Splenectomy

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Splenectomy
Spleen after spleenectomy.jpg
Surgically removed spleen of a child with thalassemia. It is about 15 times larger than normal.
ICD-9-CM 41.43, 41.5
MeSH D013156
OPS-301 code 5-413

A splenectomy is the surgical procedure that partially or completely removes the spleen. The spleen is an important organ in regard to immunological function due to its ability to efficiently destroy encapsulated bacteria. Therefore, removal of the spleen runs the risk of overwhelming post-splenectomy infection, a medical emergency and rapidly fatal disease caused by the inability of the body's immune system to properly fight infection following splenectomy or asplenia. [1]

Contents

Common indications for splenectomy include trauma, tumors, splenomegaly or for hematological disease such as sickle cell anemia or thalassemia. [2]

Indications

The spleen is an organ located in the abdomen next to the stomach. It is composed of red pulp which filters the blood, removing foreign material, damaged and worn out red blood cells. It also functions as a storage site for iron, red blood cells and platelets. The rest (~25%) of the spleen is known as the white pulp and functions like a large lymph node being the largest secondary lymphoid organ in the body. [3] Apart from regular lymphatic function the white pulp contains splenic macrophages which are particularly good at destroying (phagocytosis) encapsulated bacteria such as Streptococcus pneumoniae. [4] The spleen is also known to function as a site for the development of new red blood cells from their hematopoietic stem cell precursors, and particularly in situations in which the bone marrow, the normal site for this process, has been compromised by a disorder such as leukemia. The spleen is enlarged in a variety of conditions such as malaria, mononucleosis and most commonly in cancers of the lymphatics, such as lymphomas or leukemia.

It is removed under the following circumstances:

  1. When it becomes very large such that it becomes destructive to platelets/red blood cells or rupture is imminent
  2. For diagnosing certain lymphomas
  3. Certain cases of splenic abscess
  4. Certain cases of wandering spleen
  5. Splenic vein thrombosis with bleeding Gastric varices
  6. When platelets are destroyed in the spleen as a result of an auto-immune condition, such as idiopathic thrombocytopenic purpura.
  7. When the spleen bleeds following physical trauma
  8. Following spontaneous rupture
  9. For long-term treatment of congenital erythropoietic porphyria (CEP) if severe hemolytic anemia develops [5]
  10. The spread of gastric cancer to splenic tissue
  11. When using the splenic artery for kidney revascularisation in renovascular hypertension.
  12. For long-term treatment of congenital pyruvate kinase (PK) deficiency
  13. Those who have a severe version of the hereditary blood disorder Spherocytosis.
  14. During surgical resection of a pancreatic cancer

The classical cause of traumatic damage to the spleen is a blow to the abdomen during a sporting event. In cases where the spleen is enlarged due to illness (mononucleosis), trivial activities, such as leaning over a counter or straining while defecating, can cause a rupture.

Procedure

Laparoscopy is the preferred procedure in cases where the spleen is not too large and when the procedure is elective. Open surgery is performed in trauma cases or if the spleen is enlarged. Either method is major surgery and is performed under general anesthesia. Vaccination for S. pneumoniae, H. influenza and N. meningitidis should be given pre-operatively if possible to minimize the chance of overwhelming post-splenectomy infection (OPSI), a rapid-developing and highly fatal type of septicaemia. The spleen is located and disconnected from its arteries. The ligaments holding the spleen in place, gastrosplenic ligament, splenorenal ligament and splenocolic ligament, are dissected and the organ is removed. In some cases, one or more accessory spleens are discovered and also removed during surgery. The incisions are closed and when indicated, a drain is left. If necessary, tissue samples are sent to a laboratory for analysis.[ citation needed ]

Side effects

Splenectomy causes an increased risk of sepsis, particularly overwhelming post-splenectomy sepsis due to encapsulated organisms such as S. pneumoniae and Haemophilus influenzae which are no longer able to be destroyed. [4] It has been found that the risk of acquiring sepsis is 10 to 20 times higher in a splenectomized patient compared to a non-splenectomized patient, which can result in death, especially in young children. [6] Therefore, patients are administered the pneumococcal conjugate vaccine (Prevnar), Hib vaccine, and the meningococcal vaccine post-operatively (see asplenia). These bacteria often cause a sore throat under normal circumstances but after splenectomy, when infecting bacteria cannot be adequately opsonized, the infection becomes more severe.[ citation needed ]

