The document discusses several indications for splenectomy including immune thrombocytopenic purpura (ITP), hereditary spherocytosis, hemoglobinopathies, malignancy, splenic abscess, cysts, and vein thrombosis. For ITP, splenectomy is considered if thrombocytopenia is refractory to steroids, relapse occurs after treatment, or platelet levels remain low during pregnancy. It has a 65% success rate for improving thrombocytopenia. Hereditary spherocytosis and hemoglobinopathies can cause hemolytic anemia treated with splenectomy. Splenectomy may also be used for staging or treatment of certain lymphomas and metastases to the spleen. It is often recommended for

1. INDICATIONS FOR SPLENECTOMY
(NON-TRAUMA)
Size of spleen ~12 x 7 x 3 to 4 cm in thickness. The average weight 150 g, (80 to 300 g).
Spleen: major hematopoietic functions until 5th month of gestation ⇒ bone marrow assumes
function. No significant hematopoietic function left in the spleen except in hematologic
disorders: Myelodysplastic syndrome, hemoglobinopathies.
The functions: closely linked its unique circulatory system. The arteries the white pulp
(lymphoid tissues) endothelial cell-lined capillaries into the venous system ("closed"
theory). Most of the blood flow, however, enters the macrophage-lined reticular meshwork,
and the blood flows slowly back to the venous circulation through the venous sinuses ("open"
theory). Abnormal blood component will be trapped in the spleen and ingested by splenic
phagocytes.
The most important function: mechanical filtration, which removes senescent RBC and
control of infection.
The spleen is a major site of production for the opsonins, properdin and tuftsin. Properdin:
alternative pathway of complement activation ⇒ destruction of bacteria, foreign and
abnormal cells. Tuftsin: peptides that enhances the phagocytic activity of both PMN and
phagocytes. Decreased neutrophil function in asplenic patients: result from the absence of a
circulating mediator.
Immune Thrombocytopenic Purpura
(idiopathic thrombocytopenic purpura): Low platelet count, a normal bone marrow, and the
absence of other causes of thrombocytopenia.
Increased platelet destruction: autoantibodies to platelet membrane antigens ⇒ phagocytosis
by the reticuloendothelial system. Bone marrow megakaryocytes are present in normal or
sometimes increased numbers.
The initial treatment: prednisone (1 mg/kg) 26-30% complete response.
Indication for splenectomy in ITP:
• refractory severe thrombocytopenia
• toxic doses of steroids
• relapse after initial steroids
• >6 weeks and continue to have a platelet < 10,000/mm 3
• >3 months with incomplete response to primary therapy platelet < 30,000/mm 3
• 2nd trimester of pregnancy with medical refractory platelet <10,000/mm 3 or who
have platelet <30,000/mm 3 and bleeding problems.
The success rate (complete and permanent response) ~65%
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2. Preoperative indium-111-labeled platelet scintigraphy could be predictive of the efficacy of
splenectomy. The best long-term cure rates have been when predominantly splenic
sequestration is present (87 to 93%). hepatic sequestration ~7 to 30%
Increase platelet count usually seen within the first 10 days post op. Durable platelet
responses if platelet > 150,000/mm 3 by the 3rd day post op with relapse rates of 4 to 12%
Failure to respond to splenectomy or relapse after an initial response : Accessory spleen up
to10% the absence of asplenic red blood cell morphologic features and may also be identified
by radionuclide imaging. If accessory spleen found in a patient who remains severely
thrombocytopenic surgical excision of the accessory spleen.
Hereditary Spherocytosis
• autosomal dominant disease that results from a deficiency of spectrin, a red blood cell
cytoskeletal protein.
• This defect causes membrane abnormality in the red blood cells that are small, spherical,
and rigid.
• These cells have increased osmotic fragility.
• These. spherocytes are more susceptible to becoming trapped in the spleen and destroyed.
• Hemolytic anemia, occasionally jaundice, and splenomegaly.
• The diagnosis is made by identification of spherocytes on the peripheral blood smear, an
increased reticulocyte count, increased osmotic fragility, and a negative Coombs test.
