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 \u3c50 years was 3(7%) as opposed to 3(30%) in the 10(19.6%) patients aged \u3e50 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

Rizwan Hashim,

Saqib, Sabah

Zafar, Hasnain

English

eCommons@AKU

eCommons@AKUDepartment of Surgery Department of SurgeryOctober 2016Better outcome of splenectomy in younger patientssuffering from chronic immune thrombocytopeniaSabah SaqibAga Khan University, sabah.saqib@aku.eduRizwan HashimAga Khan UniversityHasnain ZafarAga Khan University, hasnain.zafar@aku.eduFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_surgPart of the Surgery CommonsRecommended CitationSaqib, S., Rizwan Hashim, ., Zafar, H. (2016). Better outcome of splenectomy in younger patients suffering from chronic immunethrombocytopenia. JPMA: Journal of Pakistan Medical Association, 66(10), S-62-S-64.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_surg_surg/111AbstractImmune thrombocytopenia purpura (ITP) may needsplenectomy after failure of medical treatment. The aim ofthis study was to explore the outcome of splenectomy inchronic ITP and to point out factors which can predictbetter response to splenectomy. This retrospective chartreview was conducted at the Aga Khan UniversityHospital, Karachi, and comprised adult patients whounderwent splenectomy for ITP from October 2005 toDecember 2015. Of the 51 patients, 37(72.5%) werefemales and 14(27.5%) were males. The overall medianage 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 rateof ITP at 1 year was 4(8.8%). Multivariate analysis showedthat failure rate of splenectomy in the 41(80.4%) patientsaged <50 years was 3(7%) as opposed to 3(30%) in the10(19.6%) patients aged >50 years (p=0.04). Splenectomywas found to be a safe and effective option for treatmentof ITP. Young age at the time of surgery was associatedwith good response to surgery.Keywords: Idiopathic thrombocytopenic purpura,Splenectomy, Response, Platelet.IntroductionImmune thrombocytopenia purpura (ITP), also known asidiopathic thrombocytopenia purpura, is ahaematological disorder in which there is prematuredestruction of platelets. The disease is characterised byperipheral thrombocytopenia, normal spleen and theabsence of other causes of decrease in platelets.1 Thedisease can be categorised as acute ITP, defined asthrombocytopenia occurring for <6 months, and oftenoccurs in children and young adults with no sexpredilection and is usually resolved spontaneously, orchronic idiopathic ITP, which is more common in adultsaged 20-40 years, lasting >6 months with more cases infemale, and very uncommon spontaneous remission.2The diagnosis of chronic ITP is usually a diagnosis ofexclusion. Many patients experience either no symptomsor minimal bruising, whereas others experience severebleeding, which may include gastrointestinal bleeding,extensive skin and mucosal haemorrhage, or intracranialhaemorrhage. The management of chronic ITP starts fromobservation to corticosteroids-based therapy, use ofimmunosuppressant and thrombopoietin (TPO)-receptoragonist, and eventually splenectomy when medicalmanagement fails or is contraindicated. Although theprecise mechanism of splenectomy in chronic ITP is stillnot known, it is believed that in ITP spleen is the site ofautoantibodies production against platelets andeventually these antibodies-coated platelets aredestroyed in the spleen. Hence, if the spleen is removed,platelets will remain able to survive in circulation. Butplatelets can also be removed from the liver orcombination of liver and spleen, so splenectomy is notalways successful. Much work is being done to seeresponse of ITP to splenectomy before surgery3 but todate there is no proven data in this regard.The current study was planned to see the response ofsplenectomy in chronic ITP and to find out factors whichcan predict good response.Methods and ResultsThis retrospective chart review was conducted at the AgaKhan University Hospital (AKUH), Karachi, Pakistan, andcomprised