Alternative therapy for epstein-barr virus related hemophagocytic lymphohistiocytosis

Case Reports in Oncological Medicine
Omar Al AsadBagi Jana

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rapidly fatal condition characterized by excessive immune activation. HLH can occur as a familial or sporadic acquired disorder. Acquired HLH is more frequently found in adults and is commonly secondary to infections, malignancies, or autoimmune diseases. Diagnosing HLH is challenging because of the rare occurrence, variable presentation, and nonspecific findings of this disorder. Diagnosis of HLH can be based on the diagnostic criteria which were used in the HLH-2004 trial. Given the rarity of this disease, protocols for its treatment have developed slowly, and obtaining adequate short-term and long-term control of the disease continues to be a challenge. Conventional induction therapy for HLH is dexamethasone and etoposide (VP-16), followed by or with cyclosporine. Intrathecal methotrexate ± hydrocortisone is given to those with central nervous system disease. We are reporting a patient who was diagnosed with Epstein-Barr virus (EBV) related HLH. He achieved complete remission with rituximab alone. To our knowledge, this is the first case of an adult patient with EBV related HLH who went into remission with rituximab therapy alone, without using the conventional chemotherapy.

References

Aug 29, 2006·Pediatric Blood & Cancer·Jan-Inge HenterGritta Janka
Mar 24, 2007·International Journal of Clinical Oncology·Takeshi SanoHitoshi Ohno
Aug 11, 2011·Blood·Michael B JordanKenneth L McClain
May 23, 2013·British Journal of Haematology·DeepakBabu ChellapandianUNKNOWN EBV-HLH Rituximab Study Group

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Citations

Aug 9, 2016·Transplant Infectious Disease : an Official Journal of the Transplantation Society·I RomiopoulosE Roilides

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Methods Mentioned

BETA
biopsy

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