinical status and underlying disease, expertise of the surgical team, and infection prevention measures.

In a series of 381 children and adults who underwent splenectomy for diagnosis or treatment of a hematologic condition between 1997 and 2010, the overall 30-day operative mortality was 6.3 percent; three-fourths of the deaths were directly related to an infectious complication (pneumonia, wound infection, intra-abdominal abscess) [20]. Multivariate analysis found that the three independent predictors of major complications were age >65 years, platelet count ≤50,000/microL, and Karnofsky performance score ≤60 [20]. At a high-volume referral center that reported on 500 laparoscopic procedures over a 20-year period from 1998 to 2017, there were two deaths, giving an overall mortality of 0.4 percent [25]. The causes of death were massive hemorrhage and pulmonary embolism.

Bleeding — The location of the spleen in the left upper abdominal quadrant places it in close proximity to a number of important structures and their vasculature supplies, including the stomach, pancreas, left kidney, and splenic flexure of the colon. Further, the splenic blood supply is very rich. Accordingly, removal of the spleen may place one or more of these structures and/or their vascular supplies at risk.

In a series of 604 patients from China who underwent splenectomy for trauma or portal hypertension, intraperitoneal hemorrhage occurred in 14 (2 percent); most occurred within 24 hours of the procedure, and half were preceded by sentinel bleeding (minor blood loss around the surgical drains) a few hours before major bleeding occurred [34]. All but one underwent re-exploration, and the sites of hemorrhage were found to be in the splenic bed, short gastric artery, pancreatic tail, and splenic artery. Three of the patients with hemorrhage died, compared with seven patients in the non-hemorrhage group (21 versus 1 percent, respectively). Risk factors for hemorrhage included postoperative antiplatelet agents or anticoagulation for VTE prophylaxis.

Infection — The spleen is a major immune organ. It is the principal site responsible for removing opsonized bacteria from the bloodstream via the monocyte macrophage system, as well as a key secondary organ for producing mature B-cells during the antigen-dependent phase of the humoral immune response. (See "Splenomegaly and other splenic disorders in adults", section on 'Function' and "Normal B and T lymphocyte development", section on 'Mature B cells'.)

Infections with the parasites plasmodium falciparum (malaria), Babesia microti (babesiosis), and Anaplasma phagocytophilum (human granulocytic ehrlichiosis) are also increased or of greater severity in individuals who have undergone splenectomy.

Infection, including life-threatening sepsis, is of concern in any individual who has undergone splenectomy and especially in young children or individuals with other immune deficits. As noted above, encapsulated organisms are of concern because the splenic filtering function is no longer present following splenectomy; however, infections are not limited to encapsulated organisms. (See 'Vaccinations' above.)

Rituximab may increase this risk, as noted above. (See 'Other medication management' above.)

In a series of over 8000 veterans who were followed for over 20 years after splenectomy, infection was the most common long-term complication (adjusted relative risk [RR] for sepsis, pneumonia, and meningitis, 3.4, 2.4, 1.9, respectively) [35]. These risks were independent of the underlying indication for splenectomy and persisted over the course of the study, suggesting that infectious risk does not wane over time. Other studies have reported similar findings and have identified sepsis as the most common cause of post-splenectomy death during extended follow-up [36-38].

Any sign of a systemic infection (eg, fever with a single oral temperature >38.3°C [101°F]) in an asplenic individual should be considered a medical emergency; most individuals presenting with a suspected bacterial infection should be admitted to the hospital, have blood cultures obtained, and receive broad-spectrum antibiotics pending the results [1]. Outpatient management may be appropriate for an individual who does not appear clinically ill, has normal laboratory testing (eg, complete blood count [CBC]), and in whom follow-up can be obtained in a timely manner. If patients are distant from a medical facility, oral administration of an antibiotic is appropriate; plans for this approach and a prescription should be provided to the patient. Prompt antibiotic therapy of animal bites (including dog bites) is recommended by some experts. (See "Clinical features, evaluation, and management of fever in patients with impaired splenic function", section on 'Evaluation and management'.)