patients having undergone splenectomy forITP from October 2005 to December 2015. All patientswere evaluated and diagnosed to have chronic ITP by ateam of haematologists and after adequate medicaltreatment they were referred for splenectomy as atreatment option for ITP. All patients receivedimmunisation before surgery or two weeks aftersplenectomy. For all patients, 1 mega unit of platelets wasarranged. Half volume of mega unit was transfused beforeincision and half before clamping hilar vessels of spleen.Intra-operative nasogastric (NG) tube and left sub-diaphragmatic drain were placed in all patients. Allpatients were kept in high dependency unit for one dayafter surgery. NG tube was removed on the first post-operative day while the drain was removed beforedischarge if output remained <30ml/day. Aspirin wasstarted if the platelet count was >1,000x109. Patients'platelets were checked post-operatively. To analyseJ Pak Med Assoc (Suppl. 3)S-62 2nd Annual Surgical Meeting 2016Department of Surgery, Aga Khan University Hospital, Karachi.Correspondence: Sabah Saqib. Email: sabah.saqib@aku.eduSHORT REPORTBetter outcome of splenectomy in younger patients suffering from chronicimmune thrombocytopenia (ITP)Sabah Saqib, Rizwan Sultan, Hasnain Zafaroutcome platelet count on first follow-up visit waschecked approximately 2 weeks after surgery to avoid anyfalse increase in the count because of pre-operativetransfusions. The patients' platelet count was checked at1, 3, 6 and 12 months post-operatively. Response tosplenectomy was categorised according to AmericanSociety of Haematology's guidelines of 20114 andgrouped as 'complete response' if post-operative plateletscount increased by more than 100x109, 'partial' if plateletscount remained between 30x109 to 100x109 and 'noresponse' if it remained less than 30x109. Patients withcomplete and partial response were grouped asresponders and the remainder as non-responders.Relapse was defined as a decrease in platelet count to lessthan 50×109/l5 after showing initial response tosplenectomy. A value of 50×109 was chosen becauseabove this level most patients do not require any form oftherapy. 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 ofsplenectomy was 32 years (interquartile range: 18-65years). Besides, 20(39.2%) patients underwentspleenectomy within one year of diagnosis while31(60.8%) patients were aware of their diagnosis for morethan one year. The mean platelets count before surgerywas 21.7 x 109±20x109. Of the total, 48(94.1%) patientshad received medical treatment prior to surgery as firstline of treatment and only 3(5.9%) patients directlyunderwent surgery. Of the former, 25(49%) patientsreceived only steroids, whereas 23(45.1%) receivedsteroids and immunosuppressants.In addition, 22(43.1%) patients underwent opensplenectomy, 22(43.1%) laparoscopies and 7(13.8%)required conversion from laparoscopic to an openprocedure. The mean length of hospital stay was 6±4days. Complete response was achieved in 43(84.3%)patients, whereas response was noticed in 2(3.9%) and noresponse in 6(11.7%) patients. Only 1(2%) patient expiredbecause of post-operative sepsis. Complications amongpatients included intra-abdominal collection 2(3.9%),post-operative chest infection 4(7.8%) and post-operativehaemorrhage requiring pack cells transfusion in 8(15.7%)patients. Moreover, 4(7.8%) patients showed relapse atoverall follow-up of 1 year and were labelled as refractoryto splenectomy. Normal size spleen was found onhistopathology 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 <50years, 38(92.7%) were responders and 3(7.3%) were non-responders, whereas of the 10(19.6%) patients aged>50years, 7(70%) were responders and 3(30%) were non-responders (p=0.046). Of the 39(76.5%) patients havingpre-operative platelets count of <25,000, 33(84.6%) wereresponders and 6(15.4%) were not, whereas all of the12(23.5%) patients who had platelets count of >25,000were responders (p=0.148) (Table-2).ConclusionThis retrospective study at a single institute showed aresponse rate of 84.3% to splenectomy and a meanincrease in platelets in responders after 1 year was242.8x109 requiring no further treatment in thesepatients. Moreover, 8.8% patients had relapsed with aninitial increase in platelets but after 6 months of surgery, itdecreased to less than 50x109. These patients requiredadditional medical treatment. Besides, 11.7% patients hadno response to splenectomy. These patients had noincrease in platelets at all. A similar kind of study done inVol. 