Splenectomy also increases the severity of babesiosis, Splenectomized patients are more susceptible to contracting babesiosis and can die within five to eight days of symptom onset. [7] They have severe hemolytic anemia, and occasional hepatomegaly has been documented. Parasitemia levels can reach up to 85% in patients without spleens, compared to 1–10% in individuals with spleens and effective immune systems. [8]

An increase in blood leukocytes can occur following a splenectomy. [9] The post-splenectomy platelet count may rise to abnormally high levels (thrombocytosis), leading to an increased risk of potentially fatal clot formation. Mild thrombocytosis may be observed after a splenectomy due to the lack of sequestering and destruction of platelets that would normally be carried out by the spleen. In addition, the splenectomy may result in a slight increase in the production of platelets within the bone marrow. Normally, erythrocytes are stored and removed from the circulating blood by the spleen, including the removal of damaged erythrocytes. However, after a splenectomy the lack of presence of the spleen means this function cannot be carried out so damaged erythrocytes will continue to circulate in the blood and can release substances into the blood. If these damaged erythrocytes have a procoagulant activity then the substances they release can lead to the development of a procoagulant state and this can cause thromboembolic events e.g. pulmonary embolism, portal vein thrombosis and deep vein thrombosis. [6] There also is some conjecture that post-splenectomy patients may be at elevated risk of subsequently developing diabetes. [10] Splenectomy may also lead to chronic neutrophilia. Splenectomy patients typically have Howell-Jolly bodies [11] [12] and less commonly Heinz bodies in their blood smears. [13] Heinz bodies are usually found in cases of G6PD (Glucose-6-Phosphate Dehydrogenase) and chronic liver disease. [14]

A splenectomy also results in a greatly diminished frequency of memory B cells. [15] A 28-year follow-up of 740 World War II veterans who had their spleens removed on the battlefield showed a significant increase in the usual death from pneumonia (6 deaths rather than the expected 1.74) and an increase in the deaths from ischemic heart disease (41 deaths rather than the expected 30.26) but not from other conditions. [16]

Subtotal splenectomy

Much of the spleen's protective roles can be maintained if a small amount of spleen can be left behind. [17] Where clinically appropriate, attempts are now often made to perform either surgical subtotal (partial) splenectomy, [18] or partial splenic embolization. [19] In particular, whilst vaccination and antibiotics provide good protection against the risks of asplenia, this is not always available in poorer countries. [20] However, as it may take some time for the preserved splenic tissue to provide the full protection, it has been advised that preoperative vaccination still be given. [21]

See also

Related Research Articles

<span class="mw-page-title-main">Spleen</span> Internal organ in all vertebrates

The spleen is an organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes from Ancient Greek σπλήν (splḗn).

<span class="mw-page-title-main">Hemolysis</span> Rupturing of red blood cells and release of their contents

Hemolysis or haemolysis, also known by several other names, is the rupturing (lysis) of red blood cells (erythrocytes) and the release of their contents (cytoplasm) into surrounding fluid. Hemolysis may occur in vivo or in vitro.

<span class="mw-page-title-main">Spherocytosis</span> Medical condition

Spherocytosis is the presence of spherocytes in the blood, i.e. erythrocytes that are sphere-shaped rather than bi-concave disk shaped as normal. Spherocytes are found in all hemolytic anemias to some degree. Hereditary spherocytosis and autoimmune hemolytic anemia are characterized by having only spherocytes.

