2014
DOI: 10.1097/md.0000000000000022
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Hemophagocytic Lymphohistiocytosis

Abstract: To investigate the clinical features of adult patients with hemophagocytic lymphohistiocytosis (HLH) and to explore possible risk factors for death, we retrospectively reviewed the medical records of 103 adult HLH patients hospitalized from 1997 to 2012. We analyzed the underlying diseases, clinical characteristics, 1aboratory findings, outcomes, and prognostic factors. The most common cause of HLH was hematologic malignancies (n = 49), followed by infectious diseases (n = 24) and autoimmune disorders (n = 14)… Show more

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Cited by 187 publications
(103 citation statements)
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“…These abnormalities are not usually considered a feature of adult HLH, although this case, like others 8, suggests that this may be a wrong assumption. Compared to other published cases of adult HLH 2, 8, our case has a detailed documentation of HLH neurological involvement and resembles a children primary HLH. Moreover, it has the particularity of remission of HLH markers after treatment with an almost complete resolution of CNS lesions, and the resurgence of CNS lesions without reappearance of HLH blood markers leading to death and the post‐mortem diagnosis of IVL.…”
Section: Discussionmentioning
confidence: 62%
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“…These abnormalities are not usually considered a feature of adult HLH, although this case, like others 8, suggests that this may be a wrong assumption. Compared to other published cases of adult HLH 2, 8, our case has a detailed documentation of HLH neurological involvement and resembles a children primary HLH. Moreover, it has the particularity of remission of HLH markers after treatment with an almost complete resolution of CNS lesions, and the resurgence of CNS lesions without reappearance of HLH blood markers leading to death and the post‐mortem diagnosis of IVL.…”
Section: Discussionmentioning
confidence: 62%
“…T‐cell lymphoma and Epstein‐Barr virus infection are the most common causes of adult HLH 1, 2, 3. B‐cell lymphoma presenting with HLH is less common and it is mostly seen in the Asian‐variant of IVL 9, but not in western countries 9, 10.…”
Section: Discussionmentioning
confidence: 99%
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“…Se caracteriza clínicamente por fiebre, hepatoesplenomegalia, citopenias, hiperferritinemia, hipertrigliceridemia e hipofibrinogenemia. El tratamiento se basa principalmente en el control de la enfermedad de base, sin embargo, en muchos casos se requieren esteroides y quimioterapia (2,3), y de todas maneras, la mortalidad es alta (4).…”
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