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An Unusual Presentation of Disseminated Histoplasmosis 

An Unusual Presentation of Disseminated Histoplasmosis
Chapter:
An Unusual Presentation of Disseminated Histoplasmosis
Author(s):

Lokendra Thakur

and Vivek Iyer

DOI:
10.1093/med/9780190464813.003.0032
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date: 15 October 2019

Hemophagocytic lymphohistiocytosis (HLH) is divided into primary (genetic) and secondary (acquired) forms. The familial form is an autosomal recessive disorder with an incidence of 1 in 50,000 live births. It is occasionally associated with mutations in the perforin gene, it often occurs during early childhood, and, without treatment, it is usually fatal within 2 months after the onset of symptoms. Clinical presentation is similar to that for severe sepsis and septic shock.

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