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

Abstract

IntroductionSubtotal 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.MethodsThirty 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.ResultsAfter 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.ConclusionsLSTS 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.

Current Status of Molecular Diagnosis of Hereditary Hemolytic Anemia in Korea

“… 96 Registry data also suggest that some patients with rare CDA-II and PKD may not benefit from splenectomy and are better suited for repeated transfusions or stem cell transplantation or both. 109 110 Therefore, the collection of data from large registries in combination with clinical and molecular genetic information, is critical for the development of tailored treatment plans for these patients.…”