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Better outcome of splenectomy in younger patients suffering from chronic immune thrombocytopenia (ITP)

Sabah Saqib,Rizwan Sultan,Hasnain Zafar  ( Department of Surgery, Aga Khan University Hospital, Karachi. )

October, 2016

Abstract

Immune thrombocytopenia purpura (ITP) may need splenectomy after failure of medical treatment. The aim of this study was to explore the outcome of splenectomy in chronic ITP and to point out factors which can predict better response to splenectomy. This retrospective chart review was conducted at the Aga Khan University Hospital, Karachi, and comprised adult patients who underwent splenectomy for ITP from October 2005 to December 2015. Of the 51 patients, 37(72.5%) were females and 14(27.5%) were males. The overall median age was 32 years (interquartile range: 18-65 years). Complete response was seen in 43(84.3%) patients, 2(4%) had response and 6(11.7%) had no response. Relapse rate of ITP at 1 year was 4(8.8%). Multivariate analysis showed that failure rate of splenectomy in the 41(80.4%) patients aged <50 years was 3(7%) as opposed to 3(30%) in the 10(19.6%) patients aged >50 years (p=0.04). Splenectomy was found to be a safe and effective option for treatment of ITP. Young age at the time of surgery was associated with good response to surgery.
Keywords: Idiopathic thrombocytopenic purpura, Splenectomy, Response, Platelet.

 

Introduction

Immune thrombocytopenia purpura (ITP), also known as idiopathic thrombocytopenia purpura, is a haematological disorder in which there is premature destruction of platelets. The disease is characterised by peripheral thrombocytopenia, normal spleen and the absence of other causes of decrease in platelets.1 The disease can be categorised as acute ITP, defined as thrombocytopenia occurring for <6 months, and often occurs in children and young adults with no sex predilection and is usually resolved spontaneously, or chronic idiopathic ITP, which is more common in adults aged 20-40 years, lasting >6 months with more cases in female, and very uncommon spontaneous remission.2

The diagnosis of chronic ITP is usually a diagnosis of exclusion. Many patients experience either no symptoms or minimal bruising, whereas others experience severe bleeding, which may include gastrointestinal bleeding, extensive skin and mucosal haemorrhage, or intracranial haemorrhage. The management of chronic ITP starts from observation to corticosteroids-based therapy, use of immunosuppressant and thrombopoietin (TPO)-receptor agonist, and eventually splenectomy when medical management fails or is contraindicated. Although the precise mechanism of splenectomy in chronic ITP is still not known, it is believed that in ITP spleen is the site of autoantibodies production against platelets and eventually these antibodies-coated platelets are destroyed in the spleen. Hence, if the spleen is removed, platelets will remain able to survive in circulation. But platelets can also be removed from the liver or combination of liver and spleen, so splenectomy is not always successful. Much work is being done to see response of ITP to splenectomy before surgery3 but to date there is no proven data in this regard.

The current study was planned to see the response of splenectomy in chronic ITP and to find out factors which can predict good response.

 

Methods and Results

This retrospective chart review was conducted at the Aga Khan University Hospital (AKUH), Karachi, Pakistan, and comprised patients having undergone splenectomy for ITP from October 2005 to December 2015. All patients were evaluated and diagnosed to have chronic ITP by a team of haematologists and after adequate medical treatment they were referred for splenectomy as a treatment option for ITP. All patients received immunisation before surgery or two weeks after splenectomy. For all patients, 1 mega unit of platelets was arranged. Half volume of mega unit was transfused before incision and half before clamping hilar vessels of spleen. Intra-operative nasogastric (NG) tube and left sub-diaphragmatic drain were placed in all patients. All patients were kept in high dependency unit for one day after surgery. NG tube was removed on the first post-operative day while the drain was removed before discharge if output remained <30ml/day. Aspirin was started if the platelet count was >1,000x109. Patients\\\' platelets were checked post-operatively. To analyse outcome platelet count on first follow-up visit was checked approximately 2 weeks after surgery to avoid any false increase in the count because of pre-operative transfusions. The patients\' platelet count was checked at 1, 3, 6 and 12 months post-operatively. Response to splenectomy was categorised according to American Society of Haematology\'s guidelines of 20114 and grouped as 'complete response\' if post-operative platelets count increased by more than 100x109, 'partial\' if platelets count remained between 30x109 to 100x109 and \'no response\' if it remained less than 30x109. Patients with complete and partial response were grouped as responders and the remainder as non-responders. Relapse was defined as a decrease in platelet count to less than 50×109/l5 after showing initial response to splenectomy. A value of 50×109 was chosen because above this level most patients do not require any form of therapy. Data was analysed using SPSS 19. 

