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Splenectomy for Conditions Other Than Trauma
Splenectomy for nontraumatic disorders demands careful risk-benefit analysis and surgical planning. Crucial factors considered include the nature of the underlying disease, the severity of symptoms, alternative therapeutic options, the operative risk, and the success rate of splenectomy. During the past decade, the underlying diseases have become better understood; more and effective medical therapies have become available, specifically immunomodulatory/immunosuppressive regimens; laparoscopic techniques have expanded and decreased operative risks; and prophylaxis has minimized the risk of postsplenectomy infections. These advances have challenged some of the traditional concepts regarding splenectomy. This chapter aims to summarize the current indications and contemporary outcomes of splenectomy for nontraumatic conditions encountered by surgeons in consultation. These conditions mainly include hematologic disorders but also splenic mass lesions, splenic vascular disease, iatrogenic injuries, and other rare diseases.
Splenectomy for Hematologic Disorders
The spleen performs important hematologic and immunologic functions. It maintains the circulating blood components by filtering and removing damaged or senescent cells. As the largest aggregate of lymphoid tissue in the reticuloendothelial system, the spleen functions in both antibody production and phagocytosis. Accordingly, cytopenia and splenomegaly are two common manifestations of hematologic disorders involving the spleen. Cytopenia is associated with hypersplenism, the excessive destruction of one or more blood components. Splenomegaly, defined as splenic weight of more than 175 g (normal, 90 to 150 g), can become massive (>1000 to 15,000 g). Mechanical symptoms of splenomegaly include pain and early satiety. When the spleen is the sole site of the disease or a major contributor to the underlying pathophysiology, splenectomy is performed with curative intent. In most conditions, it is performed for effective palliation of symptoms and complications in patients refractory to medical management. In general, splenectomy for hematologic disease can be diagnostic, therapeutic, curative, or palliative and is performed for specific clinical indications rather than for specific diagnoses.
Disorders Causing Thrombocytopenia
Thrombocytopenia is defined as platelet count less than 150 × 10 9 /L. Patients with platelet counts of 50 × 10 9 /L or greater are usually asymptomatic and are discovered incidentally. Excessive oozing after surgery or bruising after minor trauma usually does not occur until the platelet count is less than 30 × 10 9 to 50 × 10 9 /L. Spontaneous internal bleeding may occur with platelet counts of 10 × 10 9 to 20 × 10 9 /L. Response of thrombocytopenia to therapy has been variably defined in previous studies. Complete response (CR) is most commonly defined as achieving platelet counts of 150 × 10 9 /L for at least 30 days after splenectomy without additional therapy. Partial response (PR) results when platelet counts of at least 50 × 10 9 /L are achieved, whereas no response (NR) is defined when counts remain less than 50 × 10 9 /L for 30 days. Relapse occurs when thrombocytopenia recurs after achieving a normal platelet count.
Idiopathic Thrombocytopenic Purpura
Idiopathic thrombocytopenic purpura (ITP), also called primary immune thrombocytopenia (PIT), is the most common hematologic disease for which splenectomy is indicated. Affected patients may be asymptomatic or may present with petechiae, ecchymosis, epistaxis, gastrointestinal bleeding, or menorrhagia. Subarachnoid or intracranial hemorrhage suggests severe thrombocytopenia. ITP is mediated by autoantibodies, typically against multiple platelet membrane glycoproteins such as IIb/IIIa, Ib/Ix, Ia/IIa, IV, and V. Splenic macrophages clear platelets coated with immunoglobulin G (IgG) autoantibodies in an accelerated fashion. If compensatory platelet production is impaired or outstripped, thrombocytopenia ensues. The test for antiplatelet antibodies has a sensitivity of only 49% to 66% and a specificity of 78% to 92%. A positive test does not definitively diagnose ITP, whereas a negative result cannot exclude it. ITP remains a clinical diagnosis of exclusion. A search for a secondary cause for thrombocytopenia should be prompted by a history of drug or toxin exposure, recent viral infections, splenomegaly on physical examination, an abnormal peripheral smear, or a hypoplastic bone marrow. Although a peripheral blood smear has been required as a diagnostic test, bone marrow aspiration is considered for patients older than age 60 years with atypical presentations and in whom other disorders are suspected and splenectomy is contemplated.
