Traps and destroys intracellular parasites via phagocytosis.
Executes early antibody production following intravenous antigen exposure.
Mediates pathogenesis of immune-mediated cytopenias via macrophage phagocytosis of antibody-coated cells.
Hematopoiesis
Functions as major site of red pulp hematopoiesis during 3-6 months of fetal life.
Ceases hematopoiesis postnatally.
Resumes extramedullary hematopoiesis in severe hemolytic anemia or myelofibrosis.
Indications For Splenectomy
Hematological Disorders
Red Blood Cell Membrane Defects
Hereditary Spherocytosis (HS):
Curative in most patients; eradicates hemolysis, anemia, and hyperbilirubinemia.
Indicated for severe HS (transfusion-dependent).
Strongly considered for moderate HS exhibiting frequent hypoplastic/aplastic crises, poor growth, or cardiomegaly.
Generally avoided in mild HS.
Postponed until after 6 years of age to minimize postsplenectomy sepsis risk.
Hereditary Elliptocytosis (HE): Considered for chronic HE and hereditary pyropoikilocytosis requiring transfusions.
Red Blood Cell Enzyme Defects
Pyruvate Kinase Deficiency: Decreases transfusion requirements but does not arrest hemolysis. Triggers paradoxical reticulocytosis post-surgery.
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency: Splenectomy rarely ameliorates severe anemia; reserved exclusively for severe hypersplenism or physical impediment from massive splenomegaly.
Immune-Mediated Cytopenias
Immune Thrombocytopenic Purpura (ITP):
Indicated for older children (≥4 years) with chronic ITP (>1 year duration) refractory to medical therapy.
Indicated for acute, life-threatening hemorrhage (e.g., intracranial hemorrhage) unresponsive to rapid platelet transfusion, intravenous immunoglobulin (IVIG), and corticosteroids.
Induces complete remission in 64-88% of chronic pediatric cases.
Warm Autoimmune Hemolytic Anemia (wAIHA):
Indicated if hemolysis remains brisk despite high-dose corticosteroids, rituximab, and transfusions.
Recommended for children >5 years of age with disease duration >6-12 months. Beneficial in 60-75% of patients.
Hemoglobinopathies
Thalassemia Major/Intermedia:
Indicated for secondary hypersplenism causing falling steady-state hemoglobin or rising transfusion requirements.
Criteria include annual packed RBC requirement >200-250 mL/kg/year with uncontrolled iron overload.
Indicated for symptomatic massive splenomegaly posing rupture risk or causing severe leukopenia/thrombocytopenia.
Sickle Cell Disease:
Prophylactic splenectomy indicated for recurrent, life-threatening acute splenic sequestration crises.
Prevents fatal hypovolemic shock associated with recurrent trapping of RBCs.
Non-Hematological And Structural Disorders
Splenic Trauma
Indicated for massive abdominal bleeding and clinical instability.
Indicated when conservative, non-operative management fails.
Partial splenectomy or splenic repair preferred to preserve immune function.
Splenic Malignancies And Lesions
Primary splenic tumors (e.g., Splenic Marginal Zone Lymphoma).
Symptomatic splenic cysts, pseudocysts, or abscesses.
Category
Specific Indications
Hemolytic Anemia
Severe HS, Symptomatic HE, Pyruvate kinase deficiency
Immune Cytopenias
Refractory chronic ITP (>1 yr), Refractory wAIHA
Hemoglobinopathies
Thalassemia with PRBC need >200-250 mL/kg/yr, Sickle cell with recurrent sequestration
Trauma
Hemodynamically unstable splenic rupture
Conditions Contraindicating Splenectomy
Autoimmune Lymphoproliferative Syndrome (ALPS): Extremely high risk of fatal sepsis despite prophylaxis.
Hereditary Stomatocytosis and Hereditary Xerocytosis: High risk of severe venous thromboembolic complications post-surgery.
Paroxysmal Cold Hemoglobinuria and Cold Agglutinin Disease.
Hepatic cirrhosis with thrombocytopenia.
Complications Of Splenectomy
Overwhelming Post-Splenectomy Infection (OPSI)
Represents most severe, life-threatening long-term risk.
Highest risk in children <5 years of age at time of surgery.
Overall risk: 2-5 per 1000 asplenic patient-years; lifelong risk of 5%.
