Kojouri K, Vesely SK, Terrel DR et al.: Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess longterm platelet count responses, prediction of response, and surgical complications. Blood 104: 2623-34

Hematology-Oncology Section, Department of Medicine, College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, USA.
Blood (Impact Factor: 10.45). 12/2004; 104(9):2623-34. DOI: 10.1182/blood-2004-03-1168
Source: PubMed


Splenectomy has been a standard treatment for adult patients with idiopathic thrombocytopenic purpura (ITP) for more than 50 years. However, the durability of responses, the ability to predict who will respond, and the frequency of surgical complications with splenectomy all remain uncertain. To better interpret current knowledge we systematically identified and reviewed all 135 case series, 1966 to 2004, that described 15 or more consecutive patients who had splenectomy for ITP and that had data for 1 of these 3 outcomes. Complete response was defined as a normal platelet count following splenectomy and for the duration of follow-up with no additional treatment. Forty-seven case series reported complete response in 1731 (66%) of 2623 adult patients with follow-up for 1 to 153 months; complete response rates did not correlate with duration of follow-up (r = -0.103, P = .49). None of 12 preoperative characteristics that have been reported consistently predicted response to splenectomy. Mortality was 1.0% (48 of 4955 patients) with laparotomy and 0.2% (3 of 1301 patients) with laparoscopy. Complication rates were 12.9% (318 of 2465) with laparotomy and 9.6% (88 of 921 patients) with laparoscopic splenectomy. Although the risk of surgery is an important consideration, splenectomy provides a high frequency of durable responses for adult patients with ITP.

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    • "For patients with chronic ITP who failed to respond to corticosteroids or who can not tolerate corticosteroids, splenectomy is the second-line therapy in many centers [7]. However, about 15–20% of patients do not respond well to splenectomy and among those who respond, 15–20% have relapse weeks, months, or even years later after splenectomy [8] [9]. As the platelets in ITP are freshly out of the bone marrow and quite active, swift increases in platelet count may cause thrombosis [10]. "
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    ABSTRACT: Immune trombocytopenia is an autoimmune disease characterised by abnormal platelet destruction. Corticosteroids and intravenous immune globulin are main first line treatment options. Splenectomy is a preferred second line treatment option for unresponsive patients at many centers. Secondary thrombocytosis is a possible complication awaiting this group of patients after splenectomy. Here, we present a case of myocardial infarction at a patient who had undergone splenectomy for ITP, which is a rare event seen with secondary thrombocytosis due to splenectomy.
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    • "In addition, this type of ITP reactivation can occur any time after splenectomy, even after as long as 5 years. Systematic reviews reported a response rate after splenectomy ranging from 66% to 72% after a 5-year follow-up [15,16]. Follow-up for ITP relapse should be conducted to clarify the long-term response following splenectomy. "
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    ABSTRACT: Purpose Laparoscopic splenectomy (LS) for pediatric chronic immune thrombocytopenic purpura (ITP) patients has recently become widespread. However, its long-term result is rarely reported in children. Methods We retrospectively analyzed the patients who underwent LS for pediatric chronic ITP from June 1998 to April 2007. Results There were 18 patients (14 male and 4 female) with mean age 9.5 ± 3.8 years. 14 complete response, 3 partial response, and 1 no response were occurred. During the 82-month median follow-up period, 9 patients maintained in a remission state without any additional treatment, and 9 patients relapsed. In a comparative analysis of the relapse group and no relapse group, hospital stays were longer in the relapse group and the preoperative platelet counts and platelet counts at 1 month post were lower in relapse group. A relapse-free survival among 17 patients who achieved partial or complete responses following LS showed 76.5%, 61.8%, and 33.0% at 1-, 5-, and 10-year following LS, respectively. Conclusion Although LS in pediatric chronic ITP patients had excellent results immediately after surgery, careful follow up is needed due to late relapse even when a complete response has been noted for several years.
    Annals of Surgical Treatment and Research 06/2014; 86(6):314-8. DOI:10.4174/astr.2014.86.6.314
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    • "Corticosteroids show efficacy in 50% to 80% of cases, but if treatment is stopped, the remission rate is only 10% to 30% [8]. If patients are refractory to drug treatments, splenectomy is a second line option; two-thirds of patients who undergo splenectomy for ITP respond to the treatment [9]. However, complications may arise from this surgical procedure including hemorrhage, abscess, sepsis, thrombosis and death and relapse of ITP occurs in a median of 15% of patients [9]. "
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    ABSTRACT: Introduction Immune thrombocytopenic purpura is thought to be characterized by an immune response against the host’s own platelets. If the thrombocytopenia is severe, patients are initially treated with high-dose steroids. Other more toxic second line treatments are considered if steroids fail. Here, we report the case of two patients in whom conventional treatment was unsuccessful but who responded to hydroxychloroquine and high-dose vitamin D replacement therapy. To the best of our knowledge, this is the first description of successful treatment for immune thrombocytopenia with high-dose vitamin D and hydroxychloroquine. Case presentation Case 1: We report the case of a 79-year-old Caucasian man who presented with high titer antinuclear antibodies, positive anti-SSA/Ro autoantibodies and clinically was felt to have an overlap of systemic lupus erythematosus and/or Sjögren’s syndrome with profound life-threatening thrombocytopenia. There was no evidence of underlying malignancy. The patient’s platelet count significantly increased with vitamin D and hydroxychloroquine treatment, but upon vitamin D discontinuation his platelet levels plummeted. Hydroxychloroquine therapy was maintained throughout treatment. With reinstitution of high-dose vitamin D therapy, platelet counts were restored to normal levels. Case 2: We also report the case of an 87-year-old Caucasian woman who presented with high titer antinuclear antibodies, positive anti-SSA/Ro autoantibodies and was felt to have an overlap of systemic lupus erythematosus and/or Sjögren’s syndrome with immune thrombocytopenia; she also had severely low levels of 25-hydroxy vitamin D (17ng/mL). There was no evidence of underlying malignancy. She responded to high-dose vitamin D replacement and hydroxychloroquine treatment, thereby alleviating the need for high-dose steroid treatment. She remains in remission while taking vitamin D, hydroxychloroquine and very low-dose prednisone. No untoward side effects were observed in either patient. Conclusions In our two case reports, we found an association between vitamin D deficiency and immune thrombocytopenia where platelet levels responded to vitamin D treatment and hydroxychloroquine but not to prednisone. We believe there may be synergism between vitamin D supplementation and hydroxychloroquine. The mechanism by which high-dose vitamin D results in increased platelet counts in immune thrombocytopenia patients is unknown. However, vitamin D has long been thought to play an immunomodulatory role, which may include a dampened immune response in patients with immune thrombocytopenia or other autoimmune diseases.
    Journal of Medical Case Reports 04/2013; 7(1):91. DOI:10.1186/1752-1947-7-91
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