The time of disease onset in childhood or adulthood determines the clinical presentation, natural history, and treatment approaches. Childhood ITP most commonly affects children between 2 and 5 years of age without a gender bias. In approximately 90% of the patients the disease manifests as acute thrombocytopenia, associated with a sudden onset of petechiae occurring 4 to 8 weeks after the prodrome of viral illness, allergies, or immunizations. Antibodies formed during the preceding illnesses cross-react against platelets. The natural history of childhood ITP is favorable; a vast majority (83%) spontaneously recover within 8 weeks without therapy, with approximately 10% to 15% persisting as chronic ITP. Therefore aggressive therapy is avoided. Typical management includes observation and avoidance of platelet-inhibiting medications and of activities predisposing to trauma. The decision to initiate any form of therapy is typically driven by a concern for the risk of intracranial hemorrhage, the development of refractory clinical symptoms, and activity restrictions that compromise a child's quality of life. First-line therapy is medical and includes intravenous immunoglobulin (IVIG), corticosteroids, anti-IgD, and platelet transfusion. Splenectomy is delayed for as long as possible. However, when it is performed, response rates of 63% to 86% may be expected. The response is sustained in the long term in 45% to 60% of the patients. Benefit from splenectomy may be predicted by preoperative response to IVIG, with positive predictive values of 74% to 91% and negative predictive values of 75% to 100%. In the pediatric population, laparoscopic splenectomy does not compromise the response rates, can be safely performed, and allows faster recovery without increasing costs.
Adult ITP has an insidious onset and affects women between 18 and 40 years of age most commonly. The natural history contrasts with that of childhood ITP, in that spontaneous remission occurs in only 2% to 9% of all patients. Most patients will develop chronic ITP. Although the disease course is usually benign, those with severe or refractory thrombocytopenia face four times the risk of mortality than the general population. Initiation of therapy depends on the bleeding risk, estimated from patient's age, lifestyle, platelet count, and concomitant diseases. The therapeutic approach to ITP in adults has undergone dramatic change over the past decade. The standard first-line therapies now include corticosteroids, IVIG, and anti-IgD. Recent studies in patients undergoing treatment with dexamethasone regimens have demonstrated up to 86% response rate, with 50% to 74% sustained response. Multiple additional medical therapies are considered for second- and even third-line therapies, including rituximab, danazol, dapsone, azathioprine, cyclosporin A, cyclophosphamide, mycophenolate, and thrombopoietin (TPO) receptor agonists. Splenectomy is considered a second-line treatment in the most current international consensus guidelines but is also the most likely curative therapy for ITP. Outcomes of splenectomy for ITP have been summarized in a systematic review by Kojouri et al. reporting on 130 articles. The overall rate of platelet response to splenectomy is 67% (range, 37% to 100%), with a sustained response rate of 64% after 7 years (range, 5 to 12.75 years) of follow-up. The average relapse rate after splenectomy is 15% (range, 0% to 51%), most occurring within the first postoperative year. One single-center experience of 140 adults revealed an overall complete platelet response rate of 78% initially and 74% after 1 year. Corticosteroids, danazol, and/or IVIG salvaged 81% of those who relapsed. Factors predictive of successful outcome after splenectomy have also been investigated. Younger age (<30 years) at splenectomy and previous response to glucocorticoids most consistently correlated with good response. In addition, when platelets are mainly sequestered in the spleen rather than the liver and other lymphoid organs, as identified by indium-labeled platelet scans, a superior response rate has been observed; however, this modality is not currently widely available in the United States and requires a sufficient level of circulating platelets to be harvested, radiolabeled ex vivo, and subsequently reinfused back into the patient.
Laparoscopic splenectomy has become the criterion standard of care for ITP patients. Operative mortality has decreased from 1% for open splenectomy to 0.2% for laparoscopic splenectomy. Similarly, operative morbidity has decreased from 12.9% to 9.6%. Postoperative recovery is superior, with less pain and earlier hospital discharge. These benefits are realized without increased cost and without compromising hematologic response rates. In debilitated patients who are unsuitable for an operation, splenic irradiation or partial splenic embolization may be considered, but the experience with this treatment is limited.
Accessory splenic tissue may be present in 16% to 29% of patients with ITP. The most common locations for accessory splenic tissue include the splenic hilum, the gastrosplenic ligament, gastrocolic ligament, greater omentum, mesentery, and presacral space ( Fig. 141.1 ). A thorough search should be conducted intraoperatively whether the operative approach is open or laparoscopic because a missed accessory spleen may be the cause for relapse of ITP. The presence of residual functioning splenic tissue after splenectomy is indicated by the absence of Howell-Jolly bodies on a peripheral smear.