Recommendations for prophylactic antibiotics vary, and the approach in the post-pneumococcal era is based on expert opinion rather than randomized controlled trials. A common recommendation for children <5 years of age is for antibiotic prophylaxis for at least two years following splenectomy (table 3). Patients should be advised to carry a medical alert card or bracelet stating that they are asplenic, and the medical record should be updated to include splenectomy as an active problem. Additional details as well as other preventive measures related to travel and animal exposures are presented in detail separately. (See "Prevention of infection in patients with impaired splenic function", section on 'Antibiotic prophylaxis' and "Prevention of infection in patients with impaired splenic function", section on 'Other considerations'.)

Venous thromboembolism — Splenectomy increases the risk of venous thromboembolism (VTE), including portal and splenic vein thrombosis as well as deep vein thrombosis (DVT) and pulmonary embolism (PE) [39]. The cause may be multifactorial and include thrombocytosis, alterations in the vascular anatomy, increased blood viscosity, and/or changes in coagulation parameters that may be related to the underlying diagnosis and/or to splenectomy procedure [39].

In retrospective studies, the absolute risk of VTE has ranged from 3 to 7 percent [40-42]. Malignancy and myeloproliferative neoplasms and increased platelet count postoperatively appeared to be associated with greater risk.

In the series of over 8000 veterans who were followed for over 20 years after splenectomy, the rates of DVT and PE were both increased (RR, 2.2 for both) [35]. As with infection, this increased risk was independent of the underlying indication for splenectomy and persisted over the course of the study. Other studies have corroborated this increased risk and identified possible contributing factors such as increased age [43].

The rate of VTE appears to be especially high after splenectomy in individuals with hereditary stomatocytosis. In one large series, 11 of 13 individuals who underwent splenectomy developed severe, recurrent thromboses, whereas 9 of 10 with intact spleens did not develop VTE [44]. This is part of the rationale for avoiding splenectomy. (See 'Conditions in which splenectomy is generally contraindicated' above.)

Cardiovascular events and pulmonary hypertension — Some studies mention an association between splenectomy and an increased risk of cardiovascular complications and/or pulmonary hypertension (PH). However, evidence for a causal relationship is lacking, and the risk appears lower than that of venous thrombotic events [45].

●Ischemic heart disease has multifactorial risks that may be challenging to adjust for in postoperative series. In the series of over 8000 veterans who were followed for over 20 years after splenectomy, the risk of heart disease was not increased [35]. However, other studies have reported an increased risk of stroke and ischemic heart disease following splenectomy [39].

●PH may be due to chronic hemolysis for which splenectomy was performed. PH has been described following splenectomy for thalassemia, sickle cell disease, and hereditary spherocytosis (HS), and it was suggested that the risk may be related to ongoing hemolysis after the procedure [39]. (See "Clinical features and diagnosis of pulmonary hypertension of unclear etiology in adults", section on 'Group 5: PH due to multifactorial mechanisms'.)

Regardless of whether a causal relationship exists, these observations emphasize the importance of age-appropriate routine screening and evaluation of symptoms that suggest a cardiovascular condition [45].

Splenosis — Iatrogenic rupture of the spleen during splenectomy can cause subsequent implantation of splenic tissue within the peritoneal cavity, also referred to as splenosis [1]. This generally does not require any intervention, but it could cause abdominal pain, partial return of splenic function, or other complications [46-49]. (See "Splenomegaly and other splenic disorders in adults", section on 'Terminology' and "Evaluation of the adult with abdominal pain".)

Cancer — It is not clear whether splenectomy increases the risk of cancer, and we generally advise patients to follow age-appropriate screening practices.

Some series have reported an increased risk of cancer following splenectomy, including splenectomy following splenic trauma, for which an underlying increased cancer risk is unlikely to apply [35,50,51]. However, other series have failed to find such an association [52-54]. The tumor types do not appear to fit a pattern and include a number of adenocarcinomas, squamous cell carcinomas, hematologic malignancies, and others [35,50].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Infections in asplenic patients" and "Society guideline links: Anemia in adults".)

