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Immune thrombocytopenic purpura (ITP) associated with pregnancy often involves considerable risk both for mother and child, and usually worsens in the third trimester of gestation. Pregnancy and delivery are especially difficult in patients with severe ITP (platelet count below 20 x 10(9)/L), who are resistant to prednisone and high dose intravenous immunoglobulin (IVIgG). In those cases we applied cesarean section (CS), to prevent intracranial haemorrhage due to fetal/neonatal ITP, and splenectomy at the same time as an effective therapeutic strategy for ITP. We present 5 patients (4 with chronic ITP and 1 with ITP associated with HIV infection), aged 21-35 years, with severe ITP (platelet count 2-10 x 109/L), resistant to prednisone (1-2 mg/kg), and 2/3 were resistant to IVIgG (0.4 g/kg x 5 d). Four patients with severe resistant ITP were supported with 1-2 doses of platelets from cell separator before CS and 1-3 dose during splenectomy. One patient increased platelet count to 55 x 109/L after treatment with IVIgG and splenectomy following CS were done without platelet transfusion. Splenectomy was performed immediately after CS in all patients, and two of them were hysterectomised (one with HIV infection). After splenectomy, platelet count was normalised in all patients, and they had no haemorrhage, wound haematoma formation or any adverse events. But ITP relapsed in 2/5 patients after 1-2 months. Two newborns had severe thrombocytopenia, which solved spontaneously after 3 days in one or after treatment with IVIgG in other. We propose that splenectomy following cesarean section should be considered as approach for delivery and treatment option for mothers with severe resistant ITP.

Splenectomy is definitive treatment for idiopathic thrombocytopenic purpura (ITP) because it removes both the sites of autoantibody producing cells and also the major site of platelet destruction. The purpose of this study was to evaluate long term results of splenectomised patients with ITP and to determine predictor factors for good response. A 167 patients with chronic ITP (136 females, 31 males), median aged 35 years (17-74) was splenectomised after 2 to 160 months (Median 12) from diagnosis of ITP. Indications for splenectomy were: 6 weeks of steroid therapy with platelet count below 10 x 10(9)/l or 3 months with platelet count under 30 x 10(9)/l, or treatment with prednisone above 30 mg more of 6 months to increase platelet count over 30 x 10(9)/l, or repeated relapses. Postoperative complications developed in 16 patients (9.5%), 3 of them died (1.8%) due to thromboembolism and 17 patients discontinued later controls. During follow up to 172 months (Median 62) 111/147 splenectomised patients were in remission (75.5%), 99 in complete (above 100 x 10(9)/l), 12 in partial (50-100 x 10(9)/l) and 36 patients (24.5%) were relapsed (below 50 x 10(9)/l). Remission was achieved in 79/88 patients (89.8%) with good response to prednisone before splenectomy toward 32/62 patients (51.6%) with poor response to prednisone (p < 0.01). Remission was obtained in 9/11 patients (81.8%) who responded well to intravenous immune globulin (0.4 g/kg x 5 d) and only in 1/8 who did not (p < 0.05). Higher response rate was achieved in patients under 40 years of age (81.6%) than in older ones (63.4%) (p < 0.05). No difference was shown between sex and time intervals (3, 6, 12, 24, 36 or over 36 months) from diagnosis to splenectomy. Splenectomy is an effective treatment of refractory ITP with response rate of 75.5% after median follow up of 62 months. In our patients better results on splenectomy were associated with age less than 40 years, good responses to steroid, and intravenous immune globulin.

Idiopathic thrombocytopenic purpura is an autoimmune disease in which macrophages of reticuloendothelial system, mainly in the spleen, remove platelets covered by autoantibodies from circulation. By removing the spleen 60-80% of patients are cured. Partial remission is achieved in 10-20% cases. Very few patients do not react on splenectomy. Recurrency of idiopathic thrombocytopenic purpura in a splenectomized patient after already achieved complete remission, may be caused by hypertrophy of one or more of the retained accessory spleens. We present 3 patients, 41, 23 and 44 year old, in whom splenectomy for ITP had been performed 10, 3 and 11 years earlier. After full remission which lasted 10, 2.5 and 10.5 years a full recurrency of ITP took place with signs of severe thrombocytopenia and haemorrhagic syndrome. Using ultrasonography, computed tomography and scintigraphy accessory spleen/s, were discovered. By their removal, a full remission was achieved in all 3 patients, but full favorable effect appeared approximately three months after surgery during which period additional steroid therapy was necessary.