• Splenectomy decreases the rate of hemolysis and usually leads to resolution of the
anemia.
• Splenectomy is usually performed in childhood shortly after diagnosis but is delayed until
after the 4th year of life to preserve immunologic function of the spleen in young children
(risk for OPSI).
• Cholecystectomy: recommended at time of splenectomy if gallstones are present.
Hemoglobinopathies
• Indications for splenectomy in patients with sickle cell disease include:
○ acute splenic sequestration crisis (rapid enlargement of spleen, anemia pain, blood
transfusion)
○ hypersplenism (reduces transfusions improve cytopenia)
○ splenic abscess.
Splenic abscesses (not uncommon): fever, abdominal pain, and a tender, enlarged
spleen.
Many patients with splenic abscess have leukocytosis; thrombocytosis and
Howell-Jolly bodies also occur in these patients, indicating functional asplenia.
Common organisms in sickle cell anemia are salmonella, enterobacteria.
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3. Splenectomy for Malignancy
• Lymphomas
○ HODGKIN'S DISEASE
Most patients have asymptomatic lymphadenopathy (cervical node) at the time of
diagnosis (20s-30s), and most present with enlargement.
The improved non-operative staging has led to a decrease in the numbers of
patients requiring staging laparotomy.
Staging laparotomy and splenectomy for selected patients with an early clinical
stage of disease (Stage IA or IIA) in whom pathologic staging of the abdomen
will significantly influence the therapeutic management.
○ NON-HODGKIN'S LYMPHOMAS
Splenomegaly or hypersplenism is a common occurrence during the course of
NHL.
Splenectomy is indicated for patients with NHL for treatment of massive
splenomegaly (abdominal pain, fullness, and early satiety) and hypersplenism
Splenectomy: diagnosis and staging of patients who present with isolated splenic
disease.
The most common primary splenic neoplasm is NHL (50 to 80% of NHL)
There is frequent involvement of the splenic hilar lymph nodes, extra-hilar
nodes, bone marrow, and liver in these patients.
About 75% exhibit clinical evidence of hypersplenism.
○ Hairy cell leukemia, CML, CLL:
splenectomy only for palliation of symptoms or complications of splenomegaly
(hypersplenism, abscess, rupture)
• Nonlymphoid Tumors of the Spleen
○ Metastatic tumor in up to 7% of autopsies of cancer patients.
○ Breast, lung, and melanoma; however, virtually any primary malignancy may
metastasize to the spleen.
○ Often asymptomatic but may be associated with symptomatic splenomegaly or even
spontaneous splenic rupture.
○ Splenectomy may provide effective palliation in carefully selected symptomatic
patients with splenic metastasis.
• Vascular neoplasms:
○ commonly found incidentally.
○ Hemangiomas are usually incidental findings identified in spleens removed for other
reasons.
○ Angiosarcomas (or hemangiosarcomas) of the spleen have been associated with
environmental exposure to thorium dioxide or monomeric vinyl chloride
May present with splenomegaly, hemolytic anemia, ascites, and pleural effusions
or with spontaneous splenic rupture poor prognosis.
○ Lymphangiomas are usually benign endothelium-lined cysts that may become
symptomatic by causing splenomegaly.
○ Lymphangiosarcoma within a cystic lymphangioma has been reported.
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4. Splenectomy is appropriate for diagnosis, treatment, or palliation of the conditions cited
previously.
• Splenic Abscess
○ Splenic abscess is an uncommon and potentially fatal illness.
○ The mortality for splenic abscess ranges from about 80% in immunocompromised
patients to about 15 to 20% in previously healthy patients with solitary unilocular
lesions.
○ Predisposing illnesses include:
Malignancies
polycythemia vera
endocarditis
previous trauma
hemoglobinopathy (such as sickle cell disease)
urinary tract infection
intravenous drug abuse
AIDS
○ ~70% from hematogenous spread (endocarditis, osteomyelitis, and IVDA). but may
also occur as the result of infection of a nearby structure, such as the colon, kidney, or
pancreas.
