Biomarker and morphological characteristics of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis

Imashuku, Shinsaku; Hibi, Shigeyoshi; Tabata, Yasuhiro; Shinoda, Masahiro; Sekine, Yuriko; Hirayama, Kyosuke; Sakazaki, Hiromi; Maeda, Naoko; Kito, Hideyuki; Shichino, Hiroyuki; Mugishima, Hideo

doi:10.1002/(sici)1096-911x(199809)31:3<131::aid-mpo1>3.0.co;2-c

Cited by 43 publications

(8 citation statements)

References 34 publications

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“…The starting point of this study was that both HPS and HIV infection are cytokine diseases, that are associated with high levels of cytokines in the patients' sera and plasma. As expected, the levels of IFN‐ γ [2,7,8,10,12,17,18] and IL‐10 [10] were higher in the plasma of the HPS patient than in that of the control patients, as previously reported. However, MIP‐1 β levels were also higher, and that has never been reported before.…”

Section: Discussionsupporting

confidence: 84%

“…Epstein–Barr virus (EBV) is the virus most frequently involved. Recently, a spectrum of malignant T lymphoproliferative disorders such as childhood T or T/natural killer (NK) cell lymphoma and HPS, has been demonstrated to be frequently associated with EBV infection [5–8]. HPS has also been described in association with HIV infection, but only in a few cases [9].…”

Section: Introductionmentioning

confidence: 99%

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High levels of IL-10 and determination of other cytokines and chemokines in HIV-associated haemophagocytic syndrome

Benveniste

Dereuddre-Bosquet

Clayette

et al. 2000

Clinical and Experimental Immunology

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SUMMARYHaemophagocytic syndrome (HPS) and HIV infection are both associated with cytokine network dysregulation. We therefore analysed plasma levels and mRNA synthesis in peripheral blood mononuclear cells (PBMC) of cytokines, chemokines and chemokine receptors in one HIV-infected patient with HPS. We compared the results with those for eight HIV-infected patients with similar CD4 1 T cell counts (207/mm 3 versus controls: median 214/mm 3 ) and plasma virus load (4´1 log copies/ml, versus controls: median 4´2 log copies/ml). The HPS patient had a lower viral DNA load in PBMC and higher plasma levels of interferon-gamma, IL-10, and macrophage inflammatory protein (MIP)-1b . No difference in plasma tumour necrosis factor-alpha (TNF-a ), IL-6 and MIP-1a concentration was observed between the HPS patient and control patients. No difference was observed in TNF-a , IL-1b , IL-10, IL-4, MIP-1a , MIP-1b , RANTES, CXCR-4, and CCR-5 mRNA levels in PBMC, but IL-6 levels were higher in the HPS patient. Our results emphasize the role of IL-10 in the control of immune hyperactivation that is observed in HPS.

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Section: Discussionsupporting

confidence: 84%

Section: Introductionmentioning

confidence: 99%

High levels of IL-10 and determination of other cytokines and chemokines in HIV-associated haemophagocytic syndrome

Benveniste

Dereuddre-Bosquet

Clayette

et al. 2000

Clinical and Experimental Immunology

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“…Some authors suggest that HPS is directly triggered by TNF-α produced by EBV-infected T/NK cells [37]. Others underline its significance as a poor prognostic factor [30], and there are those who put in evidence the role of "bystander" (non-neoplastic) lymphocytes which appear to be infected more often than lymphoma cells and therefore are supposed to be involved in the development of infection-associated HPS (IHPS) [70].…”

Section: Discussionmentioning

confidence: 99%

Bone marrow biopsy in hemophagocytic syndrome

et al. 2002

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“…The selective role of INF-γ, secreted by virus-activated cytotoxic cells and NK effectors, in the manifestation of HLH was demonstrated in a perforin knock-out mouse model [62]. The alpha chain of the soluble IL-2 receptor (sCD25) appears to be a good disease marker because of constantly increased levels during active HLH [52,71,102]. In addition, sCD25 is also a marker for CNS disease in HLH.…”

Section: High Levels Of Cytokines Characterize Hemophagocytic Lymphohmentioning

confidence: 99%

Familial and acquired hemophagocytic lymphohistiocytosis

2006

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of severe hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Cardinal signs and symptoms are prolonged fever, hepatosplenomegaly and pancytopenia. Characteristic biochemical markers include elevated triglycerides, ferritin and low fibrinogen. HLH occurs on the basis of various inherited or acquired immune deficiencies. Impaired function of natural killer (NK) cells and cytotoxic T-cells (CTL) is shared by all forms of HLH. Genetic HLH occurs in familial forms (FHLH) in which HLH is the primary and only manifestation, and in association with the immune deficiencies Chédiak-Higashi syndrome 1 (CHS 1), Griscelli syndrome 2 (GS 2) and x-linked lymphoproliferative syndrome (XLP), in which HLH is a sporadic event. Most patients with acquired HLH have no known underlying immune deficiency. Both acquired and genetic forms are triggered by infections, mostly viral, or other stimuli. HLH also occurs as a complication of rheumatic diseases (macrophage activation syndrome) and of malignancies. Several genetic defects causing FHLH have recently been discovered and have elucidated the pathophysiology of HLH. The immediate aim of therapy in genetic and acquired HLH is suppression of the severe hyperinflammation, which can be achieved with immunosuppressive/immunomodulatary agents and cytostatic drugs. Patients with genetic forms have to undergo stem cell transplantation to exchange the defective immune system with normally functioning immune effector cells. In conclusion, awareness of the clinical symptoms and of the diagnostic criteria of HLH is crucial in order not to overlook HLH and to start life-saving therapy in time.

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Biomarker and morphological characteristics of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis

Cited by 43 publications

References 34 publications

High levels of IL-10 and determination of other cytokines and chemokines in HIV-associated haemophagocytic syndrome

High levels of IL-10 and determination of other cytokines and chemokines in HIV-associated haemophagocytic syndrome

Bone marrow biopsy in hemophagocytic syndrome

Familial and acquired hemophagocytic lymphohistiocytosis

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