<span class="mw-page-title-main">Babesiosis</span> Malaria-like parasitic disease caused by infection with the alveoate Babesia or Theileria

Babesiosis or piroplasmosis is a malaria-like parasitic disease caused by infection with a eukaryotic parasite in the order Piroplasmida, typically a Babesia or Theileria, in the phylum Apicomplexa. Human babesiosis transmission via tick bite is most common in the Northeastern and Midwestern United States and parts of Europe, and sporadic throughout the rest of the world. It occurs in warm weather. People can get infected with Babesia parasites by the bite of an infected tick, by getting a blood transfusion from an infected donor of blood products, or by congenital transmission . Ticks transmit the human strain of babesiosis, so it often presents with other tick-borne illnesses such as Lyme disease. After trypanosomes, Babesia is thought to be the second-most common blood parasite of mammals. They can have major adverse effects on the health of domestic animals in areas without severe winters. In cattle the disease is known as Texas cattle fever or redwater.

<span class="mw-page-title-main">Hereditary spherocytosis</span> Medical condition

Hereditary spherocytosis (HS) is a congenital hemolytic disorder, wherein a genetic mutation coding for a structural membrane protein phenotype leads to a spherical shaping of erythrocytic cellular morphology. As erythrocytes are sphere-shaped (spherocytosis), rather than the normal biconcave disk-shaped, their morphology interferes with these cells' abilities to be flexible during circulation throughout the entirety of the body - arteries, arterioles, capillaries, venules, veins, and organs. This difference in shape also makes the red blood cells more prone to rupture under osmotic and/or mechanical stress. Cells with these dysfunctional proteins are degraded in the spleen, which leads to a shortage of erythrocytes resulting in hemolytic anemia.

<span class="mw-page-title-main">Hemolytic anemia</span> Medical condition

Hemolytic anemia or haemolytic anaemia is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells (RBCs), either in the blood vessels or elsewhere in the human body (extravascular). This most commonly occurs within the spleen, but also can occur in the reticuloendothelial system or mechanically. Hemolytic anemia accounts for 5% of all existing anemias. It has numerous possible consequences, ranging from general symptoms to life-threatening systemic effects. The general classification of hemolytic anemia is either intrinsic or extrinsic. Treatment depends on the type and cause of the hemolytic anemia.

Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is used to describe reduced ('hypo-') splenic functioning, but not as severely affected as with asplenism.

An overwhelming post-splenectomy infection (OPSI) is a rare but rapidly fatal infection occurring in individuals following removal of the spleen. The infections are typically characterized by either meningitis or sepsis, and are caused by encapsulated organisms including Streptococcus pneumoniae. It is a medical emergency and requires immediate treatment. Death has been reported to occur within 12 hours.

<span class="mw-page-title-main">Splenomegaly</span> Medical condition

Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy. Splenomegaly is usually associated with increased workload, which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen, along with caput medusae, is an important sign of portal hypertension.

An autosplenectomy is a negative outcome of disease and occurs when a disease damages the spleen to such an extent that it becomes shrunken and non-functional. The spleen is an important immunological organ that acts as a filter for red blood cells, triggers phagocytosis of invaders, and mounts an immunological response when necessary. Lack of a spleen, called asplenia, can occur by autosplenectomy or the surgical counterpart, splenectomy. Asplenia can increase susceptibility to infection. Autosplenectomy can occur in cases of sickle-cell disease where the misshapen cells block blood flow to the spleen, causing scarring and eventual atrophy of the organ. Autosplenectomy is a rare condition that is linked to certain diseases but is not a common occurrence. It is also seen in systemic lupus erythematosus (SLE).

Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a type of myeloproliferative neoplasm, a group of cancers in which there is activation and growth of mutated cells in the bone marrow. This is most often associated with a somatic mutation in the JAK2, CALR, or MPL genes. In PMF, the bony aspects of bone marrow are remodeled in a process called osteosclerosis; in addition, fibroblast secrete collagen and reticulin proteins that are collectively referred to as (fibrosis). These two pathological processes compromise the normal function of bone marrow resulting in decreased production of blood cells such as erythrocytes, granulocytes and megakaryocytes, the latter cells responsible for the production of platelets.

<span class="mw-page-title-main">Howell–Jolly body</span> Cluster of DNA in red blood cells

A Howell–Jolly body is a cytopathological finding of basophilic nuclear remnants in circulating erythrocytes. During maturation in the bone marrow, late erythroblasts normally expel their nuclei; but, in some cases, a small portion of DNA remains. The presence of Howell–Jolly bodies usually signifies a damaged or absent spleen, because a healthy spleen would normally filter such erythrocytes.

