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HHV6-Associated Hydrocephalus in a Pediatric Hematopoietic Stem Cell Transplant Recipient: An Unusual Presentation
Mohammed Al Nuaimi1,2, Aisha Al Khaaldi3,4, Omar Trad1
1Division of Pediatric Hematology and Oncology, Tawam Oncology Centre.
Insights
Human herpesvirus 6 (HHV-6) reactivation caused acute cerebellitis in a pediatric hematopoietic stem cell transplant (HSCT) patient. Prompt diagnosis and combined steroid and ganciclovir treatment led to significant recovery, highlighting the importance of rapid diagnostics.
Area of Science:
- Virology
- Immunology
- Neuroscience
Background:
- Human herpesvirus 6 (HHV-6) is a common DNA virus with primary infection in childhood.
- Reactivation is frequent in immunocompromised individuals, particularly hematopoietic stem cell transplant (HSCT) recipients, impacting outcomes.
- HHV-6 reactivation presents with diverse neurological manifestations, including post-transplant limbic encephalitis (PALE).
Observation:
- A 6-year-old female, post-allogeneic HSCT for beta thalassemia major, developed acute cerebellitis with hydrocephalus.
- Cerebrospinal fluid (CSF) and serum samples tested positive for HHV-6 via PCR.
- The patient exhibited significant clinical improvement following steroid and ganciclovir therapy.
Findings:
- HHV-6 reactivation was confirmed as the cause of cerebellitis and hydrocephalus in this HSCT patient.
- Combined antiviral (ganciclovir) and anti-inflammatory (steroid) treatment proved effective.
- The case underscores the potential for HHV-6 to cause severe neurological complications post-HSCT.
Implications:
- Early and accurate diagnosis of HHV-6 reactivation is critical for managing HSCT patients.
- A multidisciplinary approach is essential for addressing complex neurological complications.
- Timely intervention can significantly improve patient morbidity and mortality associated with HHV-6 reactivation.
Abstract:
Human herpesvirus 6 (HHV-6) is a widely spread DNA virus that is ubiquitous and persistent with primary infection occurring in early childhood, with reactivation of the infection a common phenomenon in severely immunocompromised hosts, including hematopoietic stem cell transplant (HSCT) patients, influencing morbidity and mortality. A wide spectrum of clinical presentations is reported in the literature with HHV-6 reactivation including post-transplant limbic encephalitis (PALE). We report the unusual case of a 6-year-old female 107 days postallogenic HSCT due to transfusion dependent beta thalassemia major who developed acute cerebellitis with secondary supratentorial hydrocephalus that required invasive surgical intervention. In addition to accompanying imaging findings, the patient tested positive for HHV-6 by PCR from both serum and CSF samples and demonstrated dramatic improvement with the institution of steroid therapy in addition to ganciclovir treatment. The availability of rapid diagnostic measures in addition to a multidisciplinary approach is crucial to manage HHV-6 encephalitis and associated complications in HSCT patients.
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