SUMMARY AND RECOMMENDATIONS

●Indications – Splenectomy can treat conditions with hemolysis or thrombocytopenia mediated by autoantibodies or splenic reticuloendothelial function, disorders with massive splenomegaly, and hypersplenism with cytopenias. Specific indications, summarized in the table (table 1), include hereditary spherocytosis, transfusion-dependent thalassemia, immune thrombocytopenia, autoimmune hemolytic anemia, splenic marginal zone lymphoma, splenic abscess, splenic or portal vein thrombosis with bleeding varices, ovarian cancer debulking, and total pancreatectomy. (See 'Indications' above.)

●General contraindications – Splenectomy is no longer used for staging Hodgkin disease and is generally avoided in autoimmune lymphoproliferative syndrome, hereditary stomatocytosis or xerocytosis, cold agglutinin disease, paroxysmal cold hemoglobinuria, Gaucher disease, and hypersplenism from cirrhosis. (See 'Conditions in which splenectomy is generally contraindicated' above.)

●Preoperative planning – Before splenectomy, the risks and benefits of alternative therapies must be considered. Laboratory testing includes complete blood count (CBC) with platelet count, differential, and coagulation studies (prothrombin time [PT] and activated partial thromboplastin time [aPTT]). Additional testing for occult lymphoma or glycogen storage disease may be indicated depending on the clinical setting. (See 'Preoperative considerations' above.)

●Vaccinations – Vaccinations prior to splenectomy are crucial, because the spleen is the principal site responsible for filtering bacteria from the bloodstream and producing mature B-cells (table 2). Influenza vaccine, COVID-19 vaccine, and other vaccines for which the individual is due should also be administered. The ideal timing is 10 to 12 weeks before the procedure to allow the full series, but shorter timelines are effective. (See 'Vaccinations' above and "Prevention of infection in patients with impaired splenic function", section on 'Vaccinations'.)

●Other preoperative interventions – Medical interventions to raise the hemoglobin or platelet count may be needed. (See 'Optimizing hemoglobin and platelet count' above and 'Other medication management' above.)

●Surgical technique – In most cases, we suggest a laparoscopic rather than open laparotomy approach (Grade 2C). This is expected to have lower mortality, shorter hospital stay, and fewer surgical complications. However, there may be reasons to perform an open splenectomy in some cases (massive splenomegaly, need to explore for an accessory spleen, cancer surgery, insufficient surgical expertise, lack of support for laparoscopy). Other considerations include use of partial splenectomy for children <6 years, simultaneous cholecystectomy for individuals with pigment gallstones from hemolysis, and identification and removal of an accessory spleen(s). (See 'Surgical approach' above.)

●Complications – Operative mortality is declining but remains a concern. Perioperative complications include bleeding, thrombosis, and infections. Late complications include a lifetime increased risk of infection and sepsis, especially with encapsulated organisms; venous thromboembolism; and possibly thrombocytosis. Risks that are less well established include cardiovascular disorders, recurrence of primary disease, cancer, and possibly pulmonary arterial hypertension. (See 'Postoperative risks' above.)

●Splenic disorders – Evaluation of unexplained splenomegaly and splenic lesions is presented separately. (See "Approach to the child with an enlarged spleen" and "Splenomegaly and other splenic disorders in adults".)

●Traumatic splenectomy – Traumatic splenectomy and the treatment of infections in asplenic individuals are discussed separately. (See "Surgical management of splenic injury in the adult trauma patient" and "Evaluation and management of fever in children and adults with sickle cell disease" and "Clinical features, evaluation, and management of fever in patients with impaired splenic function".)

ACKNOWLEDGMENT — UpToDate gratefully acknowledges Stanley L Schrier, MD (deceased), who contributed as Section Editor on earlier versions of this topic and was a founding Editor-in-Chief for UpToDate in Hematology.

The UpToDate editorial staff also acknowledges the extensive contributions of William C Mentzer, MD, to earlier versions of this and many other topic reviews.