<span class="mw-page-title-main">Codocyte</span> Type of red blood cell

Codocytes, also known as target cells, are red blood cells that have the appearance of a shooting target with a bullseye. In optical microscopy these cells appear to have a dark center surrounded by a white ring, followed by dark outer (peripheral) second ring containing a band of hemoglobin. However, in electron microscopy they appear very thin and bell shaped. Because of their thinness they are referred to as leptocytes. On routine smear morphology, some people like to make a distinction between leptocytes and codocytes- suggesting that in leptocytes the central spot is not completely detached from the peripheral ring, i.e. the pallor is in a C shape rather than a full ring.

<span class="mw-page-title-main">Splenic injury</span> Injury to the spleen

A splenic injury, which includes a ruptured spleen, is any injury to the spleen. The rupture of a normal spleen can be caused by trauma, such as a traffic collision.

<span class="mw-page-title-main">Extramedullary hematopoiesis</span> Medical condition

Extramedullary hematopoiesis refers to hematopoiesis occurring outside of the medulla of the bone. It can be physiologic or pathologic.

Felty's syndrome (FS), also called Felty syndrome, is a rare autoimmune disease characterized by the triad of rheumatoid arthritis, enlargement of the spleen and low neutrophil count. The condition is more common in those aged 50–70 years, specifically more prevalent in females than males, and more so in Caucasians than those of African descent. It is a deforming disease that causes many complications for the individual.

<span class="mw-page-title-main">Splenic infarction</span> Medical condition

Splenic infarction is a condition in which blood flow supply to the spleen is compromised, leading to partial or complete infarction in the organ. Splenic infarction occurs when the splenic artery or one of its branches are occluded, for example by a blood clot.

<i>Babesia</i> Genus of protozoan parasites

Babesia, also called Nuttallia, is an apicomplexan parasite that infects red blood cells and is transmitted by ticks. Originally discovered by the Romanian bacteriologist Victor Babeș in 1888, over 100 species of Babesia have since been identified.

Spleen transplantation is the transfer of spleen or its fragments from one individual to another. It is under research for induction of immunological tolerance for other transplanted organs. Success has been achieved in rodent models. Recently, evidence has been obtained for a tolerogenic effect of a spleen transplant in miniature swine. Also, the spleen harbors primitive hematopoietic progenitor cells. Spleen transplantation has been performed on humans with mixed results.

Cold autoimmune hemolytic anemia caused by cold-reacting antibodies. Autoantibodies that bind to the erythrocyte membrane leading to premature erythrocyte destruction (hemolysis) characterize autoimmune hemolytic anemia.

