Poor Outcomes of Chronic Active Epstein‐Barr Virus Infection and Hemophagocytic Lymphohistiocytosis in Non‐Japanese Adult Patients

Sonke, Gabe S.; Ludwig, Inge; Oosten, Hannah van; Baars, Joke W.; Meijer, Ellen; Kater, Arnon P.; Jong, Daphne de

doi:10.1086/588790

Cited by 36 publications

(27 citation statements)

References 15 publications

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“…Fifty-six individual cases of CAEBV treated with allogeneic HSCT from Japan 12,14,33-47 and 1 case from the Netherlands 48 have been reported in the literature (Table 7). Forty-six percent (26/57) were T cell in origin, 42% (24/57) were NK cell, 5% (3/57) were NK/T, and 7% (4/57) were of undetermined origin; none were B cell in origin.…”

Section: Chronic Active Ebv Disease In the United States 5845mentioning

confidence: 99%

Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States

Cohen

Jaffe

Dale

et al. 2011

Blood

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226

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Section: Chronic Active Ebv Disease In the United States 5845mentioning

confidence: 99%

Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States

Cohen

Jaffe

Dale

et al. 2011

Blood

244

226

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“…HLH is rare, and in adults, it mainly presents as the secondary form. It progresses rapidly and has a high mortality rate despite appropriate management [11,12].…”

Section: Introductionmentioning

confidence: 99%

Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors

et al. 2011

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Although hemophagocytic syndrome (HS) featuring secondary hemophagocytic lymphohistiocytosis (HLH) has a grave prognosis, little is known about the natural course of the disease. Patients who showed the clinical features of HLH as well as tissue-proven hemophagocytosis when seen at Asan Medical Center between 1999 and 2010 were included in this analysis. Patients with proven lymphoma were excluded. The median age of our 23 study patients was 49 years. Epstein-Barr virus was suspected to have caused HS in 16 (70%) patients and hepatitis A virus in one patient. Twenty-two patients were treated, 13 according to the HLH protocol and nine using immunosuppressive agents such as corticosteroid and/or cyclosporine. Five patients undertook allogeneic hematopoietic cell transplantation (HCT) during their treatment-dependent relapse (n = 4) or responsive status (n = 1). After the median follow-up of 180 days, 17 (74%) died and six (26%) were alive. The median time from initial presentation until death was 41 days among those patients who died. The serum fibrinogen level ≥166 mg/dL determined at the initial visit was significantly associated with the survival time according to univariate analysis. The low histiocyte proportion in bone marrow and early initiation of treatment tended to correlate with a favorable outcome. On multivariate analysis, serum fibrinogen ≥166 mg/dL (hazard ratio, 0.175, P = 0.018) was an independent clinical factor for determining the patient survival time. Despite appropriate patient management, the outcome of HS featuring HLH was grave. The serum fibrinogen level at the initial presentation was significant, and selected patients obtained some benefit from allogeneic HCT.

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“…Patients with CAEBV infection have unexplained illness lasting > 6 months and may have prolonged intermittent fever, continuous debilitating fatigue, sore throat, malaise, arthralgia, myalgia, or evidence of organ involvement like lymphadenopathy, hepatosplenomegaly, pneumonitis, uveitis, etc. [3,4].…”

Section: Discussionmentioning

confidence: 99%

Chronic Active Epstein-Barr Virus Infection Mimicking Diabetic Ketoacidosis

Pattan

2014

J Endocrinol Metab

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Epstein-Barr virus (EBV) is a widely disseminated herpes virus that spreads by intimate contact between susceptible person and symptomatic EBV shedders. Most patients recover without complications. Chronic active Epstein-Barr virus (CAEBV) infection is a very rare complication of EBV in which previously healthy persons develop severe, life-threatening illness. We report a rare case of a CAEBV infection in a 20-year-old female mimicking a common clinical presentation of diabetic ketoacidosis.

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Poor Outcomes of Chronic Active Epstein‐Barr Virus Infection and Hemophagocytic Lymphohistiocytosis in Non‐Japanese Adult Patients

Cited by 36 publications

References 15 publications

Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States

Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States

Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors

Chronic Active Epstein-Barr Virus Infection Mimicking Diabetic Ketoacidosis

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