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2018 ; 18
(1
): 190
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gab.com Text
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English Wikipedia
Interferon alpha as antiviral therapy in chronic active Epstein-Barr virus
disease with interstitial pneumonia - case report
#MMPMID29678144
Roli?ski J
; Grywalska E
; Pyzik A
; Dzik M
; Opoka-Winiarska V
; Surdacka A
; Maj M
; Burdan F
; Piro?y?ski M
; Grabarczyk P
; Staros?awska E
BMC Infect Dis
2018[Apr]; 18
(1
): 190
PMID29678144
show ga
BACKGROUND: Chronic active Epstein-Barr virus (EBV) disease (CAEBV) is defined as
a severe, progressive lymphoproliferative disorder associated with active EBV
infection persisting longer than 6 months and developing in patients without
recognised immunodeficiency. Rarely, interstitial pneumonitis (IP) occurs as a
serious complication in CAEBV patients. The standard therapeutic regimen for IP
in CAEBV has not yet been defined. Although interferon alpha (IFN-alpha) is known
to suppress viral DNA replication by affecting its basal promoter activation
process, it is rarely used in CAEBV patients. CASE PRESENTATION: A 22-year-old
Caucasian woman, diagnosed with CAEBV 1.5 years earlier, was admitted to the
Immunology Clinic due to a 4-week history of productive cough, fever and general
weakness. Cultures of blood, urine and sputum were negative, but EBV DNA copies
were found in the sputum, whole blood, isolated peripheral blood lymphocytes as
well as in the blood plasma. Cytokine assessment in peripheral blood revealed the
lack of IFN-alpha synthesis. Disseminated maculate infiltrative areas in both
lungs were observed on a computed tomography (CT) chest scan. The patient was not
qualified for the allogeneic hematopoietic stem cell transplantation (allo-HSCT)
due to the risk of immunosuppression-related complications of infectious IP.
Inhaled (1.5 million units 3 times a day) and subcutaneous (6 million units 3
times a week) IFN-alpha was implemented. To the best of our knowledge, this was
the first documented use of inhaled IFN-alpha in a patient with CAEBV and
concomitant IP. Patient's status has improved, and she was eventually qualified
to allo-HSCT with reduced conditioning. Currently, the patient feels well, no EBV
was detected and further regression of pulmonary changes was documented.
CONCLUSIONS: CAEBV should be considered in patients who present with interstitial
lung infiltration and involvement of other organs. Although more promising
results have been obtained with allo-HSCT, inhaled IFN-alpha may also be a
therapeutic option in patients with CAEBV and a concomitant IP.