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Haemophagocytic Lymphohistiocytosis as a Complication of Israeli Spotted Fever
Maria Margarida Andrade1, Ana Gorgulho1, Rita Tinoco Magalhães1
1Internal Medicine Department, Hospital de Cascais Dr. José de Almeida, Alcabideche, 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.
Insights
Israeli spotted fever (ISF) can rarely cause haemophagocytic lymphohistiocytosis (HLH). This case highlights the need for early diagnosis and intervention for this severe complication of rickettsiosis.
Area of Science:
- Infectious Diseases
- Hematology
- Immunology
Background:
- Rickettsia spp. infections, including Mediterranean spotted fever and Israeli spotted fever (ISF), are endemic in Portugal.
- Haemophagocytic lymphohistiocytosis (HLH) is a rare but severe condition characterized by excessive immune activation.
Purpose of the Study:
- To describe a rare case of HLH secondary to ISF.
- To emphasize the atypical clinical manifestations of ISF-induced HLH.
- To highlight the importance of early clinical suspicion and intervention.
Main Methods:
- Case report detailing clinical presentation, diagnostic workup, and management.
- Review of literature on rickettsiosis and HLH.
Main Results:
- A patient presented with atypical manifestations of ISF leading to HLH.
- The case underscores the potential for severe, multisystemic complications from rickettsial infections.
Conclusions:
- HLH is a rare but critical complication of rickettsiosis.
- Suspicion for HLH should be raised in cases of rickettsiosis with exuberant inflammatory responses and multisystemic dysfunction.
- Prompt diagnosis and supportive care are crucial for managing ISF-induced HLH, especially with severe cutaneous and neurological involvement.
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