Gram-positive cocci, such as Staph, Strep, or Enterococcus, and gram-negative
enteric organisms.
Less common: M. tuberculosis, M. avium, and Actinomyces species.
Immunosuppressed patients may develop multiple fungal abscesses, typically
from Candida species infection.
○ Presentation is often nonspecific and insidious, including LUQ abdominal pain, fever,
peritonitis, and pleuritic chest pain.
○ Splenomegaly is present in a minority of patients.
○ Diagnosis:
most accurately by CT; however, it may also be made with ultrasonography.
○ Two thirds of splenic abscesses in adults are solitary, one third are multiple. These
ratios are reversed in children.
○ Unilocular abscesses are amenable to CT-guided drainage, along with systemic
antibiotic has a success rate of 75%-90%.
○ Failure of a prompt clinical response to percutaneous drainage should lead to
splenectomy without delay.
○ Multilocular abscesses should usually be treated by splenectomy, with drainage.
• Splenic Cysts
○ Most are incidental findings.
○ Primary true cysts of the spleen account for about 10% of all nonparasitic cysts of the
spleen.
○ Most nonparasitic cysts are pseudocysts and are secondary to trauma.
○ Focal calcifications up to half of cases.
○ Most are unilocular, and the cysts are smooth and thick-walled.
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5. ○ Small asymptomatic splenic pseudocysts (less than 4 cm) do not require treatment
and may undergo involution over time.
○ Symptomatic pseudocyst percutaneous drainage: up to 90% success rate or partial
splenectomy.
○ True splenic cyst found the second and third decades of life
squamous epithelial lining
often + for CA 19-9 and CEA
many are considered congenital.
○ The presence of symptoms is often related to the size of the cysts > 8cm
○ may present with acute symptoms related to rupture, hemorrhage, or infection.
○ The diagnosis of splenic cyst is best made with CT imaging.
○ Operative intervention is indicated for symptomatic cysts and for large cysts.
○ Either total or partial splenectomy may provide successful treatment.
○ Preservation of at least 25% of the spleen protect appears sufficient to protect against
pneumococcal pneumonia
○ Most true splenic cysts are parasitic cysts in areas of endemic hydatid disease
(Echinococcus species).
Imaging studies: cyst wall calcifications or daughter cysts.
Serologic tests for Echinococcus species are often helpful in verifying the
presence of parasites.
Spillage of cyst contents may precipitate an anaphylactic shock and risks
intraperitoneal dissemination of infective scolices.
Splenectomy is the treatment of choice.
The cysts may be sterilized by injection of a 3% sodium chloride solution,
alcohol, or 0.5% silver nitrate, as has been recommended for hydatid cysts of
the liver.
• Splenic vein thrombosis (SVT)
○ UGI bleeding from esophageal or gastric varicies without presence of cirrhosis.
○ Splenomegaly without cirrhosis, portal HTN or hematologic disease.
○ Most common cause:
pancreatitis (acute, chronic, pseudocyst).
Pancreatic neoplasms
Adenopathy
metastatic cancer
post partial gastrectomy.
○ 17% of SVT has varicies,
○ 23% progressed to PV thrombosis.
○ EUS may be more sensitive than regular sonogram.
○ Venous phase angiogram is gold standard.
○ Splenectomy is treatment of choice in SVT
sclerotherapy has high success rate
embolization in acute bleed effective, but splenic infarct up to (25%) and
abscess may follow.
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6. References
• Townsend: Sabiston Textbook of Surgery, 16th ed., Copyright © 2001 W. B. Saunders
Company
• George JN, Woolf SH, Raskob GE, et al: Idiopathic thrombocytopenic purpura: A
practice guideline developed by explicit methods for the American Society of
Hematology. Blood 88:3-40, 1996.
• Sharon M. Weber, M.D., Layton F. Rikkers, M.D. Splenic Vein Thrombosis and
Gastrointestinal Bleeding in Chronic Pancreatitis World J. Surg 2003:; 27: 1271–1274,
Naris Nilubol, M.D.
May 17, 2004
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