Hemophagocytic lymphohistiocytosis: review of etiologies and management

George, Melissa R.

doi:10.2147/jbm.s46255

Cited by 379 publications

(470 citation statements)

References 257 publications

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“…32,33 HLH can occur as primary and secondary forms, and infection is a major cause (∼49%) of secondary HLH. 34 The most common pathogen is Epstein-Barr virus, but zoonotic diseases including many rickettsial diseases and severe fever with thrombocytopenia syndrome, a tick-borne viral disease emerging in East Asia, are important causes of HLH. 35,36 The etiologic agent in a secondary HLH case may be difficult to identify in a timely manner, because many diagnostic tests are not available in every hospital and need to be referred to reference laboratories.…”

Section: Discussionmentioning

confidence: 99%

Human Granulocytic Anaplasmosis as a Cause of Febrile Illness in Korea Since at Least 2006

Yi¹,

Kim²,

Ko³

et al. 2017

The American Society of Tropical Medicine and Hygiene

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Human granulocytic anaplasmosis (HGA) is a tick-borne rickettsial zoonosis with fever, thrombocytopenia, and leukopenia. HGA has been reported in Korea in 2013 but it is uncertain how long it has existed. A retrospective study was conducted on patients who underwent bone marrow examination due to fever and cytopenia, with no clear hematologic or microbiologic causes, from 2003 through 2012. Laboratory diagnosis was made by detecting 16S rRNA genes of Anaplasma phagocytophilum from the stored blood samples. Among the 70 patients, five (7.1%) HGA cases were found, and the earliest case dated back to 2006. Two cases met the diagnostic criteria of hemophagocytic lymphohistiocytosis (HLH) and were fatal. Although HGA has been prevalent in Korea since at least 2006, it is not always diagnosed and has posed a possible lethal health risk to the people in Korea. HGA should be considered as a cause of fever with cytopenia or HLH.

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Section: Discussionmentioning

confidence: 99%

Human Granulocytic Anaplasmosis as a Cause of Febrile Illness in Korea Since at Least 2006

Yi¹,

Kim²,

Ko³

et al. 2017

The American Society of Tropical Medicine and Hygiene

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“…Hemophagocytic lymphohistiocytosis (HLH) or hemophagocytic syndrome is a severe clinical syndrome characterized by sustained activation of the mononuclear phagocytic system resulting in an uncontrolled hyperinflammatory response [1]. HLH is triggered by an underlying immune stimulating disease or illness.…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis

2015

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Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by the activation of the mononuclear phagocytic system. The diagnosis of HLH in adults is challenging not only because the majority of the reported data are from pediatric patients, but also because HLH occurs in many disease entities. This study reports the clinical and laboratory findings and prognostic factors of adult HLH in a large cohort managed at a single medical center from 2003 to 2014. Seventy-three patients met the HLH-2004 diagnostic criteria. The median age was 51 years (range, 18-82 years); 41 (56.2%) were male. Patients manifested fever, cytopenias, and elevated ferritin in >85% of cases. Likely causes of HLH were as follows: 30 (41.1%) infections, 21 (28.8%) malignancies, 5 (6.8%) attributed to autoimmune disorders, 1 (1.4%) primary immunodeficiency, 2 (2.7%) post solid organ transplantation, and 13 (17.8%) idiopathic. The median overall survival was 7.67 months. Patients with malignancy-associated HLH had a markedly worse survival compared with patients with non-malignancy-associated HLH (median overall survival 1.13 vs. 46.53 months, respectively; P < 0.0001). In a multivariable analysis, malignancy (hazard ratio 5 12.22; 95% CI: 2.53-59.02; P 5 0.002) correlated with poor survival. Ferritin >50,000 mg/L correlated with 30-day mortality. Survival after a diagnosis of HLH is dismal, especially among those with malignancy-associated HLH. The development of a registry for adults with HLH would improve our understanding of this syndrome, validate diagnostic criteria, and help develop effective treatment strategies.

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“…Although the precise mechanism leading to HLH in CD3δ deficiency remains to be determined, it is plausible to assume that the absence of cytotoxic T cells, even in the presence of normal NK cells, may be a critical predisposing factor (George 2014). This has also been observed in some case reports of PID that are not typically affecting the NK cell number or function (Introne et al 1999;Menasche et al 2000;Cesaro et al 2003;Pasic et al 2003;Faitelson and Grunebaum 2014).…”

Section: Discussionmentioning

confidence: 97%