ITP occurs in every 1 to 2 per 1000 pregnancies, with or without a preexisting diagnosis. Differential diagnosis should exclude hereditary thrombocytopenia, gestational thrombocytopenia, and syndrome of hemolysis with elevated liver enzymes and low platelets. In pregnant ITP patients, bleeding risks for both the mother and the fetus must be considered because maternal IgG antibodies cross the placenta and can cause fetal thrombocytopenia. Treatment consists of careful monitoring of maternal platelet counts that typically reach a nadir in the third trimester. Intervention is generally not needed in patients with platelet counts greater than 20 × 10 9 /L until before delivery. A maternal count greater than 50 × 10 9 /L is considered safe for any mode of delivery and is the goal of therapy. Treatment options of low teratogenic risk include corticosteroids or IVIG, but their side effects may be exacerbated in pregnancy and should be carefully monitored. Splenectomy is usually avoided, but, if necessary, splenectomy should be performed during the second trimester. With maternal platelet count greater than 50 × 10 9 /L, the incidence of fetal thrombocytopenia is 10% to 15% and that of fetal hemorrhage is less than 1%.
Emergent intervention for ITP is indicated for patients with neurologic symptoms suggestive of intracranial bleeding, with evidence of internal or widespread mucocutaneous bleeding, and for those requiring an emergency operation for other reasons. First-line therapy consists of IVIG (1 g/kg per day for 2 days), intravenous methylprednisolone (1 g/day for 3 days), and platelet transfusions. Emergency splenectomy for refractory patients is rarely needed.
Thrombotic Thrombocytopenic Purpura
Unlike ITP, thrombotic thrombocytopenic purpura (TTP) can be a highly lethal disorder. TTP is characterized by the pentad of thrombocytopenia, hemolytic anemia, fever, renal dysfunction, and less commonly, neurologic impairment. Characteristic findings include peripheral schistocytes (fragmented erythrocytes) and evidence of microvascular thrombosis. The pathophysiology of TTP involves an undefined trigger of vascular endothelial injury, leading to the release of unusually large forms of the von Willebrand factor. Abnormal platelet agglutination and marked intrasplenic phagocytosis follow. Currently, the first-line therapy consists of total plasma exchange in conjunction with corticosteroids and antiplatelet drugs such as aspirin or dipyridamole. Total plasma exchange has revolutionized the care of TTP by increasing the previously dismal survival rate to approximately 70% to 85%. Relapse rates remain as high as 36% over 10 years. TTP refractory to standard or increasing volume/frequency of plasma exchange and/or corticosteroids is most often treated with rituximab and a comprehensive evaluation of secondary causes (sepsis, drugs, etc.). Single-agent rituximab has demonstrated clinical remission in 87% to 100% of patients. Splenectomy has been suggested for patients who remain refractory after escalating medical therapies. In several small series of patients, splenectomy induced remission of TTP in 50% of refractory patients and reduced the risk of relapse by 70% to 95%. However, the operative morbidity in this patient population may be substantial at 17% to 39%. Only recent reports have suggested that laparoscopic splenectomy has lowered these operative risks.
Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown cause. Antiplatelet antibodies are demonstrable in 78% of SLE patients. These pathogenic autoantibodies and immune complexes affect virtually every body system. Destruction of antibody-coated platelets leads to severe thrombocytopenia in 8% to 20% of these patients. First-line therapy involves agents aimed at reducing the pathogenic immune response: corticosteroids, rituximab, danazol, IVIG, and immunosuppressive (e.g., mycophenolate mofetil [CellCept]) and antineoplastic (e.g., cyclophosphamide, vincristine) drugs. Response rates to medical therapy have been variable and transient. Splenectomy is considered for patients who are refractory to dependent, or intolerant of medical therapy. Despite previous concerns the operative risks of splenectomy are acceptable. The largest recent single-center experience of 25 patients undergoing splenectomy reported a 30-day mortality of 0% and morbidity of 24%, with hemorrhage and infection the most common complications. The hematologic response was comparable to splenectomy for ITP, with an initial response rate of 88% and a relapse-free long-term response rate of 64%. Previously reported initial response rates ranged from 21% to 93% and prior sustained response rates were only 10% to 32%. Although 36% of the patients relapsed after initial response (consistent with previously reported rates of 6% to 79%), additional medical therapy successfully salvaged 55% of these patients. Because splenomegaly is typically not present, laparoscopic splenectomy is the procedure of choice in this patient population.