Over 50% of OPSI episodes occur within first 2 years post-splenectomy.
Risk magnitude varies by underlying disease: 2-4% in trauma or ITP; 8-30% in thalassemia, sickle cell disease, or preexisting reticuloendothelial blockade.
Pathogens: Encapsulated bacteria dominate. Streptococcus pneumoniae (>60% of cases), Haemophilus influenzae, and Neisseria meningitidis.
Animal Bites: High risk for fulminant sepsis from Capnocytophaga canimorsus or C. cynodegmi following dog bites/licks.
Protozoal Infections: Increased susceptibility to severe malaria and babesiosis.
Clinical Course: Rapid progression to fulminant sepsis and meningitis; death frequently ensues within 12-24 hours of onset.
Thromboembolic Complications
Increased risk of arterial and venous thrombosis.
Attributed to loss of splenic filtering function, permitting abnormal RBCs to circulate and activate coagulation cascades.
Post-operative thrombocytosis common; usually resolves spontaneously but contributes to transient hypercoagulability.
Portal vein thrombosis specifically reported as complication following laparoscopic splenectomy.
Pulmonary Hypertension
Late complication increasingly recognized post-splenectomy.
Particularly prevalent when splenectomy performed for chronic hemolytic conditions (Thalassemia, Sickle Cell Disease, Hereditary Spherocytosis).
Pathogenesis linked to ongoing chronic hemolysis and circulation of procoagulant erythrocyte microparticles previously cleared by spleen.
Surgical And Perioperative Complications
Splenectomy failure/relapse: Due to un-resected accessory spleens or accidental autotransplantation of splenic tissue (splenosis) during surgery.
General surgical risks: Hemorrhage, adjacent organ injury (pancreatic tail), anesthesia complications.
Post-Operative Management And Preventive Care
Principles Of Splenic Preservation
Defer total splenectomy until patient reaches ≥5 years of age whenever possible to mitigate OPSI risk.
Utilize laparoscopic approach to decrease surgical morbidity and hospitalization duration.
Consider partial or subtotal splenectomy (removing 85-95% of volume): Decreases hemolytic rate while preserving residual phagocytic immune function.
Immunization Protocol
Administer vaccines at least 14 days prior to elective splenectomy.
Pneumococcal Vaccines: 13-valent pneumococcal conjugate vaccine (PCV13) followed by 23-valent pneumococcal polysaccharide vaccine (PPSV23) at age ≥2 years. Second dose of PPSV23 administered 5 years later.
Meningococcal Vaccine: Administer protein-conjugated formulations.
Haemophilus influenzae type b (Hib) Vaccine: Ensure up-to-date status.
Influenza Vaccine: Administer yearly; prevents viral illness that predisposes to secondary pneumococcal infections.
Antimicrobial Prophylaxis
Essential component of post-splenectomy management to prevent fatal sepsis.
Agent: Oral Penicillin VK.
Dosing:
Children <5 years: 125 mg twice daily.
Children ≥5 years: 250 mg twice daily.
Duration: Continue until at least 5 years of age, and strictly for a minimum of 2 years post-splenectomy.
Lifelong Prophylaxis: Strongly considered for high-risk patients (e.g., Sickle Cell Disease, history of invasive pneumococcal infection, underlying immune deficiency).
Acute Fever Management
Febrile episodes in splenectomized patients constitute medical emergencies.
Empiric Home Therapy: Initiate amoxicillin-clavulanate or cefdinir immediately if access to medical care is delayed.
Hospital Management: Prompt blood cultures followed by immediate administration of broad-spectrum intravenous cephalosporin (cefotaxime or ceftriaxone).
Resistant Organisms: Add Vancomycin empirically to cover penicillin-resistant pneumococci based on illness severity and local susceptibility patterns.
Additional Preventative Measures
Animal Bites: Administer prophylactic antibiotics immediately after dog bites/licks to prevent Capnocytophaga sepsis.
Travel Counseling: Advise against travel to malaria or babesiosis endemic areas without stringent prophylaxis and mosquito avoidance.
Medical Alert: Patient must wear a medical alert bracelet denoting asplenic status.
Surveillance: Monitor for thromboembolic disease and pulmonary hypertension via clinical evaluation and echocardiography.