Reversal of pure red cell aplasia by varicella zoster virus infection

Abstract

Background: Pure red cell aplasia is characterized by anemia, reticulocytopenia and diminished bone marrow erythroid precursors. It has multifactorial etiology and consequently several therapeutic interventions. Case:In August 2017, a young patient was diagnosed to have pure red cell aplasia. She was given immunosuppressive therapy for approximately two months but this treatment was stopped due to intolerance. Later on she developed herpes zoster infection that was treated with valacyclovir. Subsequently, it was noted that the patient became blood transfusion independent due to normalization of her hemoglobin and regeneration of the erythroid precursors in the bone marrow.Discussion: Varicella zoster virus behaves differently from other members of the herpes group of viruses such as cytomegalovirus and Epstein-Barr virus. Two retrospective studies, performed in patients with malignant hematological disorders and bone marrow failure, have shown that infection with the virus may cause stimulation of the three cell lines in the bone marrow and superior overall survival. Conclusion:The outcome of the patient presented confi rms the fi ndings of the two studies showing long-term benefi cial effects of varicella zoster virus infections in immunocompromised individuals.

The beneficial effects of varicella zoster virus

“…Recently, Al-Anazi KA, et al reported reversal of pure red cell aplasia (PRCA) by VZV infection [3]. A patient with BM biopsy proven PRCA was initially treated with immunosuppressive therapy, but this treatment was discontinued due to intolerance reported by the patient.…”

“…A patient with BM biopsy proven PRCA was initially treated with immunosuppressive therapy, but this treatment was discontinued due to intolerance reported by the patient. Two months after stopping cyclosporine-A and prednisolone, the patient developed localized herpes zoster (HZ) infection that was successfully treated with valaciclovir [3]. Six weeks after the VZV infection, Hb level started to increase gradually till the patient became packed red blood cell transfusion independent few months later.…”

Varicella zoster virus: The potentially useful virus

“…Varicella zoster virus (VZV), a double-stranded DNA virus, is a highly contagious human neurotropic virus that belongs to the alpha group of herpes viruses [1][2][3][4]. Primary VZV infection (chickenpox) occurs in childhood then the virus becomes latent in the nerve ganglia [1,[5][6][7].…”

“…Despite encountering VZV infections in patients with worse expected prognosis, the OS in patients who developed VZV infection was superior to that in patients who never developed the infection [19]. Recently, Al-Anazi KA et al, reported BM biopsy-proven reversal of pure red cell aplasia manifested by a gradual increase in Hb level starting 6 weeks following a localized HZ infection till the Hb level plateaued above 14g/dL fourteen months following the viral infection [4]. Additionally, several studies have demonstrated that VZV infection may trigger chronic graft versus host disease (GVHD) following allogeneic HSCT [20][21][22].…”

“…Apparently, VZV behaves differently from other herpes viruses. Additionally, it has the following peculiar features: (1) having the smallest genome; (2) losing almost all the genes that are not essential for its survival; (3) being highly fusogenic and cell-associated; (4) having no inhibitors of autophagy; (5) being an exclusively human pathogen; (6) having a species-speci ic cytokine pro ile; (7) having an inverse relationship with glioma; (8) association with GVHD in recipients with allogeneic HSCT; and (9) having BM stimulatory effects as well as several antitumor actions in patients with HMs and BM failure syndromes [1][2][3][4][18][19][20][21][22]34,35,86,87].…”