Human Immunodeficiency Virus
Chronic thrombocytopenia affects approximately 10% of patients infected with the human immunodeficiency virus (HIV) and 33% of those with acquired immunodeficiency syndrome (AIDS). Bleeding complications are infrequent and rarely severe even in the 1% to 5% of the patients with severe thrombocytopenia. Most patients have platelet counts higher than 50 × 10 9 /L; some may even spontaneously correct their thrombocytopenia. The pathogenesis of HIV-thrombocytopenia involves (1) immune-mediated platelet destruction, similar to that in ITP, and (2) impaired platelet production due to infected megakaryocytes in the bone marrow. Accordingly, first-line therapy consists of (1) corticosteroids, IVIG, and anti-D, similar to ITP, and (2) antiviral agents such as azidothymidine (AZT) or combination highly active antiretroviral therapy to treat the primary disease. The immunosuppressive effects of corticosteroids make them unsuitable for long-term administration. Splenectomy is indicated in patients unresponsive, refractory, or intolerant of medical therapy. Operative mortality is minimal, although the complication rate approaches 24%. Favorable response is achieved in 83% of HIV patients and slightly fewer AIDS patients. Splenectomy has not been shown to adversely impact the progression to AIDS, overall survival, and AIDS-free survival. Despite encouraging results, the timing and patient selection for splenectomy during the course of HIV infection remain controversial.
Wiskott-Aldrich Syndrome
Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency disorder characterized by thrombocytopenia, eczema, vasculitis, progressive immunodeficiency, and increased risk for malignancy. Its pathogenesis involves defective cytoplasmic scaffolding proteins. Although phenotypic expression varies, thrombocytopenia is the most common manifestation of WAS. For patients with severe symptoms and available human leukocyte antigen (HLA)-matched donors, bone marrow transplant is performed with curative intent. For symptomatic patients without appropriate donors, splenectomy is indicated in combination with prophylactic antibiotics and immunization. Median survival of up to 25 years has been reported, representing substantial improvement over the previously dismal median survival of less than 5 years. An IVIG may be used alone or in combination with splenectomy.
Disorders Causing Anemia
Hereditary Anemia
Hereditary anemias can be categorized by (1) defects of the erythrocyte membrane (e.g., hereditary spherocytosis [HS], hereditary elliptocytosis); (2) defects of an erythrocyte enzyme (e.g., pyruvate kinase deficiency, glucose-6-phosphate dehydrogenase deficiency); and (3) defects of hemoglobin synthesis (e.g., thalassemias, sickle cell anemia [SS]). All of these mutations result in abnormal erythrocyte morphology and stability and lead to increased hemolysis and phagocytosis by the spleen. The benefit and use of splenectomy vary depending on the diagnosis.
Red Blood Cell Membrane Defects
HS is the most common inherited hemolytic disorder in North America and Europe. It is transmitted mainly as an autosomal dominant trait. The pathogenesis of HS involves deficiencies in membrane structural proteins. The affected family of spectrin proteins, including β spectrin, ankyrin, band 3, and protein 4 to 2, normally forms the supportive cytoskeleton of the red blood cell (RBC). Dysfunction of these proteins results in abnormal RBC morphology, increased cell membrane fragility, and shortened life span. Clinical findings are variable and include anemia, jaundice, and splenomegaly. Pigmented gallstones form in up to 41% of patients screened with ultrasonography, and their prevalence is higher in patients who co-inherit Gilbert disease. HS is distinguished from other anemias by the findings of elevated reticulocyte counts, hyperbilirubinemia, negative direct antiglobulin test (DAT), spherocytes on peripheral smear, and increased erythrocyte osmotic fragility.
The indication for splenectomy is not based on the diagnosis of HS, per se, but on its symptoms and complications ( Table 141.1 ). For patients with mild HS and no gallstones, splenectomy has no benefit. For patients with moderate or severe disease, splenectomy is indicated but usually delayed until after the sixth year of life but before puberty to minimize the risk of postsplenectomy sepsis. Children with accelerating anemia, frequent hemolytic crises, transfusion dependency, or intractable leg ulcers may require earlier intervention. For patients with symptomatic cholelithiasis, laparoscopic splenectomy and cholecystectomy are indicated and can be performed safely together. When gallstones are asymptomatic or found incidentally, the best approach has not been established. Options include observation, cholecystotomy with stone removal, or cholecystectomy.
