دورية أكاديمية
Hemophagocytic lymphohistiocytosis: presentation and outcome of twenty-one patients at a single institution.
| العنوان: | Hemophagocytic lymphohistiocytosis: presentation and outcome of twenty-one patients at a single institution. |
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| المؤلفون: | Rodrigues JB; Pediatric Oncology, Instituto de Oncologia Pediátrica (IOP/GRAACC), São Paulo, SP, Brazil; Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil. Electronic address: jessicabenignor@hotmail.com., Nasr BP; Pediatric Oncology, Instituto de Oncologia Pediátrica (IOP/GRAACC), São Paulo, SP, Brazil; Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil., Cypriano MDS; Pediatric Oncology, Instituto de Oncologia Pediátrica (IOP/GRAACC), São Paulo, SP, Brazil. Electronic address: monicacypriano@graacc.org.br. |
| المصدر: | Hematology, transfusion and cell therapy [Hematol Transfus Cell Ther] 2022 Oct-Dec; Vol. 44 (4), pp. 485-490. Date of Electronic Publication: 2021 Jun 24. |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Elsevier Editora Ltda Country of Publication: Brazil NLM ID: 101725732 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2531-1387 (Electronic) Linking ISSN: 25311379 NLM ISO Abbreviation: Hematol Transfus Cell Ther Subsets: PubMed not MEDLINE |
| أسماء مطبوعة: | Original Publication: [Rio de Janeiro, Brazil] : Elsevier Editora Ltda, [2018]- |
| مستخلص: | Introduction: Hemophagocytic lymphohistiocytosis comprises a systemic hyperactivation of macrophages that requires prompt recognition of symptoms and early treatment. Objective and Method: In this context, we described clinical and laboratory characteristics, therapeutic modality and outcome of 21 patients with HLH treated at a pediatric oncology hospital between January 2000 and February 2019. Results: HLH mainly affected females, fever was the most frequent clinical sign and hyperferritinemia was the most prevalent laboratory abnormality. All patients were admitted to the intensive care unit (ICU) at some point. Fifteen (71.4%) patients presented resolution criteria and eight (53.3%) of them presented reactivation. The mortality rate was 57.1% and the mean time between diagnosis and death was 9.98 months. The 5-year overall survival (OS) was 36.7%. We observed a significant difference in prognosis associated with reactivation of HLH. These patients demonstrated an estimated 5-year OS of 25%, while all patients that did not reactivate were alive until the end of the follow-up. Conclusion: In conclusion, HLH is a rare disease with a high mortality rate, especially in patients with disease reactivation and those with familial- or immunodeficiency-associated forms, which makes early recognition and genetic testing crucial for appropriate management and prompt SCT indication. Competing Interests: Conflicts of interest The author declares no conflicts of interest. (Copyright © 2021. Published by Elsevier España, S.L.U.) |
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| فهرسة مساهمة: | Keywords: Cytokine storm; Cytopenia; Hemophagocytic lymphohistiocytosis; Hyperferritinemia; Macrophage activation syndrome |
| تواريخ الأحداث: | Date Created: 20210709 Latest Revision: 20221029 |
| رمز التحديث: | 20240628 |
| مُعرف محوري في PubMed: | PMC9605882 |
| DOI: | 10.1016/j.htct.2021.05.003 |
| PMID: | 34238727 |
| قاعدة البيانات: | MEDLINE |
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Full Text Finder | تدمد: | 2531-1387 |
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| DOI: | 10.1016/j.htct.2021.05.003 |