66, No.10 (Suppl. 3), October 20162nd Annual Surgical Meeting 2016 S-63Table-1: Outcomes of splenectomy.Variable FrequencyMean length of hospital stay 6±4 daysMortality 1(1.96%)ComplicationsAbdominal collection 2(3.92%)Chest infection 4(7.8%)Need for packed cell transfusion 8(15.6%)OutcomeComplete response 43(84.3%)Partial response 2(4%)No response 6(11.7%)Relapse at 1 year 4 (8.8%)Table-2:Multivariate Analysis for predictive factors for Response to Splenectomy.Variables Responders Non responders P valuen=44 n=6GenderMale 11 (78.6%) 3 (21.4%)Female 33 (91.9%) 4 (8.1%) 0.18Age<50 years 38 (92.6%) 3 (7.4%)>50 years 7 (70%) 3 (30%) 0.046Timing of surgery<1 year 16 (80%) 4 (20%)>1 year 29 (93.5%) 2 (6.5%) 0.143Preoperative platelets<25000 33 (84.7%) 6 (15.3%)>25000 12 (100%) 0 (0%) 0.148Symptomatic 9 (100%) 0 (0%)Asymptomatic 36(85.8%) 6 (14.2%) 0.227Prior Medical managementNo or single medical therapy 27(93.1%) 2(6.9%)Multimodal therapy 18 (82%) 4 (18%) 0.215six European haematology centres6 showed overallresponse of 88%, a Italian study7 reported a rate of 86%,whereas a study conducted in South Korea8 showed aresponse rate of 73%. These results are quite comparableto 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 wasstatistically significant (p=0.04) for better response aftersplenectomy. This factor has been found persistentlysignificant in literature in previous studies as well. Asystematic review9 in 2004 showed better outcome ofsplenectomy in younger age group of 32 to 51 years.The current study was a retrospective audit with a smallnumber of patients over 10 years showing that disease isnot much prevalent in our part of world. Our studyshowed that the response rate to splenectomy in ourpopulation was better. The exact aetiology of this betterresponse is not known, but it may be because of geneticor autoimmune factors which need to be looked at, asthese may give us clue to improving treatment results innon-responders.References1. Nugent D, McMillan R, Nichol JL, Slichter SJ. Pathogenesis ofchronic immune thrombocytopenia: increased plateletdestruction and/or decreased platelet production. Br J Haematol2009; 146: 585-96.2. Cines DB, Blanchette VS. Immune thrombocytopenic purpura. NEngl J Med 2002; 346: 995-1008.3. Park YH, Yi HG, Kim CS, Hong J, Park J, Lee J, et al. ClinicalOutcome and Predictive Factors in the Response toSplenectomy in Elderly Patients with Primary ImmuneThrombocytopenia: A Multicenter Retrospective Study. ActaHaematol 2016; 135: 162-71.4. Neunert C, Lim W, Crowther M, Cohen A, Solberg L, Crowther MA.The American Society of Hematology 2011 evidence-basedpractice guideline for immune thrombocytopenia. Blood 2011;117: 4190-207.5. Kumar S, Diehn F, Gertz M, Tefferi A. Splenectomy for immunethrombocytopenic purpura: long-term results and treatment ofpostsplenectomy 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 immunethrombocytopenia: a retrospective analysis of 233 patients with aminimum 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 newperspectives. 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 adultpatients with idiopathic thrombocytopenic purpura: a systematicreview to assess long-term platelet count responses, prediction ofresponse, and surgical complications. Blood 2004; 104: 2623-34.J Pak Med Assoc (Suppl. 3)S-64 2nd Annual Surgical Meeting 2016

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

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

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