References

  1. ↑ Taniguchi, Leandro Utino; Correia, Mário Diego Teles; Zampieri, Fernando Godinho (December 2014). "Overwhelming post-splenectomy infection: narrative review of the literature". Surgical Infections. 15 (6): 686–693. doi:10.1089/sur.2013.051. ISSN   1557-8674. PMID   25318011.
  2. ↑ Weledji, Elroy P. (2014). "Benefits and risks of splenectomy". International Journal of Surgery (London, England). 12 (2): 113–119. doi: 10.1016/j.ijsu.2013.11.017 . ISSN   1743-9159. PMID   24316283.
  3. ↑ Cesta, Mark F. (2006). "Normal structure, function, and histology of the spleen". Toxicologic Pathology. 34 (5): 455–465. doi:10.1080/01926230600867743. ISSN   0192-6233. PMID   17067939. S2CID   39791978.
  4. 1 2 Di Sabatino, Antonio; Carsetti, Rita; Corazza, Gino Roberto (2011-07-02). "Post-splenectomy and hyposplenic states". Lancet. 378 (9785): 86–97. doi:10.1016/S0140-6736(10)61493-6. ISSN   1474-547X. PMID   21474172. S2CID   30554953.
  5. ↑ Frye R. (2006-03-02). "Porphyria, Cutaneous". eMedicine. Retrieved 2006-03-28.
  6. 1 2 Tarantino G, Scalera A, Finelli C (June 2013). "Liver-spleen axis: intersection between immunity, infections and metabolism". World Journal of Gastroenterology. 19 (23): 3534–42. doi: 10.3748/wjg.v19.i23.3534 . PMC   3691032 . PMID   23801854.
  7. ↑ "Babesiosis". National Institute of Allergy and Infectious Diseases, National Institutes of Health. 2009-02-19. Archived from the original on 2009-03-05.
  8. ↑ Gelfand, Jeffrey A.; Vannier, Edouard (6 March 2008). "Ch. 204: Babesiosis". Harrison's Principles of Internal Medicine, 17e. McGraw-Hill's Access Medicine. ISBN   978-0071466332.
  9. ↑ Working Party of the British Committee for Standards in Haematology Clinical Haematology Task Force (February 1996). "Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen". BMJ. 312 (7028): 430–4. doi:10.1136/bmj.312.7028.430. PMC   2350106 . PMID   8601117.
  10. ↑ Kolata G (9 November 2004). "A Diabetes Researcher Forges Her Own Path to a Cure". The New York Times.
  11. ↑ synd/1596 at Who Named It?
  12. ↑ Katcher AL (Mar 1980). "Familial asplenia, other malformations, and sudden death". Pediatrics. 65 (3): 633–5. doi:10.1542/peds.65.3.633. PMID   7360556. S2CID   25109676.
  13. ↑ Rodak B, Fritsma G, Doig K. Hematology: Clinical Principles and Applications.
  14. ↑ Irwin JJ, Kirchner JT (October 2001). "Anemia in children". American Family Physician. 64 (8): 1379–86. PMID   11681780.
  15. ↑ Kruetzmann S, Rosado MM, Weber H, Germing U, Tournilhac O, Peter HH, Berner R, Peters A, Boehm T, Plebani A, Quinti I, Carsetti R (April 2003). "Human immunoglobulin M memory B cells controlling Streptococcus pneumoniae infections are generated in the spleen". The Journal of Experimental Medicine. 197 (7): 939–45. doi:10.1084/jem.20022020. PMC   2193885 . PMID   12682112.
  16. ↑ Robinette CD, Fraumeni JF (July 1977). "Splenectomy and subsequent mortality in veterans of the 1939-45 war". Lancet. 2 (8029): 127–9. doi:10.1016/S0140-6736(77)90132-5. PMID   69206. S2CID   38605411.
  17. ↑ Grosfeld JL, Ranochak JE (June 1976). "Are hemisplenectomy and/or primary splenic repair feasible?". Journal of Pediatric Surgery. 11 (3): 419–24. doi:10.1016/S0022-3468(76)80198-4. PMID   957066.
  18. ↑ Bader-Meunier B, Gauthier F, Archambaud F, Cynober T, Miélot F, Dommergues JP, Warszawski J, Mohandas N, Tchernia G (January 2001). "Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis". Blood. 97 (2): 399–403. doi:10.1182/blood.V97.2.399. PMID   11154215. S2CID   22741973.
  19. ↑ Pratl B, Benesch M, Lackner H, Portugaller HR, Pusswald B, Sovinz P, Schwinger W, Moser A, Urban C (January 2008). "Partial splenic embolization in children with hereditary spherocytosis". European Journal of Haematology. 80 (1): 76–80. doi:10.1111/j.1600-0609.2007.00979.x. PMID   18028435. S2CID   41343243.
  20. ↑ Sheikha AK, Salih ZT, Kasnazan KH, Khoshnaw MK, Al-Maliki T, Al-Azraqi TA, Zafer MH (October 2007). "Prevention of overwhelming postsplenectomy infection in thalassemia patients by partial rather than total splenectomy". Canadian Journal of Surgery. 50 (5): 382–6. PMC   2386178 . PMID   18031639.
  21. ↑ Kimber C, Spitz L, Drake D, Kiely E, Westaby S, Cozzi F, Pierro A (June 1998). "Elective partial splenectomy in childhood". Journal of Pediatric Surgery. 33 (6): 826–9. doi:10.1016/S0022-3468(98)90651-0. PMID   9660206.
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