Spleen is a vital hematopoietic and immune organ in human body. Spleen plays a complex role in hematological diseases. It is not only the site where blood cells are destroyed, but also participates in multiple physiological functions such as immune response, hematopoiesis and filtration, etc. In hematological diseases, pathological enlargement of the spleen or hypersplenism can lead to abnormal destruction of blood cells, thereby aggravating anemia, thrombocytopenia and other symptoms. Splenectomy, as an effective treatment, can mitigate the negative effect of spleen on blood components. However, with in-depth understanding of spleen function and its role in immune regulation, as well as the application of new drug therapy, partial patients with hematological diseases can also obtain favorable clinical outcomes through non-surgical treatment. Hence, the role of splenectomy in the treatment of hematological diseases has also changed. Disputes and challenges still exist in the indications, timing and postoperative management of splenectomy. In this article, clinical application, efficacy and the latest research progress in splenectomy for hematological diseases were reviewed, aiming to discuss current hot topics and future direction of splenectomy.