Haemophagocytic Lymphohistiocytosis as a Complication of Israeli Spotted Fever

Maria Margarida Andrade; Ana Gorgulho; Rita Tinoco Magalhães; Rita Valadas; Luís Miguel Pereira; Sara Freire; Diogo Cruz

doi:10.12890/2022_003073

Haemophagocytic Lymphohistiocytosis as a Complication of Israeli Spotted Fever

Eur J Case Rep Intern Med. 2022 Jan 17;9(1):003073. doi: 10.12890/2022_003073. eCollection 2022.

Authors

Maria Margarida Andrade¹, Ana Gorgulho¹, Rita Tinoco Magalhães¹, Rita Valadas¹, Luís Miguel Pereira¹, Sara Freire¹, Diogo Cruz^{1

2}

Affiliations

¹ Internal Medicine Department, Hospital de Cascais Dr. José de Almeida, Alcabideche, Portugal.
² Faculty of Medicine, University of Lisbon, Lisbon, Portugal.

Abstract

Rickettsia spp. human infection is endemic in Portugal in the form of Mediterranean spotted fever caused by R. conorii subsp. conorii and Israeli spotted fever (ISF) caused by R. conorii subsp. israelensis. We describe a rare case of haemophagocytic lymphohistiocytosis (HLH) due to ISF, with atypical manifestations. We highlight the need for clinical suspicion for this diagnosis and the importance of timely intervention and support.

Learning points: Haemophagocytic lymphohistiocytosis is a rare complication of rickettsiosis.An exuberant inflammatory reaction with multisystemic dysfunction should raise suspicion.Cutaneous and neurological manifestations can be serious and extensive.

Keywords: Rickettsiosis; haemophagocytic; polyneuropathy; purpura fulminans; spotted fever.