The hyper-reactive malarial splenomegaly: a systematic review of the literature

Malaria Journal

Research

Zeno Bisoffi¹  Andrea Angheben¹  Federico Gobbi¹  Dora Buonfrate¹  Stefania Leoni² 

[1] Centre for Tropical Diseases, S Cuore Hospital, 37024, Verona, Negrar, Italy;Centre for Tropical Diseases, S Cuore Hospital, 37024, Verona, Negrar, Italy;Internal Medicine Department, Verona University, Piazzale L A Scuro, 10, 37134, Verona, Italy;

关键词: Hyper-reactive;    Malarial;    Splenomegaly;    Management;    Treatment;    Epidemiology;    Review;   

DOI  :  10.1186/s12936-015-0694-3

 received in 2014-12-29, accepted in 2015-04-14,  发布年份 2015

来源: Springer

【 摘 要 】

BackgroundThe hyper-reactive malarial splenomegaly syndrome (HMS) is a leading cause of massive splenomegaly in malaria-endemic countries. HMS is caused by a chronic antigenic stimulation derived from the malaria parasite. Classic Fakunle’s major criteria for case definition are: persistent gross splenomegaly, elevated anti-malarial antibodies, IgM titre >2 SD above the local mean value and favourable response to long-term malaria prophylaxis. The syndrome is fatal if left untreated. The aim of this study is to systematically review the literature about HMS, particularly focussing on case definition, epidemiology and management.MethodsThe search strategy was based on the following database sources: Pubmed, EmBase, Scopus. Search was done in March, 2014 and limited to English, Spanish, Italian, French, and Portuguese.ResultsPapers detected were 149, of which 89 were included. Splenomegaly was variably defined and the criterion of increased IgM was not always respected. The highest prevalence was reported in Papua New Guinea (up to 80%). In different African countries, 31 to 76% of all splenomegalies were caused by HMS. Fatality rate reached 36% in three years. The most frequent anti-malarial treatments administered were weekly chloroquine or daily proguanil from a minimum of one month to lifelong. In non-endemic countries, a few authors opted for a single, short anti-malarial treatment. All treated patients with no further exposure improved. Cases not completely fulfilling Fakunle’s criteria and therefore untreated, subsequently evolved into HMS. It seems thus appropriate to treat incomplete or ‘early’ HMS, too.ConclusionsFor patients not re-exposed to endemic areas, a short course of treatment is sufficient, showing that eradicating the infection is sufficient to cure HMS. Longer (probably lifelong) courses, or intermittent treatments, are required for those who remain exposed. Splenectomy, associated with high mortality, should be strictly limited to cases not responding to medical treatment.

【 授权许可】

Unknown   
© Leoni et al.; licensee BioMed Central. 2015. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.