eMedicine Specialties > Hematology > Immune System and Disorders

Splenomegaly: Follow-up

Author: David J Draper, MD, Hematology/Oncology Fellow, The University Hospital, Cincinnati, Ohio
Coauthor(s): Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center; Emmanuel N Dessypris, MD, Professor of Medicine, Medical College of Virginia; Chief, Medical Service, Hunter Holmes McGuire Department of Veterans Affairs Medical Center; Lewis J Kaplan, MD, FACS, FCCM, FCCP, Director, SICU and Surgical Critical Care Fellowship, Associate Professor, Department of Surgery, Section of Trauma, Surgical Critical Care, and Surgical Emergencies, Yale University School of Medicine
Contributor Information and Disclosures

Updated: Oct 4, 2009

Follow-up

Further Inpatient Care

• Inpatient care for patients with splenomegaly depends on the treatment modality that is used to treat the underlying cause and on the complications of that care. These therapies are not unique to splenomegaly treatment and, therefore, are not discussed.

Further Outpatient Care

• Outpatient care of patients with splenomegaly consists of 2 main focus areas: (1) monitoring for thrombocytosis and (2) monitoring for overwhelming postsplenectomy sepsis (OPSS).
  • Thrombocytosis may require treatment when the platelet count exceeds 1 million/μ L. Multiple modalities have been used, including hydroxyurea and aspirin. No randomized, placebo-controlled studies demonstrate a better survival benefit with one therapy over the other. Whether any discrete benefit is gained by also controlling the platelet count remains unclear.
  • OPSS is a relatively rare condition that may follow a splenectomy. Infection is with encapsulated organisms (S pneumonia, H influenza, N meningitidis). Because these organisms are encapsulated and the spleen is integral in the removal of opsonized bacteria, affected patients are at increased risk for unimpeded sepsis. Rapid therapy is essential because of the increased risk of death from a rapidly progressive infection. A prototype infection is meningococcemia (ie, Waterhouse-Friderichsen syndrome), which is complicated by adrenal hemorrhage and infarction.

Transfer

• Transfer of patients with splenomegaly is not generally required except for complications of surgery or OPSS. Patients undergoing elective splenectomy for splenomegaly may develop significant hemorrhaging during their operation if difficulty occurs controlling the splenic hilum. Such patients may require abdominal packing and transfer to a tertiary center with personnel who have expertise in angioembolization and splenic resection for splenomegaly.¹⁵ Such centers usually have the additional resources (eg, well-stocked blood bank, tertiary level intensive care unit) to support the organ systems in these patients. Multisystem organ failure is not uncommon following severe hemorrhage, and these patients are no exception.

Deterrence/Prevention

• The only applicable prevention is attempts at preventing droplet transfer/infection in patients with known mononucleosis and splenomegaly as an accompanying feature. This maneuver may decrease the risk of mononucleosis and splenomegaly in others, but it does nothing to aid the index patient.

