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. 2013 Aug;162(3):376-82.
doi: 10.1111/bjh.12386. Epub 2013 May 21.

Treatment of Epstein Barr Virus-Induced Haemophagocytic Lymphohistiocytosis With Rituximab-Containing Chemo-Immunotherapeutic Regimens

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Free PMC article

Treatment of Epstein Barr Virus-Induced Haemophagocytic Lymphohistiocytosis With Rituximab-Containing Chemo-Immunotherapeutic Regimens

DeepakBabu Chellapandian et al. Br J Haematol. .
Free PMC article

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a life threatening complication of Epstein-Barr virus (EBV) infection. The anti-CD20 antibody rituximab depletes B cells, leading to improved outcomes for patients with EBV-associated B-lymphoproliferative disorders. To gather data on the use of rituximab in EBV-HLH, we performed a retrospective investigation involving 42 EBV-HLH patients who had received treatment with rituximab-containing regimens. On average, patients received 3 rituximab infusions (range 1-10) at a median dose of 375 mg/m(2) . In all patients, rituximab was administered with other HLH-directed medications, including steroids, etoposide and/or ciclosporin. Rituximab-containing regimens appeared well tolerated and improved clinical status in 43% of patients. Examination of laboratory data obtained prior to and within 2-4 weeks after the first rituximab dose revealed significant reductions in EBV load (median load pre-rituximab: 114,200 copies/ml, median post-rituximab: 225 copies/ml, P = 0.0001) and serum ferritin levels (median ferritin pre-rituximab: 4260 μg/l, median post-rituximab: 1149 μg/l, P = 0.001). Thus, when combined with conventional HLH-directed therapies, rituximab improves symptoms, reduces viral load and diminishes inflammation. These data support the incorporation of rituximab into future prospective clinical trials for patients with EBV-HLH.

Keywords: Epstein-Barr virus; haemophagocytic lymphohistiocytosis; macrophage activation syndrome; rituximab; x-linked lymphoproliferative disease.

Figures

Fig 1
Fig 1. Laboratory parameters before and after administration of the first dose of rituximab
Box plots representing laboratory values prior to and within 2 – 4 weeks after delivery of the first dose of rituximab. The central line of the box plots represents the median value with the whiskers representing the minimum and maximum levels. Laboratory parameters included: Epstein-Barr virus (EBV) DNA levels (n=36; A), ferritin (n=35; B), white blood cell count (WBC, n=37; C), absolute neutrophil count (ANC, n=36; D); haemoglobin concentration (Hb, n=37; E), platelet count (PLT, n=37; F), alanine transaminase (ALT, n=36; G), aspartate transaminase (AST, n=36; H). Statistical associations between pre- and post-rituximab values were calculated using the Wilcoxon matched-pairs signed rank test.
Fig 2
Fig 2. Probability of survival
Kaplan-Meier estimates of the probability of survival for the 42 patients in this cohort based on a combined reduction in viral load to ≤1,500 copies/ml and ferritin to ≤1,000 μg/l (A), reduction in viral load alone (B) or reduction in ferritin alone (C). Statistical differences between groups were evaluated using the log-rank test.

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