TABLE 141.1
Classification of Hereditary Spherocytosis
| Variable | Trait/Carrier | Mild | Moderate | Severe |
|---|---|---|---|---|
| Hemoglobin, g/dL | Normal | 11–15 | 8–12 | 6–8 |
| Reticulocyte, % | <3 | 3–6 | >6 | >10 |
| Bilirubin, µmol/L | <17 | 17–34 | >34 | >51 |
| Spectrin per RBC, % normal | 100 | 80–100 | 50–80 | 40–60 |
| Splenectomy | Not indicated | Usually not indicated | Consider before puberty | Usually necessary, delay until age 6 years if possible |
RBC , Red blood cell.
The optimal approach for splenectomy remains controversial. Laparoscopic splenectomy offers a faster postoperative recovery in the pediatric population. It should be the approach of choice when splenomegaly is not present to increase the operative risks. Partial (80% to 90%) open or laparoscopic splenectomy has been advocated for very young patients with severe disease, but preservation of splenic function must be balanced against the risks of disease recurrence. Recently, near-total splenectomy (98%) has been proposed as a means to optimize this balance. Partial splenectomy has been demonstrated to be effective in resolution of hematologic parameters in the short term; however, total splenectomy has demonstrated more vigorous responses in hematologic parameters. There have been reports of increased vascular events (arterial and venous) in patients with HS undergoing splenectomy, thought to be due to protective effects of lower hemoglobin and/or cholesterol metabolism in nonsplenectomized HS patients; thus close monitoring or postoperative prophylaxis should be considered.
Hereditary elliptocytosis is a variant of HS also involving defective spectrin proteins. These patients typically have mild anemia requiring no intervention. Splenectomy does not correct the abnormal RBC morphology but is effective for the rare patient with severe transfusion-dependent anemia. HS must also be differentiated from other rare disorders of RBC membrane permeability, such as hereditary stomatocytosis or cryohydrocytosis. Splenectomy is ineffective and unwarranted and carries a high risk of postsplenectomy venous thrombosis in these patients.
Red Blood Cell Enzymatic Defects
Glucose-6-phosphate dehydrogenase deficiency is the most common RBC enzymatic defect. It manifests as a mild anemia and rarely splenomegaly. Experience with splenectomy in this disease is limited. Pyruvate kinase deficiency results in reduced energy generation in RBCs. The homozygous form of this disease results in a severe anemia with splenomegaly. Splenectomy is effective in reducing transfusion requirements.
Hemoglobinopathies
Sickle cell disease includes SS, hemoglobin C disease (SC), and the sickle β-thalassemia. The inherited point mutation on the sickle gene leads to an abnormal β-chain forming a hemoglobin with decreased solubility in its deoxygenated form. Pathogenesis of sickle disease results from abnormal polymerization of hemoglobin S with low cellular oxygen content. Exponential propagation of this process stiffens and distorts erythrocytes. Further compounding factors include abnormal endothelial adhesion, formation of heterocellular aggregates, dysregulation of nitric oxide–mediated vasodilation, and local inflammation. All of these factors lead to slowed RBC transit and their entrapment in the vasculature and in the spleen. Microvascular occlusion results, and sickle patients suffer from end-organ damage of the eyes, kidneys, subcutaneous tissue, and bone. Splenic sequestration occurs when the RBC is trapped in the enlarged spleen, which then undergoes autoinfarction; it is observed in 7% to 30% of SS patients between 2 and 5 years of life. Acute manifestation, known as acute splenic sequestration crisis (ASSC), is potentially fatal. Patients present with profound acute anemia (decrease in hemoglobin by >2 g/dL), reticulocytosis, and thrombocytopenia. Acute therapy requires resuscitation by RBC transfusions. However, recurrence carries a 20% mortality rate and can occur in 50% of those who survive ASSC. As a means to prevent future ASSC, elective splenectomy has been indicated in children older than 2 or 3 years of age after the first episode of ASSC. The operative mortality is 7%, and 5-year mortality is 3.4%. The risk of postsplenectomy sepsis is approximately 2% in this patient population but increases substantially if splenectomy is performed before 4 years of age. More recently partial splenectomy has been compared with total splenectomy in pediatric patients and has demonstrated no change in postsplenectomy hemoglobin levels in either group, underscoring the decision-making process for splenectomy which focuses on minimizing complications and improvement of quality of life. Importantly in this multiinstitutional observational study, no difference was found in the rate of postsplenectomy sepsis comparing partial to total splenectomy. Splenectomy (partial or total) has not been proven to increase survival, but the benefits include reducing transfusion dependency, relief from pain from splenomegaly, and treatment of splenic abscesses resulting from splenic infarctions.
