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Haematologica. 2017 Aug;102(8):1304-1313. doi: 10.3324/haematol.2016.161166. Epub 2017 May 26.

Recommendations regarding splenectomy in hereditary hemolytic anemias.

Author information

1
Department of Molecular Medicine and Medical Biotechnology, University Federico II Naples, Italy achille.iolascon@unina.it.
2
CEINGE Biotecnologie Avanzate, Naples, Italy.
3
Department of Molecular Medicine and Medical Biotechnology, University Federico II Naples, Italy.
4
Oncohematology Unit, IRCCS Ca' Granda Foundation, Ospedale Maggiore Policlinico, Milan, Italy.
5
Department of General Surgery, Monaldi Hospital A.O.R.N. dei Colli, Naples, Italy.
6
Service d'Hématologie Biologique, CHU Amiens Picardie, Amiens, France.
7
Department of Medicine, University of Verona and AOUI-Verona, Italy.
8
Department of Translational Medical Sciences, Federico II University of Naples, Italy.
9
Department of Clinical Science and Community Health, Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, University of Milan, Italy.
10
Department of Pediatrics, Faculty of Medicine and Dentistry, Palacky University Olomouc and University Hospital Olomouc, Czech Republic.
11
Department of Paediatric Haematology, King's College Hospital, King's College London School of Medicine, UK.
12
Pediatrics Department, Necker Hospital, Paris, France.
13
Department of Pediatrics, Division of Hematology-Oncology, Children's Blood and Cancer Foundation Laboratories, Weill Cornell Medical College, New York, NY, USA; Department of Pediatrics, Division of Hematology, Children's Hospital of Philadelphia, PA, USA.
14
Hematology Service, Hospital and University Center of Coimbra (CHUC), Portugal.
15
Red Cell Pathology Unit, Hospital Clínic de Barcelona, Spain.
16
Laboratory of Hematology, CHRU de Montpellier, Hôpital Saint Eloi, France.
17
First Department of Pediatrics, University of Athens, Greece.
18
Department of Clinical Chemistry, Hôpital Erasme, U.L.B., Brussels, Belgium.
19
Department of Paediatrics, Children's Hospital, University of Oxford, John Radcliffe Hospital, UK.
20
Pediatric Hematology Unit, Schneider Children's Medical Center of Israel, Petah Tiqva, Sackler Faculty of Medicine, Tel Aviv University, Israel.

Abstract

Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications. In light of the priorities identified by the European Hematology Association Roadmap we generated specific recommendations for each disorder, except thalassemia syndromes for which there are other, recent guidelines. Our recommendations are intended to enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and the possible consequences. As no randomized clinical trials, case control or cohort studies regarding splenectomy in these disorders were found in the literature, recommendations for each disease were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children.

PMID:
28550188
PMCID:
PMC5541865
DOI:
10.3324/haematol.2016.161166
[Indexed for MEDLINE]
Free PMC Article

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