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Immune Thrombocytopenia (ITP)

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Surgery

Immune thrombocytopenia (ITP) is an autoimmune condition in which autoantibodies attack proteins on the surface membranes of platelets. This leads to sequestration of autoantibody-platelet complexes in the spleen and premature destruction via phagocytosis by mononuclear macrophages within the spleen.1

The decreased number of functional, circulating platelets combined with the inability of megakaryocytes within the bone marrow to compensate with increased platelet production results in thrombocytopenia.1 

Low platelet levels increase bleeding risk, often (but not always) resulting in bruising (purpura). If platelet levels decrease below 10,000/µL, serious, life-threatening bleeding complications, including intracranial, uterine, or gastrointestinal hemorrhage, may occur.1,2

Perioperative Care

Surgical procedures for patients with ITP must be managed with caution due to the increased risk of bleeding. Preoperative thrombocytopenia has been shown to correlate with higher risks of transfusion, intraoperative and postoperative complications, hospital readmission, long-term care, reoperation, and mortality.3,4

Perioperative Procedures for Elective ITP Surgeries

Elective surgeries with flexible scheduling can employ various prophylactic procedures to elevate a patient’s preoperative platelet counts to safe levels and decrease the risk of hemorrhaging during surgery. Such prophylactic procedures may include medications, including properly timed glucocorticoids and/or intravenous immunoglobulin treatments to raise platelet counts prior to the operation.5 

Limited data exist on the efficacy of preoperative thrombopoietin receptor agonist (TPO-RA) administration in patients with ITP. One systematic review of 2 TPO mimetics, romiplostim and eltrombopag, demonstrated that although these agents effectively increased platelet counts, patients with ITP did not experience a significant reduction in bleeding risk.5-8

Read more about ITP therapies

Perioperative Procedures for Emergency ITP Surgeries

Patients with ITP who require emergency surgeries usually lack the time required to elevate their platelet counts prior to the operation, yet they still need specialized care for successful surgeries. This often means blood transfusions, specifically platelet transfusions. Platelet transfusions are recommended prior to emergency surgeries if platelet levels are below 50,000/µL. For specific procedures involving neurosurgery or ocular surgery, platelet transfusion may be recommended at even higher thresholds (<100,000/µL).5

Following any surgeries, patients with ITP should be closely monitored for postoperative thrombocytopenia or bleeding that may require platelet or blood transfusions.3

A study conducted in 2015 demonstrated that platelet transfusions increased the risk of arterial thrombosis and death in hospitalized patients with heparin-induced thrombocytopenia and thrombotic thrombocytopenia. In contrast, patients with ITP did not share this elevated risk of arterial thrombosis or mortality following platelet transfusions.9

Read more about ITP prognosis

Splenectomy

Over the past 25 years, the pharmaceutical management of ITP has advanced, offering many first-line, second-line and refractory treatment options. Prior to these advances, splenectomy was the standard treatment of choice for patients with ITP. The use of splenectomy to treat ITP has declined following their development. However, splenectomy still remains a relevant treatment option, albeit much later in the course of the disease and particularly for those with severe hemorrhaging and chronic, refractory ITP that has not responded to multiple therapy options.10-12

Splenectomy removes the primary location of ITP pathogenesis. In most patients with ITP, splenic macrophages destroy antibody-coated platelets that are sequestered in the spleen. The spleen is also the site where immune and plasma cells produce antiplatelet antibodies.11

Read more about ITP pathophysiology

Compared with other ITP treatments, splenectomy results in the highest response rate (50% to 70% durable remission), with around 80% of patients exhibiting an immediate postsplenectomy elevation of platelet levels. One retrospective, multicenter study demonstrated an 86% response rate (66% complete, 20% partial response) postsplenectomy in 402 patients with ITP. After a median follow-up of 92 months, 75% of these responses were stable.11,13 

However, guidelines recommend the avoidance of splenectomy for the first 12 months following ITP diagnosis to allow for spontaneous or therapy-induced remission, especially in patients of advanced age with a higher risk of postsurgical morbidity. Additionally, providers must weigh the risk of postsplenectomy complications against possible benefits of the operation. Postsplenectomy complications include a long-term increased risk of infections, sepsis from encapsulated bacteria or Babesia, pulmonary hypertension, and cardiovascular problems due to increased procoagulant status, especially venous thromboembolism, arterial thrombosis, and cardiovascular disease.11,14

