Splenectomy


A splenectomy is the surgical removal of the spleen, a small, hand-sized organ located in front of the left kidney and behind the stomach. The spleen acts like a large lymph node, helping to maintain a healthy immune system and cleaning the blood of foreign matter.

In ITP, the antibody-coated platelets are often removed from circulation by the spleen. Theoretically, if the spleen is removed, the platelets will remain in the blood stream. The spleen can also be the site of antibody production. Therefore removing the spleen may reduce the amount of anti-platelet antibodies in addition to removing the antibody-coated platelets.

Although the spleen is often the major site of antibody-coated platelet destruction, platelets may also be removed from circulation by the liver, by a combination of the spleen and liver, or within the blood stream. Therefore, splenectomies are not always successful in raising the platelet count and may fail over time, prompting a return of low platelets.


Treatment History

Splenectomies have been used to treat ITP since 1913. There are two types of splenectomies: laparoscopic where the spleen is removed through a few small holes in the abdomen and open, requiring a large incision. The laparoscopic splenectomy is preferred, when possible, since the healing time is reduced. It has the same rate of success as an open splenectomy and there are fewer complications.1

While a splenectomy may raise the platelet count, it does not eliminate ITP since the antibody-coated platelets remain in circulation. In pregnancy, these antibody-coated platelets may cross the placenta and have the potential for reducing the platelet count of the newborn.

Doctors vaccinate those about to have a splenectomy with polyvalent pneumococcal, meningococcal C conjugate, and H influenza b (Hib) vaccines. Timeframes may be different for those on other immune suppressing therapies.3

A small percent of the splenectomized ITP population develops an accessory (extra) spleen. Occasionally, a second surgery is suggested to remove the accessory spleen if the patient has relapsed following a successful first surgery.3


Side Effects

The immediate complication rate from surgery is about 10 percent, although estimates vary. The fatality rate from the surgery is about one percent for an open splenectomy and much less for a laproscopic procedure. Patients over 65 have a higher complication and fatality rate.3

Since the spleen is responsible for making antibodies, filtering the blood, and removing bacteria, those without a spleen have an impaired immune system. Because of this, splenectomized patients have a more difficult time recovering from pneumonia, meningitis, Hib flu,3 sepsis,9 hospital-based infections,7 malaria and other parasitic diseases,5 babesiosis (a tick-borne disease)10and gram-negative bacterial diseases from animal bites.11

People who have had a splenectomy have more microparticles in their blood, giving them an increased risk of dementia4 and heart attacks6 from blood clots. They are also more prone to blood vessel complications.8


Predicting Success

About 10 to 15 percent of people have no meaningful response to the operation. For those who do respond, from 30 to 35 percent relapse over time.16  The published success rates are about 66 percent, although the measurement criteria for success and the duration of follow-up are not standardized in the studies.1 Splenectomies are more successful and last longer in younger people (under 40 years of age).2

Other Treatments as a Predictor
Previous response to corticosteroids (such as Prednisone), IVIG or other treatments are not very good predictors of splenectomy success.3, 12, 13

Age as Predictor 
A study reported in the British Journal of Haematology  (March 2001) found "the only positive predictive factor for the long-term response to splenectomy was age <40 years."2

Indium Screening Test as Predictor
Researchers in the United Kingdom, France and Spain feel that an indium white blood cell screening test has some value in predicting whether a splenectomy will successfully raise your platelet counts. The test determines whether your spleen, liver, or a combination of both is responsible for your platelet destruction.14, 15

  • Barts and the Royal London Hospital, London, UK  - Click here for contact information to schedule a scan at this hospital.
  • Hospital Universitari de Bellvitge, Barcelona, Spain - Contact Dr. Jaume Mora, Nuclear Medicine Specialist, jmoras@bellvitgehospital.cat, and Dr. Manel Roca, Radiopharmacist, mrocae@bellvitgehospital.cat. This test is part of their Nuclear Medicine practice. They do a maximum of two tests per week for adults only. The test starts on a Tuesday and ends on Friday the same week or Monday of next week, according to the results. If you are interested in the test send an e-mail to the contacts and include your complete name, date of birth, address, e-mail and possible procedure dates.

