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

HLH was first described by Farquhar and Claireaux in 1952 as a familial disease [1]. Interestingly in their cases hemophagocytosis, which has given the disease its name, could not be found during lifetime but was prominent on autopsy. Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive disorder with an estimated frequency of 0.12/100 000 children per year [2]. Several g...

Hemophagocytic Lymphohistiocytosis (HLH) is a rare, lifethreatening syndrome of excessive multisystem inflammation and tissue destruction due to abnormal immune activation. It is more frequently seen in the pediatric population, either as a genetic disorder or secondary to infection or autoimmune disease, but can present in acquired forms at any age. It is thought to be due to an absence of dow...

Background: Patients with Coronavirus Disease 2019 (COVID-19) frequently experience a hyperinflammatory syndrome leading to unfavorable outcomes. This condition resembles Secondary Hemophagocytic Lymphohistiocytosis (sHLH) described in neoplastic, rheumatic and other infectious diseases. A scoring system (HScore) that evaluates underlying immunosuppression, temperature, organomegaly, cytopenias...