Murine typhus complicated by sHLH mimicking adult-onset Still's disease

Jacquot, Robin; Gerfaud‐Valentin, Mathieu; Lega, Jean‐Christophe; Becker, Ann De; Jamilloux, Yvan; Sève, P.

doi:10.1016/j.revmed.2022.05.011

Cited by 4 publications

(3 citation statements)

References 27 publications

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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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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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“…Other important etiological factors that are considered rare but play an important role in the etiology of this disease are surgery, speci cally silicone implants surgery for breast augmentation and reconstruction (3), which were found to be associated with anaplastic large cell lymphoma, despite being extremely rare but continuing to be reported (25,26), proving that tumorogenesis and tumor progression have been linked to in ammation activating the pro in ammatory mediators as well as infections that are considered to be the key role for the activation of B and T cells (26). Systemic lupus erythematosus and adult-onset still's disease, which represent a slightly different condition known as macrophage activation syndrome or MAS (22), as well as four genes (PRF1, UNC13D, STX11, and STXBP2), which all encode proteins necessary for lymphocyte cytotoxicity, have so far been found to have disease-causing mutations in primary HLH, which is passed down in an autosomal recessive manner. (2,20,27) .…”

Section: Case Presentationmentioning

confidence: 99%

“…Jaundice and rash (sometimes known as a maculopapular rash or a nonspeci c rash) are two common skin symptoms (39,40). Up to 40% of patients have reported having respiratory involvement, which can involve infections or acute respiratory distress syndrome (ARDS) (22).…”

Section: Case Presentationmentioning

confidence: 99%

Haemophagocytic lymphocytosis (HLH) vicious cycle in a Cannabinoids addict patient case report and disease review.

Amer

Hamdar

Amer

et al. 2023

Preprint

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Haemophagocytic lymphocytosis (HLH) is a rare life-threatening syndrome caused by the uncontrolled activity of cytokines, natural killers, and macrophages, which can alter the activity of the organism, resulting in multiple organ dysfunction and mortality. Fever, splenomegaly, coagulopathies, dyspnea, changes in mental status, or irritability may be associated with HLH diseases. Depending on the underlying causes, such as bacterial or viral infections, HLH may be primary, hereditary, or secondary. The early diagnosis and treatment of patients are directly related to their prognosis and clinical outcome. On the other hand, HLH can present a number of obstacles, particularly for children and newborns, as well as hematological defects which might cause other autoimmune disorders. Case presentation: We present a case of a 28-year-old male patient admitted to the hospital with a history of persistent high grade fever for two weeks, right lower limb swelling three weeks prior to admission, and right side weakness for one month. Aside from being addicted to cannabis for three years, the patient has no prior medical history. On admission, the patient was awake, conscious, oriented, and hemodynamically stable. A complete blood count, ultrasound examinations, and a whole-body CT scan revealed that the results were favorable for multiple enlarged lymph nodes and hepatosplenomegaly. The patient was given acyclovir, Vfend, Colistin, Targocid, Tinam, Septrin, and anticoagulant during the follow-up. The patient's clinical condition was rapidly deteriorating; a bone marrow biopsy was performed, which revealed haemophagocytosis; and dexamethasone was started. The patient's clinical condition deteriorated during his hospital stay until he went into cardiac arrest and had to be resuscitated for 30 minutes with pulseless electrical activity. Background and Aim: This case suggests that we should be vigilant to the patient who is admitted to the hospital with symptoms for unknown reasons, in order to diagnose HLH as soon as possible and clarify its cause, and it also puts several theories regarding the pathogenicity of this disease in our hands, which will be described in this case, making this case a subject for discussion and research in the medical field. Conclusion: Haemophagocytic lymphocytosis (HLH) is a severe inflammatory disease that improperly controls the body's immune response. Viruses and bacterial infections are just two examples of the various etiological causes that can cause it. Lab results and symptom presentations that are particular to the primary infection can indicate it. Diagnosis and treatment must be provided as soon as possible in order to reduce morbidity and mortality.

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Severe flea-borne typhus complicated by hemophagocytic lymphohistiocytosis: A case report and review of literature

Zamora Gonzalez,

Mayo,

Jeng

2024

IDCases

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Murine typhus complicated by sHLH mimicking adult-onset Still's disease

Cited by 4 publications

References 27 publications

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

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

Haemophagocytic lymphocytosis (HLH) vicious cycle in a Cannabinoids addict patient case report and disease review.

Severe flea-borne typhus complicated by hemophagocytic lymphohistiocytosis: A case report and review of literature

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