Immunoglobulin levels in the Kaiapit and upper Watut areas of New Guinea

Crane, G.G.; Pitney, W. R.; Hobbs, J. R.; Gunn, Catherine

doi:10.1016/0035-9203(71)90095-2

Cited by 25 publications

(2 citation statements)

References 15 publications

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“…There are scanty data regarding the prevalence of the syndrome in the general population. Studies performed in Papua New Guinea reported an extraordinarily high prevalence of the syndrome (85% in five years old children and 80% in adults) among inhabitants of the Upper Watut Valley [ 58 ]. The relationship between the parasite rate and the spleen size varied with age: in children the spleen size appeared to be directly related to the parasite rate, while in adults the opposite occurred [ 44 ].…”

Section: Resultsmentioning

confidence: 99%

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

Leoni

Buonfrate

Angheben

et al. 2015

Malar J

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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.

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