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脾切除术后难治性免疫性血小板减少性紫癜(ITP):病例报告及文献复习。

Refractory immune thrombocytopenic purpura (ITP) after accessory splenectomy: A case report and literature review.

机构信息

Ayub Teaching Hospital, Abbottabad, Pakistan.

Qena Faculty of Medicine, South Valley University, Qena, Egypt.

出版信息

Clin Hemorheol Microcirc. 2023;85(2):189-194. doi: 10.3233/CH-231881.

Abstract

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a platelet count of less than 100 x 109 /L, resulting from antibody-mediated platelet destruction. Treatment for ITP typically involves steroids, and intravenous immunoglobulins (IVIG) can be added. Splenectomy is performed in cases with refractory ITP. Rituximab can suppress immunity but has limited efficacy in ITP cases. Herein, we present a rare case of a 30-year-old female who was first diagnosed with ITP and underwent a splenectomy two years later. However, seven years after surgery, she was presented with symptoms of ITP. A splenic scan showed an accessory spleen in the spleen bed, for which she underwent accessory spleen removal surgery. Her laboratory tests three days post-operation showed a rise in platelet count and hence was discharged a few days later. The patient had recurrent attacks of ITP even after the removal of the normal and accessory spleen, suggesting that accessory spleen removal may not always be an effective treatment for ITP. The patient eventually died. While splenectomy is a common treatment for ITP, it may not always be effective in all cases, and other treatments such as bone marrow transplantation may be necessary.

摘要

免疫性血小板减少性紫癜(ITP)是一种自身免疫性疾病,其特征是血小板计数低于 100×109/L,是由抗体介导的血小板破坏引起的。ITP 的治疗通常包括使用类固醇,并且可以添加静脉注射免疫球蛋白(IVIG)。对于难治性 ITP 会进行脾切除术。利妥昔单抗可以抑制免疫,但在 ITP 病例中的疗效有限。在此,我们报告了一例罕见的 30 岁女性病例,她最初被诊断为 ITP,并在两年后进行了脾切除术。然而,手术后 7 年,她出现了 ITP 的症状。脾扫描显示脾床中有副脾,因此她接受了副脾切除术。她术后三天的实验室检查显示血小板计数升高,几天后出院。尽管切除了正常和副脾,但该患者仍反复发生 ITP 发作,这表明切除副脾并不总是治疗 ITP 的有效方法。最终该患者死亡。虽然脾切除术是 ITP 的常见治疗方法,但在所有病例中并不总是有效,可能需要其他治疗方法,如骨髓移植。

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