Splenectomy in Egyptian Children with Immune Thrombocytopenia: A 10-Year Retrospective Single Center Study

Splenectomy represents a second-line treatment of patients with chronic refractory immune thrombocytopenia (ITP),that is recommended to be postponed for at least 12 months after the initial diagnosis. Post-splenectomy long-term remission is achieved in more than two thirds of children with low risk of short- and long-term severe adverse events. Indication for splenectomy in pediatrics is individually based and remains on expert opinion, local practice and availability of resources. Aim of the study is to assess short- and long-term response to splenectomy in Egyptian children diagnosed with ITP.

Methods: All children were diagnosed with chronic refractory ITP in Cairo University Pediatric Hospital and were candidates for a splenectomy( steroids and other immunosuppressive therapy fail to control mucous membrane bleeds and /or accomplish a hemostatic platelet count and patient usually ≥ 5 years of age ) over the past 10 years were included in the study .Their records were reviewed and they were further assessed clinically and by platelet counts. Chronic ITP children were divided into 2 groups; those who conducted a splenectomy(Group 1) and those who did not(Group2) .All patients'data was analysed. Descriptive statistics were used to describe results by percentages, means, and standard deviations and Chi square test was used to compare two of more than two qualitative groups and P ≤ 0.05 was considered the cut-off value for significance.

Results: The study included 31 children diagnosed with chronic ITP and a teenage girl with newly diagnosed ITP having persistent severe menorrhagia and who was not responding to standard therapy. There were 51.6% males and 48.4% females. Their mean initial and current age was 5.1±2.8 and 10.2±4.8 years, respectively. 16.1% of patients were of consanguineous marriage and one patient had a similarly affected sibling. The mean initial platelet count was 19+/-14x10⁹/L whilst pre- and post-splenectomy platelet counts were 24+/-19x10⁹/L and 183+/-221x10⁹/L, respectively. Splenectomy was conducted after a mean period of 2.9 years of follow-up. All children exhibited subcutaneous bleedings at the time of initial presentation, 24 of 32 children had mucous membrane bleeds with a mean initial hemoglobin (Hb) of 11.1+/-1.3g/L, 2 of 32 children experienced an intracranial hemorrhage and 6 of 9 teenage girls had menorrhagia. After the splenectomy 16 of 32 children had subcutaneous bleeds and 10 of 32 had mucous membrane bleeds. Treatment was administered in all children due to frequent and/or severe mucous membrane bleeds and consisted in newly diagnosed ITP of methylprednisolone (HDMP) in 61.3%, anti-D in 16.1%, immunoglobulines in 25.8%.Second line therapies in chronic ITP consisted of azathioprine in 48.4% and triple therapy (prednisolone 0.8mg/kg/d, azathioprine 2mg/kg/d, and cyclosporine 2.5mg/kg/d) in 22.6% of children . These treatments were also used to boost platelet counts pre-splenectomy. 25 of 32 children (78.1%, half requiring HDMP initially) achieved a complete remission after 2 year of follow-up. 7 of 32 children (18.9%) required post-splenectomy treatment. One patient had post-operative hernia and another died unexplained on her way to hospital with a history suggestive of ICH, although doing well before and on no treatment for 2 years. In children with chronic ITP without splenectomy(Group2), the mean initial platelet count was lower with more frequent mucous membrane bleeds (p=0.001) as compared to those who underwent splenectomy(Group1

Conclusion: Splenectomy can be considered an appropriate second-line therapy for children with chronic refractory ITP especially in countries with resource constraint.