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Efficacy of Moderat...
Efficacy of Moderately Dosed Etoposide in Macrophage Activation Syndrome-Hemophagocytic Lymphohistiocytosis
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- Horne, A. (författare)
- Karolinska Institutet
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- Greenwood, T. V. (författare)
- Karolinska Institutet
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- Chiang, S. C. C. (författare)
- Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med, Stockholm, Sweden.;Karolinska Univ Hosp Huddinge, Stockholm, Sweden.
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- Meeths, M. (författare)
- Karolinska Institutet
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- Bjorklund, C. (författare)
- Umeå Univ Hosp, Dept OfPediat Hematol & Oncol, Umeå, Sweden.
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- Ekelund, Maria (författare)
- Uppsala universitet,Pediatrisk inflammations- och metabolismforskning samt barnhälsa,Ryhov Cty Hosp, Dept OfPediat, Jönköping, Sweden.;Uppsala Univ, Dept Womens & Childrens Hlth, Uppsala, Sweden.
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- Erensjo, P. (författare)
- Falun Cent Hosp, Dept Pediat, Falun, Sweden.
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- Berg, Stefan, 1959 (författare)
- Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics,Univ Gothenburg, Sahlgrenska Acad, Inst Clin Sci, Dept Pediat, Gothenburg, Sweden.
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- Hagelberg, S. (författare)
- Karolinska Inst, Dept Womens & Childrens Hlth, Pediat Rheumatol Unit, Stockholm, Sweden.;Karolinska Univ Hosp, Theme Childrens Hlth, Stockholm, Sweden.
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- Bryceson, Y. T. (författare)
- Karolinska Institutet
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- Andersson, U. (författare)
- Karolinska Institutet
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- Henter, J. I. (författare)
- Karolinska Institutet
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Karolinska Institutet Karolinska Inst, Ctr Hematol & Regenerat Med, Dept Med, Stockholm, Sweden;Karolinska Univ Hosp Huddinge, Stockholm, Sweden. (creator_code:org_t)
- 2021-02-15
- 2021
- Engelska.
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Ingår i: Journal of Rheumatology. - : The Journal of Rheumatology. - 0315-162X .- 1499-2752. ; 48:10, s. 1596-1602
- Relaterad länk:
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https://doi.org/10.3...
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https://gup.ub.gu.se...
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https://doi.org/10.3...
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https://urn.kb.se/re...
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http://kipublication...
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Abstract
Ämnesord
Stäng
- Objective. Macrophage activation syndrome (MAS) constitutes 1 subtype of the hyperinflammatory syndrome hemophagocytic lymphohistiocytosis (HLH), and the term MAS-HLH was recently proposed for HLH with underlying autoimmune/autoinflammatory conditions. The mortality of MAS-HLH has been estimated at 5-10%. Here we report our experiences with moderately dosed etoposide in severe MAS-HLH; the objective was to effectively reduce severe hyperinflammatory activity with limited side effects. Methods. In addition to conventional antiinflammatory treatment, moderately dosed etoposide was administered to 7 children affected by rapidly progressing MAS-HLH with central nervous system (n = 5) and/ or pulmonary (n = 5) involvement. Three had underlying systemic juvenile idiopathic arthritis (sJIA), 2 had atypical sJIA (no arthritis at diagnosis), and 2 had systemic lupus erythematosus. We performed lymphocyte cytotoxicity analyses in all 7 and genetic analyses in 6. Results. All children promptly responded to moderately dosed etoposide (50-100 mg/m(2Y) once weekly), added to conventional MAS-HLH treatment that was considered insufficient. The mean accumulated etoposide dose was 671 mg/m(2) (range 300-1050 mg/m(2)) as compared to 1500 mg/m(2) recommended in the first 8 weeks of the HLH-94/HLH-2004 protocols. One child developed neutropenic fever and another neutropenic sepsis (neutrophils 0.3 x 10(9)/L at therapy onset). Five of 7 children had low percentages (< 5%) of circulating natural killer (NK) cells prior to or in association with diagnosis; NK cell activity was pathologically low in 2 of 5 children studied. Disease-causing variants in HLH-associated genes were not found. All children were alive at latest follow-up (2-9 yrs after onset); neurological symptoms had normalized in 4 of 5 affected children. Conclusion. Moderately dosed etoposide may be beneficial in severe and/or refractory MAS-HLH.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Reumatologi och inflammation (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Rheumatology and Autoimmunity (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Nyckelord
- Key Indexing Terms
- etoposide
- hemophagocytic lymphohistiocytosis
- juvenile idiopathic arthritis
- macrophage activation syndrome
- systemic
- lupus erythematosus
- systemic-lupus-erythematosus
- classification
- arthritis
- cells
- Rheumatology
- Key Indexing Terms
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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Till lärosätets databas
- Av författaren/redakt...
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Horne, A.
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Greenwood, T. V.
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Chiang, S. C. C.
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Meeths, M.
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Bjorklund, C.
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Ekelund, Maria
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Erensjo, P.
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Berg, Stefan, 19 ...
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Hagelberg, S.
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Bryceson, Y. T.
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Andersson, U.
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Henter, J. I.
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- Av lärosätet
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Göteborgs universitet
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Uppsala universitet
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Karolinska Institutet