eMedicine Specialties > Pediatrics: General Medicine > Hematology
Splenomegaly: Treatment & Medication
Updated: Sep 4, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Because splenomegaly is usually the result of a systemic disease, the primary goal is treatment of the underlying process. In certain circumstances, splenectomy may be the therapy of choice for symptoms or complications caused by the enlarged organ. However, because of the risk of overwhelming sepsis in children who are asplenic, the risks and benefits must be carefully weighed when splenectomy is being considered.10,11
- The new PCV7 conjugated pneumococcal vaccine and the older PPV23 polyvalent pneumococcal vaccines and the Haemophilus influenzae vaccine should be administered to all children who are asplenic and to those about to undergo splenectomy. The polyvalent pneumococcal vaccine is effective only if the patient is aged 2 years or older. Meningococcal vaccine, MCV4, is often administered to these patients in this age group as well. To maximize antibody formation, vaccines should be administered at least 10 days before splenectomy.23
- Daily penicillin is recommended to prevent pneumococcal septicemia in asplenic children younger than 5 years. Antibiotic prophylaxis is often administered for several years after splenectomy in patients older than 5 years, but the role of prophylaxis in these children is less well documented than it is in others.
- In patients with homozygous sickle cell anemia or sickle beta-zero thalassemia, oral penicillin prophylaxis should be started when the diagnosis is established. This therapy should be administered until at least age 5 years. The role of penicillin prophylaxis in patients with hemoglobin sickle cell disease is controversial. Patients with sickle beta+ thalassemia do not appear to need penicillin prophylaxis.
- Febrile illnesses in asplenic children should be approached as potentially life-threatening events and evaluated thoroughly, with a low threshold for treatment with intravenous antibiotics that cover Streptococcus pneumoniae and H influenzae. The increasing prevalence of drug-resistant S pneumoniae and the decreasing incidence of H influenzae infections are complicating factors in determining the optimal choice of antibiotics.
Surgical Care
Splenic trauma is the most common indication for splenectomy, although attempts at splenic preservation are increasingly important. Nonsurgical management for splenic trauma has success rates of 52-98%, with failure usually occurring in the first 96 hours. Splenic cysts, tumors, and vascular lesions may also require surgical removal. Whenever possible, splenic tissue is preserved to decrease the risk of septicemia, but total splenectomy is occasionally necessary.24,25
- Splenectomy can cure hypersplenism but is not usually indicated because the secondary cytopenias rarely cause serious problems. However, in patients with portal hypertension, vascular shunts may be necessary to prevent esophageal variceal bleeding.
- Splenectomy may be helpful in improving cytopenias in several medical conditions, including congenital anemias (eg, hereditary spherocytosis, elliptocytosis) and autoimmune disorders (eg, immune thrombocytopenic purpura, autoimmune hemolytic anemia, hypersplenism). In thalassemia major, splenectomy may initially decrease the transfusion requirements caused by hypersplenism. However, the benefit must be carefully weighed against the risk of sepsis.
- In Gaucher disease, splenectomy may be necessary when the mechanical strain of the enlarged spleen requires intervention.
- Splenectomy may be indicated in children with sickle cell anemia and a history of splenic sequestration crisis in order to prevent recurrences of the crisis.
- As part of exploratory laparotomy, splenectomy was once an important component of staging of Hodgkin disease. This procedure is rarely used because of improvements in imaging modalities, the high risk of postsplenectomy sepsis, and the increased use of chemotherapy in patients, which allows treatment decisions to be made on the basis of radiologic evaluation alone. Furthermore, data suggest that splenectomy increases the risk of second malignancy in patients treated for Hodgkin disease.
Consultations
A pediatric hematologist and/or oncologist is the usual consultant when the cause of splenomegaly is not obvious or when a primary hematologic or oncologic disorder is suspected.
Activity
- According to the American Academy of Pediatrics and the Council of Sports Medicine and Fitness, children with acutely enlarged spleens should avoid contact, collision, or limited-contact sports.23 Viral-related splenomegaly rarely lasts longer than 2 months.