Complications

• Postsplenectomy infection
  • Fulminant, life-threatening infection represents a major long-term sequela after splenectomy in patients with splenomegaly. Splenic macrophages play a major role in filtering and phagocytizing bacteria and parasitized blood cells from the circulation. In addition, the spleen is a significant source of antibody production.
  • Overwhelming postsplenectomy infection (OPSI), also known as postsplenectomy sepsis syndrome, begins as a nonspecific flulike prodrome that is followed by a rapid evolution to full-blown bacteremic septic shock — accompanied by hypotension, anuria, and clinical evidence of disseminated intravascular coagulation — thus making this syndrome a true medical emergency. The subsequent clinical course often mirrors that of the Waterhouse-Friderichsen syndrome, with bilateral adrenal hemorrhages noted at autopsy.
  • Despite appropriate antibiotics and intensive therapeutic intervention, the overall mortality rate in older published studies of established cases of OPSI varied from 50-70%. More recent information suggests that if patients seek medical attention promptly, the mortality rate may be reduced to approximately 10%. Of those patients who die, more than 50% die within the first 48 hours of hospital admission.
  • Most instances of serious infection are due to encapsulated bacteria, such as pneumococci (eg, S pneumoniae). Pneumococcal infections account for 50-90% of cases reported in the literature and may be associated with a mortality rate of up to 60%. H influenza type B, meningococci, and group A streptococci account for an additional 25% of infections.
  • Possible OPSI involving an asplenic individual constitutes a medical emergency. The critical point in management remains early recognition of the patient at risk, followed by subsequent aggressive intervention. The diagnostic workup should never delay the use of empiric therapy.Possible choices of empiric antimicrobial agents include cefotaxime (adult dose of 2 g IV q8h; pediatric dose of 25-50 mg/kg IV q6h) or ceftriaxone (adult dose of 2 g q12-24h; pediatric dose of 50 mg/kg IV q12h). Unfortunately, some penicillin-resistant pneumococcal isolates are also resistant to cephalosporins. If such resistance is suggested, consider using vancomycin.
  • The precise incidence of OPSI remains controversial. Overall, the most reliable data related to incidence estimate approximately 1 case occurring per 500 person-years of observation. Asplenic children younger than 5 years, especially infants splenectomized for trauma, may have an infection rate of greater than 10%.
  • Splenectomy performed for a hematologic disorder, such as thalassemia, hereditary spherocytosis, or lymphoma, appears to carry a higher risk than splenectomy performed as a result of trauma. A major contributing factor is the frequent existence of splenic implants or accessory spleens in traumatized patients.
  • Preventative strategies for OPSI fall into 3 major categories: education, immunoprophylaxis, and chemoprophylaxis.
    • Education represents a mandatory strategy in attempting to prevent OPSI. Asplenic patients should be encouraged to wear a Medi-Alert (Pinellas Park, Fla/Henderson, Nev) bracelet and carry a wallet card explaining their lack of a spleen. Patients should also be aware of the need to notify their physician in the event of an acute febrile illness, especially if it is associated with rigors or systemic symptoms.
    • An appropriate factor in preventing OPSI entails vaccination. This has best been defined for S pneumoniae. Unfortunately, the most virulent pneumococcal serotypes tend to be the least immunogenic, and evidence indicates that the efficacy of the vaccine is poorest in younger patients, who would be at higher risk. However, under ideal conditions in a healthy immunocompetent host, the vaccine offers a 70% protection rate.The pneumococcal vaccine should be administered at least 2 weeks before an elective splenectomy. If the time frame is not practical, the patient should be immunized as soon as possible after recovery and before discharge from the hospital.
    • Most authorities recommend antibiotic prophylaxis for asplenic children, especially for the first 2 years after splenectomy. Some investigators advocate continuing chemoprophylaxis in children for at least 5 years or until age 21 years. However, the value of this approach in older children or adults has never been adequately evaluated in a clinical trial.

Prognosis

• The prognosis of patients with splenomegaly is usually excellent and not substantially different from age-matched controls, but it is impacted by the underlying disease state rather than the presence of splenomegaly or the postsplenectomy state.

Patient Education

• Patients with splenomegaly need education regarding decreasing their risk of splenic hemorrhage. These patients must be cautioned about contact sports and other activities that may acutely increase their intra-abdominal pressure or place excessive forces on the left upper quadrant, left flank, or lateral back. This decreases the likelihood of splenic rupture in a patient with an abnormal splenic mass and capsule. The routine use of seat belts is essential while driving or riding in a motor vehicle.
• Additional education regarding the signs and symptoms of postsplenectomy sepsis cannot be overstressed. Prompt antibiotic therapy may be lifesaving.
• Preoperative and preprocedure antibiotic prophylaxis is equally important in procedures that are associated with a high likelihood of bacteremia.

Miscellaneous

Medicolegal Pitfalls

• Failure to recognize the need for immunization after splenectomy for protection against encapsulated organisms such as H influenzae, N meningitidis, and S pneumoniae  may be a medicolegal pitfall.

Special Concerns

• The major special concern is antibiotic use in splenectomized patients. Those who have undergone splenectomy should receive antibiotic prophylaxis prior to undergoing procedures associated with a risk of transient or sustained bacteremia. Antibiotics should cover encapsulated organisms and organisms likely found at the operative site. This is an important maneuver to decrease the risk of overwhelming postsplenectomy sepsis.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor David Coffman, MD, to the development and writing of this article.

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