Visceral varicella-zoster after bone marrow transplantation: report of a case series and review of the literature

Abstract

Objectives: Infection with varicella-zoster virus after bone marrow transplantation (BMT) is a common cause of morbidity and mortality. Visceral involvement with varicella-zoster may be incorrectly ascribed to graft-versus-host disease, resulting in delayed diagnosis and misguided therapy.

Methods: A 4-yr retrospective chart review was performed to determine the presenting symptoms and clinical outcome of visceral varicella-zoster virus infection in BMT recipients.

Results: Ten BMT recipients who subsequently developed visceral varicella-zoster virus infection were identified. The mean age at diagnosis was 40 yr (range 27-56 yr). Primary hematological malignancies were leukemia (N = 7), myelodysplasia (N = 2), and myelofibrosis (N = 1). Bone marrow transplants in affected patients were autologous (N = 2), related allogeneic (N = 5), or matched unrelated allogeneic (N = 3). The mean time interval from BMT to symptomatic visceral varicella-zoster virus infection was 153 days (range 60-280 days). Presenting symptoms included abdominal pain in all patients, nausea (60%), fever > 38 degrees C (60%), vomiting (50%), pneumonitis (50%), skin rash (40%), and diarrhea (30%). All patients had moderately or profoundly elevated aminotransferases and most had elevated pancreatic enzymes (80%). The mean time interval from the development of abdominal pain to the characteristic skin rash and then diagnosis was 6 and 7 days, respectively (range 4-10 and 4-14 days). Active graft-versus-host disease had previously been documented in five of the eight allogeneic BMT recipients. Immunosuppressive medications were increased at the onset of the abdominal pain in seven of these eight patients for suspected exacerbation of graft-versus-host disease. After recognition of varicella infection, antiviral therapy was promptly initiated; despite this, mortality was still 50%.

Conclusions: Visceral involvement with varicella-zoster virus infection can occur as a late complication after both allogeneic and autologous BMT. In these cases, symptoms of severe abdominal pain with associated nausea, vomiting, and diarrhea and elevated liver and pancreatic enzymes preceded the vesicular skin eruption and were confused with graft-versus-host disease. With the increasing application of high-dose chemotherapy followed by stem cell rescue for both hematological and solid tumors, clinicians should be aware of this potentially treatable and often lethal complication.