Patients with thalassemia major (or homozygous β-thalassemia) synthesize structurally abnormal hemoglobin that deforms erythrocytes. They typically depend on multiple transfusions to maintain a hemoglobin level greater than 10 g/dL. When complications of hypersplenism develop, as measured by transfusion requirement of greater than 250 mL/kg per year and iron overload, splenectomy is indicated. Splenectomy reduces the requirements for both transfusions and deferoxamine (an iron chelator) in 32% of patients. More than 80% of children with thalassemia regain normal weight and growth rates after splenectomy. The risk for overwhelming postsplenectomy sepsis (OPSS) is high in this patient population, approximately 10% in the long term. Therefore splenectomy is usually delayed until after 6 to 8 years of age. Partial splenectomy has been advocated in younger children, and laparoscopic splenectomy is definitely feasible in these patients.
Acquired Hemolytic Anemia
Hemolytic anemia may result from numerous etiologies. Autoimmune hemolytic anemia (AIHA) is an IgG-mediated (so-called warm agglutinin) hemolytic anemia with a positive Coombs antiglobulin test. Erythrocyte destruction is mediated by splenic macrophages. AIHA may be idiopathic or a manifestation of a systemic disease, such as viral infection, SLE, rheumatoid arthritis, ulcerative colitis, or chronic lymphocytic leukemia (CLL). Although corticosteroids remain the first-line therapy, rituximab is favored as the initial choice of second-line therapies despite the absences of randomized data supporting this approach. Splenectomy is indicated for disease refractory to corticosteroids (or other second-line therapies). It succeeds in nearly 64% of patients and reduces the steroid requirement in an additional 21% of patients. The success rate is greater when AIHA is associated with systemic disease. It is important for slow steroid taper postsplenectomy because rapid withdrawal can lead to acute hemolytic crises, which may mimic postoperative bleeding. In contrast, so-called cold agglutinin hemolytic anemia is mediated by IgM. Erythrocytes are sequestered and destroyed in the liver, and splenectomy therefore plays no role in this condition.
Miscellaneous Hematologic Disorders
Evans Syndrome
Patients with Evans syndrome present with a combination of autoimmune thrombocytopenia (ITP) and AIHA. Medical therapy typically involves multiple agents, with corticosteroids and IVIG being used most commonly. Experience with splenectomy for this rare disease is limited. Although long-term remission has been reported, one study observed the median duration of response following splenectomy to be only 1 month.
Felty Syndrome
Felty syndrome, defined as a combination of rheumatoid arthritis, splenomegaly, and neutropenia, affects a small subset of patients, particularly those with destructive rheumatoid arthritis, severe extraarticular symptoms, and an HLA-DR4 haplotype. Neutropenic sepsis is the main cause of patient demise. First-line therapy consists of hematopoietic growth factors and often leads to rapid, favorable responses. Splenectomy is indicated when the neutropenia fails to improve adequately or rapidly enough. Neutropenia is corrected by splenectomy in 80% of patients, and active preoperative infections resolve in nearly half of patients.
Autoimmune Neutropenia
Patients affected by autoimmune neutropenia, a rare disorder, usually have neutrophil counts of 500 to 1000/µL but manifest granulocyte-specific antibodies. It commonly presents in infancy as recurrent infections. When present in adults, it may be associated with underlying diseases such as viral infection, collagen vascular diseases, ITP, or AIHA. Autoimmune neutropenia is typically characterized by spontaneous disappearance of autoantibodies and does not require specific intervention. However, for acute infections or operative procedures, granulocyte colony-stimulating factors effectively improve the neutrophil counts. A total of 50% to 60% of the patients also respond to corticosteroids and IVIG. Therefore the role for splenectomy is limited only to the rare patient who is refractory to medical interventions.
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