Of the 51 patients, 14(27.5%) were males and 37(72.5%) were females. The overall median age at the time of splenectomy was 32 years (interquartile range: 18-65 years). Besides, 20(39.2%) patients underwent spleenectomy within one year of diagnosis while 31(60.8%) patients were aware of their diagnosis for more than one year. The mean platelets count before surgery was 21.7 x 109±20x109. Of the total, 48(94.1%) patients had received medical treatment prior to surgery as first line of treatment and only 3(5.9%) patients directly underwent surgery. Of the former, 25(49%) patients received only steroids, whereas 23(45.1%) received steroids and immunosuppressants.

In addition, 22(43.1%) patients underwent open splenectomy, 22(43.1%) laparoscopies and 7(13.8%) required conversion from laparoscopic to an open procedure. The mean length of hospital stay was 6±4 days. Complete response was achieved in 43(84.3%) patients, whereas response was noticed in 2(3.9%) and no response in 6(11.7%) patients. Only 1(2%) patient expired because of post-operative sepsis. Complications among patients included intra-abdominal collection 2(3.9%), post-operative chest infection 4(7.8%) and post-operative haemorrhage requiring pack cells transfusion in 8(15.7%) patients. Moreover, 4(7.8%) patients showed relapse at overall follow-up of 1 year and were labelled as refractory to splenectomy. Normal size spleen was found on histopathology in all patients (Table-1)

Of all, 45(88.2%) patients were responders and 6(11.8%) were non-responders. Of the 41(80.4%) patients aged <50 years, 38(92.7%) were responders and 3(7.3%) were non-responders, whereas of the 10(19.6%) patients aged>50 years, 7(70%) were responders and 3(30%) were non-responders (p=0.046). Of the 39(76.5%) patients having pre-operative platelets count of <25,000, 33(84.6%) were responders and 6(15.4%) were not, whereas all of the 12(23.5%) patients who had platelets count of >25,000 were responders (p=0.148) (Table-2)

 

 

Conclusion

This retrospective study at a single institute showed a response rate of 84.3% to splenectomy and a mean increase in platelets in responders after 1 year was 242.8x109 requiring no further treatment in these patients. Moreover, 8.8% patients had relapsed with an initial increase in platelets but after 6 months of surgery, it decreased to less than 50x109. These patients required additional medical treatment. Besides, 11.7% patients had no response to splenectomy. These patients had no increase in platelets at all. A similar kind of study done in six European haematology centres6 showed overall response of 88%, a Italian study7 reported a rate of 86%, whereas a study conducted in South Korea8 showed a response rate of 73%. These results are quite comparable to our study which had overall response of 84.3%.

In our study, when the variables of responders and non-responders were compared, age below 50 years was statistically significant (p=0.04) for better response after splenectomy. This factor has been found persistently significant in literature in previous studies as well. A systematic review9 in 2004 showed better outcome of splenectomy in younger age group of 32 to 51 years.

The current study was a retrospective audit with a small number of patients over 10 years showing that disease is not much prevalent in our part of world. Our study showed that the response rate to splenectomy in our population was better. The exact aetiology of this better response is not known, but it may be because of genetic or autoimmune factors which need to be looked at, as these may give us clue to improving treatment results in non-responders.

 

References

1.Nugent D, McMillan R, Nichol JL, Slichter SJ. Pathogenesis of chronic immune thrombocytopenia: increased platelet destruction and/or decreased platelet production. Br J Haematol 2009; 146: 585-96.

2.Cines DB, Blanchette VS. Immune thrombocytopenic purpura. N Engl J Med 2002; 346: 995-1008.

3.Park YH, Yi HG, Kim CS, Hong J, Park J, Lee J, et al. Clinical Outcome and Predictive Factors in the Response to Splenectomy in Elderly Patients with Primary Immune Thrombocytopenia: A Multicenter Retrospective Study. Acta Haematol 2016; 135: 162-71.

4.Neunert C, Lim W, Crowther M, Cohen A, Solberg L, Crowther MA. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood 2011; 117: 4190-207.

5.Kumar S, Diehn F, Gertz M, Tefferi A. Splenectomy for immune thrombocytopenic purpura: long-term results and treatment of postsplenectomy relapses. Ann Hematol 2002; 81: 312-9.

6.Vianelli N, Palandri F, Polverelli N, Stasi R, Joelsson J, Johansson E, et al. Splenectomy as a curative treatment for immune thrombocytopenia: a retrospective analysis of 233 patients with a minimum follow up of 10 years. Haematologica 2013; 98: 875-80.

7.Baccarani U, Terrosu G, Donini A, Zaja F, Bresadola F, Baccarani M. Splenectomy in hematology. Current practice and new perspectives. Haematologica 1999; 84: 431-6.

8.Hurford WE, Hong S, Park YS, Ahn D, Shiraki K, Mohri M, et al. Splenic contraction during breath-hold diving in the Korean ama. J Appl Physiol (1985) 1990; 69: 932-6.

9.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: 2623-34.


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