- The duration for which contact restriction should persist after acute infectious mononucleosis remains uncertain. In the absence of trauma the incidence of splenic rupture is approximately 1:1000, usually occurring in the first 3 weeks of illness. No evidence-based guidelines are available. More conservative suggestions recommend that children with infectious mononucleosis and resolution of splenomegaly noted on examination have 3 weeks of rest with a 4-week graded return to activity. However, it has also been suggested that contact sports can resume as soon as 4 weeks after onset of illness.26
- Even with large spleens, disorders associated with chronic splenomegaly have reduced splenic friability with minimal risk of rupture. In children with chronically enlarged spleens, decision to restrict activity should be made on a case-by-case basis.27
Medication
The choice of therapy depends on the specific etiology of the splenomegaly.
Vaccines
Active immunization increases resistance to infection. Vaccines consist of microorganisms or cellular components, which act as antigens. Administration of the vaccine stimulates the production of antibodies with specific protective properties.
With the increased problem of penicillin resistance in S pneumoniae, prevention by using the conjugated pneumococcal vaccine in children or by using the unconjugated 23-valent pneumococcal vaccine in adults is mandatory. Likewise, immunizations with the conjugated H influenzae type B and meningococcal A and C vaccines are essential. Vaccines are administered at least 10 days before splenectomy.
Pneumococcal 7-valent conjugate vaccine (Prevnar)
Sterile solution of saccharides of capsular antigens of S pneumoniae serotypes 4, 6B, 9V, 14, 18C, 19F, and 23F individually conjugated to diphtheria CRM197 protein. These 7 serotypes responsible for >80% of invasive pneumococcal disease in children <6 y in the United States and account for 74% of penicillin-nonsusceptible S pneumoniae (PNSP) and 100% of pneumococci with high-level penicillin resistance.
Customary age for first dose is 2 mo, but can be administered as young as 6 wk. Preferred sites for IM injection are anterolateral aspect of thigh in infants or deltoid muscle of upper arm in toddlers and young children. Do not inject in gluteal area or areas with a major nerve trunk or blood vessel.
Number of 0.5-mL doses is 3 for infants aged 7-11 mo (4 wk apart; third dose after first birthday), 2 for those aged 12-23 mo (2 mo apart), and 1 for those aged 2-5 y.
Minor illnesses (eg, mild upper respiratory tract infection with or without low-grade fever) are not generally contraindications.
Adult
Not established
Pediatric
0.5 mL IM; 3 doses q2mo; fourth dose of 0.5 mL at 12-15 mo of age, or at 2 mo or later after third dose; recommended dosing interval is 4-8 wk
Immunosuppressive agents (immunosuppressive doses of corticosteroids, antimetabolites, alkylating agents, cytotoxic agents) may decrease effects; may increase effects of anticoagulant therapy; globulin preparations may interfere with immune response and reduce efficacy (do not administer within 3 mo of vaccination)
Documented hypersensitivity to any component or diphtheria toxoid; severe or moderate febrile illness; thrombocytopenia or coagulation disorder contraindicating IM injection (unless benefits outweigh risks)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
For IM use only, do not administer IV under any circumstances; take special care to prevent injection into or near blood vessel or nerve; caution in patients with possible history of latex sensitivity (packaging contains dry natural rubber); does not replace 23-valent pneumococcal polysaccharide vaccination in children >24 mo of age with sickle cell disease, asplenia, HIV infection, chronic illness, or those who are immunocompromised; caution in coagulation disorders
Pneumococcal vaccine (Pneumovax-23)
Polyvalent vaccine used for prophylaxis against infection from S pneumoniae. Used in populations at increased risk of pneumococcal pneumonia (ie, >55 y, chronic infection, asplenia, immunocompromise).
Adult
0.5 mL IM/SC once
Pediatric
<2 years: Contraindicated (antibody response poor in this age group)
>2 years: 0.5 mL IM/SC; repeat dose after 3-5 y in high-risk children (eg, those with functional or anatomic asplenia or conditions associated with rapid antibody decline after initial vaccination)
Immunosuppressive agents (eg, large amounts of corticosteroids, antimetabolites, alkylating agents, cytotoxic agents) may reduce effectiveness; therapy with immunoglobulin preparations likely to block active immunity induced; withhold for 3 mo after discontinuation of immunoglobulin therapy
Documented hypersensitivity to any component or thimerosal; severe or even moderate febrile illness; age <2 y; thrombocytopenia or any coagulation disorder that contraindicates IM injection unless potential benefit clearly outweighs risk
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Arthralgia, fever, urticaria, and Guillain-Barré syndrome (rare)
Meningococcal vaccine (Menomune A/C/Y/W-135, Menactra)
Capsular polysaccharide antigens (groups A, C, Y, and W-135) of Neisseria meningitidis. For active immunization against invasive meningococcal disease caused by inclusive serogroups. May be used to prevent and control outbreaks of serogroup C meningococcal disease according to Centers for Disease Control and Prevention (CDC) guidelines.