Haptoglobin (Hp) as a Predictor
Researchers performed a detailed analysis of the blood, pre and post operation, of people undergoing splenectomy searching for some blood protein that was different in those who responded to splenectomy, versus those who didn’t. The researchers found that haptoglobin (Hp), a protein that binds to free hemoglobin released by red blood cells, of non-responders before the operation was significantly lower than those who responded or the healthy controls. The researchers suggested their theory predicted splenectomy success in about 80 percent of the cases.17


References

  1. Kojouri K, et al, "Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications". Blood.2004 Nov 1: 104(9): 2623-34 http://www.ncbi.nlm.nih.gov/pubmed/15217831
  2. Fabris F, et al, "Age as the Major Predictive Factor of Long-Term Response to Splenectomy in Immune Thrombocytopenia Purpura", British Journal of Haematology, 2001, vol. 112 pp 637 -640. http://www.ncbi.nlm.nih.gov/pubmed/11260065
  3. Provan A, et al. "International consensus report on the investigation and management of primary immune thrombocytopenia," Blood, 14 January 2010, Vol. 115, No. 2, pp. 168-186.http://bloodjournal.hematologylibrary.org/cgi/content/full/115/2/168#B110
  4. Ahn YS, et al, "Vascular dementia in patients with immune thrombocytopenic purpura" J Clin Lab Med 119:334, 1992; Throm Res 107: 337, 2002 http://www.ncbi.nlm.nih.gov/pubmed/12565721
  5. Chotivanich K, et al, "Central role of the spleen in malaria parasite clearance." J Infect Dis.  2002 May15: 185(10):1538-41http://www.ncbi.nlm.nih.gov/pubmed/11992295
  6. Fontana V, et al, "Increased procoagulant cell-derived microparticles (C-MP) in splenectomized patients with ITP".Thromb Res. 2008: 122(5):599-603. .http://www.ncbi.nlm.nih.gov/pubmed/18334267
  7. Thomsen RW et al, "Risk for hospital contact with infection in patients with splenectomy: a population-based cohort study." Ann Intern Med. 2009, Oct 20; 151(8):546-55.http://www.ncbi.nlm.nih.gov/pubmed/19841456
  8. Crary SE, et al, "Vascular complications after splenectomy for hematologic disorders." Blood. 2009 Oct 1: 114 (14):2861-8 http://www.ncbi.nlm.nih.gov/pubmed/19636061
  9. Sarangi J, et al, "Prevention of post splenectomy sepsis: a population based approach." J Public Health Med. 1997 Jun: 19(2):208-12.http://www.ncbi.nlm.nih.gov/pubmed/9243438
  10. Hohenschild S et al,"Babesiosis--a dangerous infection for splenectomized children and adults, KlinPadiatr." 1999 May-Jun: 211(3): 137-40. http://www.ncbi.nlm.nih.gov/pubmed/10412122
  11. http://emedicine.medscape.com/article/224920-treatment
  12. Law C, et al, "High-dose intravenous immune globulin and the response to splenectomy in patients with idiopathic thrombocytopenic purpura." N Engl J Med. 1997: 336(21):1494–1498.http://content.nejm.org/cgi/content/abstract/336/21/1494
  13. Bussel JB, et al, "Do the acute platelet responses of patients with immune thrombocytopenic purpura (ITP) to IV anti-D and to IV gammaglobulin predict response to subsequent splenectomy?" Am JHematol. 2001: 67(1):27–33. http://www.ncbi.nlm.nih.gov/pubmed/11279654
  14. Najean Y, et al, "The site of platelet destruction in thrombocytopenic purpura as a predictive index of the efficacy of splenectomy." 1992 British Journal of Haematology,1992: 79, 271-276. http://www.ncbi.nlm.nih.gov/pubmed/1958485
  15. Najean Y, et al,  "The site of destruction of autologous 111In-labelled platelets and the efficiency of splenectomy in children and adults with idiopathic thrombocytopenic purpura: a study of 578 patients with 268 splenectomies." British Journal of Haematology 1997, 97, 547-550. http://www.ncbi.nlm.nih.gov/pubmed/9207397
  16. Stasi R. “ITP, interrupted.” Blood. 2011 Oct 20;118(16):4297-8.http://bloodjournal.hematologylibrary.org/content/118/16/4297.long
  17. Zheng CX et al. “Proteomics-based identification of haptoglobin as a favourable serum biomarker for predicting long-term response to splenectomy in patients with primary immune thrombocytopenia.” RJ Transl Med. 2012 Oct 7;10(1):208.  http://www.translational-medicine.com/content/10/1/208/abstract 

 

 

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