Assessment of the HScore for reactive haemophagocytic syndrome in patients with rheumatic diseases

Batu, Ezgi Deniz; Erden, Abdülsamet; Seyhoglu, E.; Kılıç, Levent; Büyükaşık, Yahya; Karadağ, Ömer; Bilginer, Yelda; Bilgen, Ş. A.; Akdoğan, Ali; Kiraz, Sedat; Ertenli, A. I.; Özen, Seza; Kalyoncu, Umut

doi:10.3109/03009742.2016.1167951

Cited by 51 publications

(46 citation statements)

References 21 publications

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“…Here we present the largest population of adult SLE-associated MAS evaluated by the H-score so far. Our data seem to confirm the utility of the H-score in this setting, even if it should always be considered that sensitivity may not be 100% (13,17). However, since in our study all the patients also fulfilled the HLH-2004 classification criteria (10), it was not possible to evaluate the sensitivity of the H-score as compared with the HLH-2004 criterion, the main limitation of which is indeed the lack of sensitivity, especially at the beginning of the MAS.…”

Section: Original Papersupporting

confidence: 67%

“…Median HLH score was 248 (IQR 226-263), with a median probability of having MAS of 99% (Table II). In all patients the Hscore was higher than 169, the cut-off for classification of reactive MAS in the original study (13); however, in one patient the score was lower than 190.5, a cut-off suggested by other authors to better perform in the classification of reactive MAS in patients with rheumatic diseases (17). In three cases bone marrow aspirate was not performed; nevertheless, the H-score was higher than the suggested cut-offs in all these cases (Table II).…”

Section: Original Papermentioning

confidence: 92%

“…The H-score was designed to calculate the individual risk of adult patients having reactive MAS (15); however it was developed and validated in a population including a very small number of patients with SLE (and other autoimmune diseases) (15). The performance of the H-score in the classification of MAS associated with rheumatic diseases was recently evaluated in a series of 30 patients, including 6 cases of SLE (17). In this study, a cut-off value for the H-Score different from the original study performed better.…”

Section: Original Papermentioning

confidence: 99%

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Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

et al. 2018

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SUMMARYThe aim was to describe the macrophage activation syndrome (MAS), a life-threatening syndrome characterized by excessive immune activation that can be triggered by conditions affecting immune homeostasis, in a cohort of adult Italian patients with systemic lupus erythematosus (SLE). This was a monocentric retrospective evaluation. The utility of the H-score, developed to estimate the individual risk of having reactive MAS in adult patients, was assessed. Among 511 patients with SLE, 7 cases (1.4%) of MAS (all females) were identified and their medical records reviewed. In all cases, MAS was simultaneous to the onset of SLE. All patients had fever, lymphadenopathy, hematological involvement, and high titer of anti-dsDNA antibodies. Workup for infections and malignancies was negative. In all cases, the H-score was higher than the cut-off suggested for the classification of reactive MAS. All cases required hospital admission, and 2 patients were admitted to the intensive care unit. Most patients were treated successfully with high doses of corticosteroids and with immunosuppressive drugs, whereas the full therapeutic regimen developed for primary hemophagocytic lymphohistiocytosis HLH was used only in one case. No death from MAS was observed. MAS is a rare and severe disorder that complicated the onset of SLE in our cohort. The H-score may be useful in the classification of these patients.

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Section: Original Papersupporting

confidence: 67%

Section: Original Papermentioning

confidence: 92%

Section: Original Papermentioning

confidence: 99%

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Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

et al. 2018

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“…Several criteria have been developed for children, but these still need validation in adult patients (see Statement 3). 42,[106][107][108][109] Notably, symptoms of MAS-HLH may be different in patients treated with biologic agents 110.…”

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confidence: 99%

Recommendations for the management of hemophagocytic lymphohistiocytosis in adults

Rosée

Horne

Hines

et al. 2019

Blood

734

713

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Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic T cells. The primary (genetic) form, caused by mutations affecting lymphocyte cytotoxicity and immune regulation, is most common in children, whereas the secondary (acquired) form is most frequent in adults. Secondary HLH is commonly triggered by infections or malignancies but may also be induced by autoinflammatory/autoimmune disorders, in which case it is called macrophage activation syndrome (MAS; or MAS-HLH). Most information on the diagnosis and treatment of HLH comes from the pediatric literature. Although helpful in some adult cases, this raises several challenges. For example, the HLH-2004 diagnostic criteria developed for children are commonly applied but are not validated for adults. Another challenge in HLH diagnosis is that patients may present with a phenotype indistinguishable from sepsis or multiple organ dysfunction syndrome. Treatment algorithms targeting hyperinflammation are frequently based on pediatric protocols, such as HLH-94 and HLH-2004, which may result in overtreatment and unnecessary toxicity in adults. Therefore, dose reductions, individualized tailoring of treatment duration, and an age-dependent modified diagnostic approach are to be considered. Here, we present expert opinions derived from an interdisciplinary working group on adult HLH, sponsored by the Histiocyte Society, to facilitate knowledge transfer between physicians caring for pediatric and adult patients with HLH, with the aim to improve the outcome for adult patients affected by HLH.

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“…Of note, use of different cut-off values of the HScore might be necessary in patients with underlying rheumatic conditions [18]. EBV-related HLH secondary to hematological malignancies has been successfully treated with rituximab-containing regimens, the rationale being depletion of EBV-infected B-cells [19].…”

Section: Discussionmentioning

confidence: 99%

Combination Immunosuppressive Therapy Including Rituximab for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in Adult-Onset Still’s Disease

Schäfer

Jung

Korsten

2016

Case Reports in Rheumatology

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Hemophagocytic lymphopcytosis (HLH) is a life-threatening condition. It can occur either as primary form with genetic defects or secondary to other conditions, such as hematological or autoimmune diseases. Certain triggering factors can predispose individuals to the development of HLH. We report the case of a 25-year-old male patient who was diagnosed with HLH in the context of adult-onset Still's disease (AOSD) during a primary infection with Epstein-Barr virus (EBV). During therapy with anakinra and dexamethasone, he was still symptomatic with high-spiking fevers, arthralgia, and sore throat. His laboratory values showed high levels of ferritin and C-reactive protein. His condition improved after the addition of rituximab and cyclosporine to his immunosuppressive regimen with prednisolone and anakinra. This combination therapy led to a sustained clinical and serological remission of his condition. While rituximab has been used successfully for HLH in the context of EBV-associated lymphoma, its use in autoimmune diseases is uncommon. We hypothesize that the development of HLH was triggered by a primary EBV infection and that rituximab led to elimination of EBV-infected B-cells, while cyclosporine ameliorated the cytokine excess. We therefore propose that this combination immunosuppressive therapy might be successfully used in HLH occurring in the context of autoimmune diseases.

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Assessment of the HScore for reactive haemophagocytic syndrome in patients with rheumatic diseases

Abstract: In our study, a cut-off value for the HScore different from the original study performed better. Further studies are warranted to determine optimum cut-off values in different studies.

Cited by 51 publications

References 21 publications

Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center

Recommendations for the management of hemophagocytic lymphohistiocytosis in adults

Combination Immunosuppressive Therapy Including Rituximab for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in Adult-Onset Still’s Disease

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