Reactive macrophage activation ayndrome: validation of a simple screening strategy and its potential in early treatment initiation

Emmenegger, Urban; Reimers, Andrea; Frey, U.; Fux, Ch; Bihl, Florian; Semela, David; Cottagnoud, Philippe; Cerny, Andreas; Spaeth, Peter; Neftel, Klaus

doi:10.4414/smw.2002.09941

Cited by 30 publications

(6 citation statements)

References 21 publications

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“…Because the condition frequently occurs secondary to other diseases and clinical signs resemble those associated with bacterial sepsis and the systemic inflammatory response syndrome, the condition may be underdiagnosed. 1,11,12 Furthermore, cytopenias may be attributed to excessive demand for neutrophils in tissues, consumption of platelets secondary to disseminated intravascular coagulation, or intravascular fragmentation of erythrocytes. Therefore, it is important to determine in which dogs bone marrow evaluation is needed for detection of hemophagocytic syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…In humans, serum ferritin concentration has been used as a screening test for detection of hemophagocytic syndrome and malignant histiocytosis. 11,12 In 1 study, 12 91% of adult patients with hemophagocytic syndrome had serum ferritin concentrations > 10,000 µg/L. 12 Serum ferritin concentration has not been evaluated as a diagnostic test in dogs with hemophagocytic syndrome, but has been evaluated in dogs with malignant histiocytosis.…”

Section: Discussionmentioning

confidence: 99%

“…11,12 In 1 study, 12 91% of adult patients with hemophagocytic syndrome had serum ferritin concentrations > 10,000 µg/L. 12 Serum ferritin concentration has not been evaluated as a diagnostic test in dogs with hemophagocytic syndrome, but has been evaluated in dogs with malignant histiocytosis. a Moderate hyperferritinemia was detected in dogs with hemolymphatic neoplasia and hepatic disease.…”

Section: Discussionmentioning

confidence: 99%

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Hemophagocytic syndrome in dogs: 24 cases (1996–2005)

Weiss

2007

javma

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Results indicated that hemophagocytic syndrome may occur more frequently in dogs than has previously been suspected on the basis of the paucity of reported cases. Although most dogs had definable underlying disease conditions, idiopathic hemophagocytic syndrome was also identified. Hemophagocytic syndrome of any cause is potentially life-threatening; however, the prognosis should be adjusted on the basis of the associated disease process and potential for successful treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Hemophagocytic syndrome in dogs: 24 cases (1996–2005)

Weiss

2007

javma

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“…Regardless of its aetiology, dysfunctional regulation of cytokines leads to a cytokine storm clinically resembling systemic inflammatory response syndrome (SIRS). In some cases this may result in multiple organ dysfunction or failure, and even death [9]. The diagnosis of HLH is challenging owing to the lack of pathognomonic clinical and laboratory markers.…”

Section: Introductionmentioning

confidence: 99%

Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis

Schweizer¹,

Goede²,

Briner³

2015

Swiss Med Wkly

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HScore reflects a higher specificity than the algorithm for diagnosing HLH. The discrepancy may indicate the difficulty that a specific marker still is missing. Hyperferritinaemia was strongly associated with HLH in patients with haematological or oncological malignancies. HLH may be underdiagnosed because the majority of these patients suffer from a severe underlying disease, which easily might suggest a flare or infection. In this population, hyperferritinaemia and SIRS should rise suspicion because mortality in HLH is high.

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“…While a consensus panel in 2016 developed diagnostic criteria to differentiate between systemic juvenile idiopathic arthritis flare and HLH, these criteria have not been validated in autoimmune diseases other than children. 16 Most studies have diagnosed HLH using the Henter et al diagnostic guidelines. 5 However, this strict set of criteria may delay diagnosis in patients with a milder initial presentation.…”

Section: Discussionmentioning

confidence: 99%

Uncovering the Spectrum of Hemophagocytic Lymphohistiocytosis: A Nephrology Department’s Analysis of 14 Cases

Hajji,

Barbouch,

Kaaroud

et al. 2023

Clin Med Insights Case Rep

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Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a disease of multi-organ dysfunction due to excessive immune activation causing widespread inflammation and tissue destruction. It is a severe condition associated with high morbidity and mortality. Early identification is crucial for prompt treatment. The objective of this case series is to underscore the intricacy of managing HLH in individuals with renal dysfunction. Methods: This is a retrospective study of patients diagnosed with HLH in a nephrology department over a period of 30 years. We retrospectively reviewed the medical files by applying the Revised HLH-2004 criteria. Results: Among the 14 female patients included, the mean age was 45.2 years (range 23-78). Nine patients presented with sudden onset of fever and chills. Physical examination revealed purpura in 3 cases, hepatomegaly and splenomegaly in 6 and 5 cases respectively, and peripheral lymphadenopathy in 1 case. Hemorrhagic complications were observed in 5 cases, hypertriglyceridemia in 9 cases, and hyperferritinemia in all cases. Hypothyroidism was observed in all cases, and impaired renal function was detected in 11 of them, with 5 experiencing it as a result of lupus nephritis, and 1 case attributed to pre-eclampsia. Hemophagocytosis was confirmed through sternal puncture in 11 cases. Treatment involved etiological therapy with corticosteroids and immunosuppressants and/or anti-infectives. Intravenous immunoglobulins were administered in 6 cases, while 2 cases required coagulation factor transfusions. Unfortunately, 9 patients did not survive. Conclusion: The study highlights the need for increased awareness and prompt recognition of HLH, particularly in patients with associated renal complications.

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Reactive macrophage activation ayndrome: validation of a simple screening strategy and its potential in early treatment initiation

Cited by 30 publications

References 21 publications

Hemophagocytic syndrome in dogs: 24 cases (1996–2005)

Hemophagocytic syndrome in dogs: 24 cases (1996–2005)

Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis

Uncovering the Spectrum of Hemophagocytic Lymphohistiocytosis: A Nephrology Department’s Analysis of 14 Cases

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