Long‐term haematological response and maintained immunological function after laparoscopic subtotal splenectomy in patients with hereditary spherocytosis

遗传性球形红细胞增多症 脾切除术 医学 脾脏 球形红细胞增多 红细胞 内科学 胃肠病学 外科
作者
Alica L. Münch,Eva‐Maria Jacobsen,Ansgar Schulz,Wolfgang Loichinger,Tobias Wowra,Lisa Schiefele,Julia Elsner,Mike‐Andrew Westhoff,Alexandre Serra,Gabriele Strauß,K. Schaarschmidt,Holger Cario
出处
期刊:European Journal of Haematology [Wiley]
卷期号:111 (5): 777-786 被引量:4
标识
DOI:10.1111/ejh.14077
摘要

Subtotal or total splenectomy are recommended in severe and should be considered in intermediate forms of hereditary spherocytosis (HS). Data on laparoscopic subtotal splenectomy (LSTS) in HS patients are sparse.Thirty three patients with HS (median age 10.7 years (yrs), range 1.8-15.5) underwent LSTS. Baseline and follow-up investigation included haematological parameters, microscopic analysis of pitted erythrocytes (pitE), and B-cell subpopulations assessed by flow cytometry. Results were compared to those of non-splenectomised HS patients, HS patients after total splenectomy (TS), and healthy individuals.After LSTS, haemoglobin levels were normalised in all patients. During median long-term follow-up of 3.9 yrs (range 1.1-14.9), only four patients presented mild anaemia. Despite re-growing of the remnant spleen none of the patients required a second surgical intervention. As compared to TS, PitE in LSTS patients were significantly lower and indicated normal to only moderately decreased spleen function. Relative but not absolute IgM memory B-cell counts were reduced in both LSTS and TS patients.LSTS is effective for the treatment of patients with HS. A small remnant spleen is sufficient to provide adequate phagocytic function and to induce a pool of IgM memory B-cells.
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