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Splenomegaly Treatment &
Management
Updated: Jan 09, 2018
Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD  more...

TREATMENT

Approach Considerations
Successful medical treatment of the primary disorder in cases of splenomegaly can lead to regression
of the hypersplenism without the need for surgery.

Splenectomy is indicated to help control or stage the underlying disease in cases of splenomegaly.
These diseases can include hereditary spherocytosis, immune thrombocytopenia (ITP) or
autoimmune hemolysis. In addition, splenectomy enables pathological diagnosis in
lymphoproliferative disorders such as splenic marginal zone lymphoma or hairy cell leukemia

Splenectomy is also indicated for the treatment of chronic, severe hypersplenism. [20] This can occur
in conditions such as the following:

Hairy cell leukemia

Felty syndrome
Primary myelofibrosis (agnogenic myeloid metaplasia)
Thalassemia major
Gaucher disease
Hemodialysis splenomegaly
Splenic vein thrombosis [8]

Treatment of splenic sequestration involves conservative management with blood

transfusions/exchange transfusions to reduce the number of sickled red blood cells, or splenectomy.
Splenectomy, if full, will prevent further sequestration and if partial, may reduce the recurrence of
acute splenic sequestration crises. However, there is a lack of evidence from trials showing that
splenectomy improves survival and decreases morbidity in sickle cell disease. [21]

In rare cases, splenectomy may be used to treat thrombotic thrombocytopenic purpura (TTP).
However, therapeutic plasma exchange transfusion (plasmapheresis) has largely supplanted the need
for splenectomy in these patients

Low-dose radiotherapy has been used as palliative care for splenomegaly in patients with hematologic
disorders such as primary myelofibrosis. [22] Bruns et al reported that low-dose splenic irradiation
produced hematologic response and long-term relief of splenic pain in four of five patients with
symptomatic congestive splenomegaly. [23]

In a review by Zaorsky et al that included 766 courses of splenic irradiation for 486 patients from 1960
to 2016, the most common cancers treated included chronic lymphocytic leukemia and
myeloproliferative disorders. Splenic irradiation produced a partial or complete response in 85-90% of
patients; 30% were retreated within 6-12months. [24]

Although the most common splenic irradiation regimen was 10Gy in 1Gy fractions over 2 weeks, these
authors concluded that lower doses (eg, 5Gy in 5 fractions) might be as effective as higher doses, as
they found no correlation between the biologically equivalent dose of radiation therapy and response
duration, pain relief, spleen reduction, or cytopenia improvement. [24]

Inpatient care

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

Outpatient care

Outpatient care of patients with splenomegaly consists of three main focus areas: (1) primary
etiologic disease; (2) blood count monitoring, especially when associated with a myeloproliferative
disease as the cause; and (3) monitoring for overwhelming postsplenectomy infection (OPSI).

Thrombocytosis may require treatment when the platelet count exceeds 1 million/μL. Multiple
modalities have been used to reduce the platelet count or inhibit their thrombotic effects, including
hydroxyurea, aspirin, or plateletpheresis (collection and removal of platelets from the circulation). No
randomized, placebo-controlled studies have demonstrated a better survival benefit with one therapy
over the other. Whether any discrete benefit is gained by also controlling the platelet count remains
unclear.

Transfer

Patients undergoing elective splenectomy for splenomegaly may develop significant hemorrhaging
during their operation if controlling the splenic hilum proves difficult. Such patients may require
abdominal packing and transfer to a tertiary center with personnel who have expertise in
angioembolization and splenic resection for splenomegaly. [3]

Such centers usually have the additional resources (eg, a well-stocked blood bank, a 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.

Consultations

Consultation with a hematologist is ideal before surgery for enlarged spleens in order to secure
necessary blood products. Postoperative management does not usually require intervention from a
hematologist.
Activity
The usual postoperative activity restrictions imposed on a patient who has undergone a laparotomy or
laparoscopy also apply to patients after a laparoscopic splenectomy.

Patients with uncorrected splenomegaly should be counseled to refrain from contact sports or
activities that would predispose them to blunt abdominal trauma. Examples include skydiving,
horseback riding, soccer, football, and ice hockey. These restrictions reduce the likelihood that blunt
injury will lead to splenic rupture and uncontrolled hemorrhage.

Pharmacologic Therapy
Chemotherapy is used for hematologic malignancies. Antibiotics are used for infection, with the
exception of infection associated with a splenic abscess; this requires surgical intervention.

Immunosuppression is used for autoimmune or inflammatory disorders, treatment of cirrhosis, and

CHF. All patients scheduled for elective splenectomy (either diagnostic or therapeutic) should receive
a pneumococcal vaccine. Also consider administering prophylaxis against Haemophilus influenzae
and Neisseria meningitidis.

Eliglustat, an oral substrate reduction therapy, significantly improved spleen volume, hemoglobin level,
liver volume, and platelet count in 40 previously untreated adults with Gaucher disease type 1 in a
randomized, double-blind, placebo-controlled study. [25]

Splenectomy
The vast majority of splenectomies are performed using laparoscopic techniques. Laparoscopic
splenectomy is safe and is associated with reduced hospital stays. Furthermore, this procedure has a
postoperative survival advantage when compared with open procedures. Laparoscopic surgery can be
performed even on individuals with massive splenomegaly. (See the images below.) [26, 27]
Intraoperative photograph of a laparoscopic splenectomy being taken down using the hanging-pedicle technique. The tip of
the spleen is visualized in the background, whereas the stapler is detailed in the foreground across a segment of the pedicle.

Massive splenomegaly does not preclude splenectomy through a minimally invasive approach. This photograph depicts a
fragmented 3.2 kg (7.05 lb) spleen after removal via a hand-assisted laparoscopic technique.
A portion of a massive spleen is extracted via hand-assisted laparoscopy.

Occasionally, a reactive thrombocytosis occurs following splenectomy. Thrombocytosis in the face of

splenectomy rarely requires treatment. It is most common in patients with massive splenomegaly
from myeloproliferative disorders.

An onset of fever several days following splenectomy can be due to a recrudescence of malaria. This
should be considered as a cause of fever in patients who have lived in areas commonly associated
with malaria and in persons who abuse intravenous (IV) drugs who share needles.

With Plasmodium malariae infection, this may occur decades after the initial infection. Malaria from P
vivax (3-7 y) and P falciparum (about 1 y) remain active for shorter intervals after the initial infection.

Treatment of 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%. Information now suggests,
however, that if patients seek medical attention promptly, the mortality rate may be reduced to
approximately 10%. Of those patients who die, more than 50% do so within the first 48 hours of
hospital admission.
Most instances of serious infection are due to encapsulated bacteria, such as pneumococci (eg,
Streptococcus pneumoniae). 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.
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, although accessory spleens can also be seen as a developmental anomaly.

Prevention

Preventative strategies for OPSI fall into 3 major categories: education, immunoprophylaxis, and
chemoprophylaxis.

As previously mentioned, education represents a mandatory strategy in the prevention of 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.

Vaccination is also appropriate in the prevention of OPSI. 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 or, at the latest, 24 hours following the procedure.

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.

Preprocedure prophylaxis
A major 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 to be found at the operative site.

Medication