Routine vaccination recommended for high-risk groups (eg, patients with deficiencies in late complement components [C3, C5-C-9], functional or actual asplenia, or laboratory or industrial exposure to N meningitidis aerosols; travelers or residents of hyperendemic areas).
Vaccine induces antibody response for serogroup A in individuals as young as 3 mo, but poorly immunogenic for serogroup C in recipients <18-24 mo.
Adult
0.5 mL SC
Pediatric
<2 years: Not established
>2 years: Administer as in adults
Coadministration with whole-cell pertussis or whole-cell typhoid vaccines may increase endotoxin content; immunosuppressive drugs may interfere with immune response
Documented hypersensitivity; acute illness (avoid); IV/IM/ID administration
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Asplenic patients with lymphoid tumors who receive chemotherapy or irradiation respond poorly; for information concerning geographic areas where vaccination is recommended, see the Meningococcal Conjugate Vaccine: ACIP Recommendation page from the CDC Advisory Committee on Immunization Practices (ACIP); spontaneous reports of Guillain-Barré syndrome recently (2005) reported with Menactra
H influenzae B vaccine (PedvaxHIB, HibTITER, ActHIB)
For routine immunization of children against invasive diseases caused by H influenzae type B by decreasing nasopharyngeal colonization. The CDC ACIP recommends that all children receive one of the conjugate vaccines licensed for use in infants beginning routinely at age 2 mo.
Adult
Not indicated
Pediatric
Regimens vary depending on product.
Example:
HibTITER:
2-6 months: 0.5 mL IM q2mo for 3 doses
7-11 months: 0.5 mL IM q2mo for 2 doses in previously unvaccinated children
12-14 months: 0.5 mL IM once in previously unvaccinated children
15-71 months: 0.5 mL IM once in previously unvaccinated children
Booster dose: 0.5 mL at age 15 mo or at least 2 mo after last dose of immunization series; booster dose unnecessary in children vaccinated for the first time at age 15-71 mo
Immunoglobulins given within 1 mo or concurrently with immunosuppressants may inhibit full immunologic response
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Delay immunization if febrile illness evident; may cause erythema, swelling, or tenderness; cause-and-effect relationship with Guillain-Barré syndrome after vaccination not established
Antibiotics
Daily antibiotic prophylaxis with penicillin is recommended to prevent pneumococcal septicemia.
Penicillin VK (V-Cillin K, Veetids, Pen-Vee K)
Inhibits biosynthesis of cell-wall mucopeptide. Bactericidal against sensitive organisms when adequate concentrations reached. Most effective during stage of active multiplication. Low concentrations produce bacteriostatic effects.
Adult
Pediatric
<5 years: 125 mg PO bid
>5 years: 250 mg PO bid
Probenecid may increase effectiveness by decreasing clearance; tetracyclines are bacteriostatic, decreasing in effectiveness of penicillins when administered concurrently
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in renal impairment
More on Splenomegaly |
| Overview: Splenomegaly |
| Differential Diagnoses & Workup: Splenomegaly |
 Treatment & Medication: Splenomegaly |
| Follow-up: Splenomegaly |
| References |
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References
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Further Reading
Keywords
splenomegaly, splenic enlargement, enlarged spleen, palpable spleen, hypersplenism, splenic enlargement, splenectomy, mononuclear-phagocyte system, MPS, malaria, schistosomiasis, hyperreactive malarial splenomegaly, splenic rupture, noncirrhotic portal fibrosis, sickle cell disease, hepatitis, portal hypertension, abdominal trauma, splenic hematoma, diarrhea, salmonellosis, leukemia, Hodgkin disease, jaundice, sepsis, hypotension, umbilical catheter thrombosis, anemia, leishmaniasis, trypanosomiasis, splenic pseudocyst, cytomegalovirus, human immunodeficiency virus, HIV, hemolytic anemia–associated gallstones, hemolytic anemia, thalassemia, glucose-6-phosphate dehydrogenase deficiency, G6PD deficiency, hereditary pyropoikilocytosis, pyruvate kinase deficiency, hereditary spherocytosis
