预防溶血和改善贫血:有中等质量的证据表明,脾切除术对 HS 有效,可预防溶血,并提高血红蛋白水平。[31]Baird RN, Macpherson AI, Richmond J. Red-blood-cell survival after splenectomy in congenital spherocytosis. Lancet. 1971;2:1060-1061.http://www.ncbi.nlm.nih.gov/pubmed/4106913?tool=bestpractice.com[32]Chapman RG, McDonald LL. Red cell life span after splenectomy in hereditary spherocytosis. J Clin Invest. 1968;47:2263-2267.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC297390/pdf/jcinvest00245-0091.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/5676521?tool=bestpractice.com[33]Agre P, Asimos A, Casella JF, et al. Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in hereditary spherocytosis. N Engl J Med. 1986;315:1579-1583.http://www.ncbi.nlm.nih.gov/pubmed/3785322?tool=bestpractice.com[34]Reliene R, Mariani M, Zanella A, et al. Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin/ankyrin- and band 3-deficient hereditary spherocytosis. Blood. 2002;100:2208-2215.http://www.bloodjournal.org/content/100/6/2208.fullhttp://www.ncbi.nlm.nih.gov/pubmed/12200387?tool=bestpractice.com 一些重度 HS 患者在脾切除术后可能没有正常的红细胞存活率。[31]Baird RN, Macpherson AI, Richmond J. Red-blood-cell survival after splenectomy in congenital spherocytosis. Lancet. 1971;2:1060-1061.http://www.ncbi.nlm.nih.gov/pubmed/4106913?tool=bestpractice.com[32]Chapman RG, McDonald LL. Red cell life span after splenectomy in hereditary spherocytosis. J Clin Invest. 1968;47:2263-2267.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC297390/pdf/jcinvest00245-0091.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/5676521?tool=bestpractice.com[33]Agre P, Asimos A, Casella JF, et al. Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in hereditary spherocytosis. N Engl J Med. 1986;315:1579-1583.http://www.ncbi.nlm.nih.gov/pubmed/3785322?tool=bestpractice.com[34]Reliene R, Mariani M, Zanella A, et al. Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin/ankyrin- and band 3-deficient hereditary spherocytosis. Blood. 2002;100:2208-2215.http://www.bloodjournal.org/content/100/6/2208.fullhttp://www.ncbi.nlm.nih.gov/pubmed/12200387?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
预防溶血和改善贫血:有中等质量的证据表明,脾切除术对 HS 有效,可预防溶血,并提高血红蛋白水平。[31]Baird RN, Macpherson AI, Richmond J. Red-blood-cell survival after splenectomy in congenital spherocytosis. Lancet. 1971;2:1060-1061.http://www.ncbi.nlm.nih.gov/pubmed/4106913?tool=bestpractice.com[32]Chapman RG, McDonald LL. Red cell life span after splenectomy in hereditary spherocytosis. J Clin Invest. 1968;47:2263-2267.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC297390/pdf/jcinvest00245-0091.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/5676521?tool=bestpractice.com[33]Agre P, Asimos A, Casella JF, et al. Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in hereditary spherocytosis. N Engl J Med. 1986;315:1579-1583.http://www.ncbi.nlm.nih.gov/pubmed/3785322?tool=bestpractice.com[34]Reliene R, Mariani M, Zanella A, et al. Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin/ankyrin- and band 3-deficient hereditary spherocytosis. Blood. 2002;100:2208-2215.http://www.bloodjournal.org/content/100/6/2208.fullhttp://www.ncbi.nlm.nih.gov/pubmed/12200387?tool=bestpractice.com 一些重度 HS 患者在脾切除术后可能没有正常的红细胞存活率。[31]Baird RN, Macpherson AI, Richmond J. Red-blood-cell survival after splenectomy in congenital spherocytosis. Lancet. 1971;2:1060-1061.http://www.ncbi.nlm.nih.gov/pubmed/4106913?tool=bestpractice.com[32]Chapman RG, McDonald LL. Red cell life span after splenectomy in hereditary spherocytosis. J Clin Invest. 1968;47:2263-2267.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC297390/pdf/jcinvest00245-0091.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/5676521?tool=bestpractice.com[33]Agre P, Asimos A, Casella JF, et al. Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in hereditary spherocytosis. N Engl J Med. 1986;315:1579-1583.http://www.ncbi.nlm.nih.gov/pubmed/3785322?tool=bestpractice.com[34]Reliene R, Mariani M, Zanella A, et al. Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin/ankyrin- and band 3-deficient hereditary spherocytosis. Blood. 2002;100:2208-2215.http://www.bloodjournal.org/content/100/6/2208.fullhttp://www.ncbi.nlm.nih.gov/pubmed/12200387?tool=bestpractice.com