Pediatricians recommend that splenectomies are delayed in children until they are at least 5 years of age due to the 1% to 2% increased risk of bacterial sepsis following splenectomy.14 To complicate matters, while postsplenectomy response rates are generally high, there are no reliable predictors of response in patients with ITP.11

Recurrent ITP may occur in patients with accessory spleens that are missed during open or laparoscopic splenectomy. One study reviewed ITP recurrence rates after open (n=54) and laparoscopic (n=51) splenectomies performed in 105 patients with ITP at 4 sites over 18 years. Recurrent ITP occurred in 27.6% of patients who underwent open splenectomies and 14.6% of patients who underwent laparoscopic splenectomies. None of these recurrent cases resulted from missed accessory spleens in either group.15

Read more about ITP risk factors

References

  1. Kessler CM. Immune thrombocytopenia (ITP): practice essentials. Medscape. Updated January 7, 2021. Accessed October 25, 2022.
  2. Arnold DM. Bleeding complications in immune thrombocytopenia. Hematology Am Soc Hematol Educ Program. 2015;2015(1):237-242. doi:10.1182/asheducation-2015.1.237
  3. Hess AS, Ramamoorthy J, Hess JR. Perioperative platelet transfusions. Anesthesiology. 2021;134(3):471-479. doi:10.1097/ALN.0000000000003670
  4. Weil IA, Kumar P, Seicean S, Neuhauser D, Seicean A. Platelet count abnormalities and peri-operative outcomes in adults undergoing elective, non-cardiac surgery. PLoS One. 2019;14(2):e0212191. doi:10.1371/journal.pone.0212191
  5. Graetz TJ, Nuttall G, Shander A. Perioperative blood management: strategies to minimize transfusions. UpToDate. Updated May 20, 2022. Accessed October 25, 2022.
  6. Al-Samkari H, Marshall AL, Goodarzi K, Kuter DJ. Romiplostim for the management of perioperative thrombocytopenia. Br J Haematol. 2018;182(1):106-113. doi:10.1111/bjh.15280
  7. Estcourt LJ, Malouf R, Doree C, Trivella M, Hopewell S, Birchall J. Prophylactic platelet transfusions prior to surgery for people with a low platelet count. Cochrane Database Syst Rev. 2018;9(9):CD012779. doi:10.1002/14651858.CD012779.pub2
  8. Zeng Y, Duan X, Xu J, Ni X. TPO receptor agonist for chronic idiopathic thrombocytopenic purpura. Cochrane Database Syst Rev. 2011;7:CD008235. doi:10.1002/14651858.CD008235.pub2
  9. Goel R, Ness PM, Takemoto CM, Krishnamurti L, King KE, Tobian AAR. Platelet transfusions in platelet consumptive disorders are associated with arterial thrombosis and in-hospital mortality. Blood. 2015;125(9):1470-1476. doi:10.1182/blood-2014-10-605493
  10. Remiker A, Neunert C. Splenectomy for immune thrombocytopenia: the evolution and preservation of treatment. Haematologica. 2020;105(11):2507-2509. doi:10.3324/haematol.2020.261099
  11. Chaturvedi S, Arnold DM, McCrae KR. Splenectomy for immune thrombocytopenia: down but not out. Blood. 2018;131(11):1172-1182. doi:10.1182/blood-2017-09-742353
  12. Kojouri K, Vesely SK, Terrell DR, George JN. 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;104(9):2623-2634. doi:10.1182/blood-2004-03-1168
  13. Vianelli N, Galli M, de Vivo A, et al; Gruppo Italiano per lo Studio delle Malattie Ematologiche dell’Adulto. Efficacy and safety of splenectomy in immune thrombocytopenic purpura: long-term results of 402 cases. Haematologica. 2005;90(1):72-77. doi:10.3324/%25x
  14. Kessler CM. Immune thrombocytopenia (ITP) treatment & management: surgical care. Medscape. Updated January 7, 2021. Accessed October 25, 2022.
  15. Sampath S, Meneghetti AT, MacFarlane JK, Nguyen NH, Benny WB, Panton ONM. An 18-year review of open and laparoscopic splenectomy for idiopathic thrombocytopenic purpura. Am J Surg. 2007;193(5):580-584. doi:10.1016/j.amjsurg.2007.02.002

Reviewed by Kyle Habet, MD, on 10/31/2022.

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