Secondary hemophagocytic lymphohistiocytosis associated with Rocky Mountain spotted fever in a toddler: A case report

Vasanna, Smitha Hosahalli; Lim, Peter Paul; Khan, Tanya Saeeda; Dalal, Jignesh

doi:10.1002/jha2.405

Cited by 4 publications

(3 citation statements)

References 12 publications

(12 reference statements)

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“…The use of immunomodulatory doses similar to the therapy directed to medium and small vessel vasculitis demonstrates the reduction of IL-1 in macrophages and IL-6 production by monocytes. A case report showed a successfully treated case of HLH secondary to rickettsiosis with anakinra, an IL-1 receptor antagonist [9]. The mechanism of modulation of immune response mediated by IVIG also demonstrates effects in the inhibition of phagocytosis by inhibiting FCR.…”

Section: Discussionmentioning

confidence: 99%

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A confirmed case of macrophage activation syndrome secondary to spotted fever rickettsiosis: Response to immunomodulation in a pediatric patient in northeastern, Mexico

Galindo Hayashi,

Sifuentes-Aguilar,

Soto-Chavez

et al. 2023

Indian J Case Reports

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An 8-year-old girl with a rash and high-grade fever for 6 days arrived at the emergency room. She had an erythematous macular rash on the face, trunk, arms, and legs. Further interrogation called attention to the presence of close contact with stray dogs. Her town had been recognized as a site of a rickettsiosis outbreak in the past year. Spotted fever rickettsiosis was suspected, and doxycycline treatment was initiated. Macrophage activation syndrome (MAS) secondary to Rickettsia rickettsii infection was diagnosed according to the Hemophagocytic lymphohistiocytosis and EULAR/PRINTO/PRES 2016 criteria. As there are no clear guidelines on the treatment of MAS secondary to R. rickettsii. the course of action taken by the pediatric intensive care unit team was to avoid disseminated intravascular coagulopathy and treat MAS, both life-threatening conditions. Directed therapy with high doses of methylprednisolone and intravenous immunoglobulin therapy was initiated. The patient recovered, regaining her functional state before the illness. Few articles have described the association between MAS and rickettsiosis, an illness with high mortality, which makes it paramount to detect and treat promptly.

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Section: Discussionmentioning

confidence: 99%

“…Avoiding DIC by monitoring platelets, fibrinogen, coagulation factors, D-dimer, and treating with blood products as necessary, should have an impact on mortality in this context [9,12].…”

Section: Discussionmentioning

confidence: 99%

A confirmed case of macrophage activation syndrome secondary to spotted fever rickettsiosis: Response to immunomodulation in a pediatric patient in northeastern, Mexico

Galindo Hayashi,

Sifuentes-Aguilar,

Soto-Chavez

et al. 2023

Indian J Case Reports

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“…The following were excluded from the analysis: articles published in languages other than English; case reports not providing an appropriate amount of information, or where the diagnosis was not certain; and those reporting non-human subjects. The final number of articles included was 69, which resulted in a total number of 98 patients ( Figure 1 ) [ 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , ...…”

Section: Methodsmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis (HLH) in Patients with Tick-Borne Illness: A Scoping Review of 98 Cases

Jevtic,

da Silva,

Haylock

et al. 2024

Infectious Disease Reports

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Hemophagocytic lymphohistiocytosis (HLH) secondary to tick-borne infections is a rare but potentially life-threatening syndrome. We performed a scoping review according to PRISMA guidelines to systematically analyze the existing literature on the topic. A total of 98 patients were included, with a mean age of 43.7 years, of which 64% were men. Most cases, 31%, were reported from the USA. Immunosuppression was present in 21.4%, with the most common cause being previous solid organ transplantation. Constitutional symptoms were the most common, observed in 83.7% of the patients, while fever was reported in 70.4% of cases. Sepsis was present in 27.6%. The most common laboratory abnormalities in this cohort were thrombocytopenia in 81.6% of patients, while anemia, leukopenia, and leukocytosis were observed in 75.5%, 55.1%, and 10.2%, respectively. Liver enzyme elevation was noted in 63.3% of cases. The H-score was analyzed in 64 patients, with the mean value being 209, and bone marrow analysis was performed in 61.2% of patients. Ehrlichia spp. was the main isolated agent associated with HLH in 45.9%, followed by Rickettsia spp. in 14.3% and Anaplasma phagocytophilum in 12.2%. Notably, no patient with Powassan virus infection or Lyme borreliosis developed HLH. The most common complications were acute kidney injury (AKI) in 35.7% of patients, shock with multiple organ dysfunction in 22.5%, encephalopathy/seizure in 20.4%, respiratory failure in 16.3%, and cardiac complications in 7.1% of patients. Treatment included antibiotic therapy alone in 43.9%, while 5.1% of patients were treated with immunosuppressants alone. Treatment with both antibiotics and immunosuppressants was used in 51% of patients. Appropriate empiric antibiotics were used in 62.2%. In 43.9% of cases of HLH due to tick-borne disease, patients received only antimicrobial therapy, and 88.4% of those recovered completely without the need for immunosuppressive therapy. The mortality rate in our review was 16.3%, and patients who received inappropriate or delayed empiric therapy had a worse outcome. Hence, we suggest empiric antibiotic treatment in patients who are suspected of having HLH due to tick-borne disease or in whom diagnostic uncertainty persists due to diagnostic delay in order to minimize mortality.

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Zoonotic Bacterial Infections Triggering Cytokine Storm Syndrome

Otrock,

Eby

2024

Advances in Experimental Medicine and Biology

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Secondary hemophagocytic lymphohistiocytosis associated with Rocky Mountain spotted fever in a toddler: A case report

Cited by 4 publications

References 12 publications

A confirmed case of macrophage activation syndrome secondary to spotted fever rickettsiosis: Response to immunomodulation in a pediatric patient in northeastern, Mexico

A confirmed case of macrophage activation syndrome secondary to spotted fever rickettsiosis: Response to immunomodulation in a pediatric patient in northeastern, Mexico

Hemophagocytic Lymphohistiocytosis (HLH) in Patients with Tick-Borne Illness: A Scoping Review of 98 Cases

Zoonotic Bacterial Infections Triggering Cytokine Storm Syndrome

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