Primary varicella-zoster virus infection of the immunocompromised associated with acute pancreatitis and hemophagocytic lymphohistiocytosis

Abstract

Rationale: Primary varicella-zoster virus (VZV) infection may be associated with hemophagocytic lymphohistiocytosis (HLH), as well as with acute pancreatitis. However, there is few data concerning the evolution and the optimal treatment of these rare associations. Patient concerns: A 57-year-old immunocompromised woman, who was treated for chronic lymphocytic leukemia 3 years prior to admission, was hospitalized with abdominal pain revealing severe acute pancreatitis. The day after admission, a pruritic rash appeared on her face, trunk, and limbs, sparing the palmoplantar regions. At the same time, fever, thrombocytopenia (27 × 10 9 /L), major hyperferritinemia (11,063 μg/mL), hypertriglyceridemia (2.56 mmol/L) and elevated lactate dehydrogenase levels (1441 IU/L) suggested HLH. Diagnosis: The diagnosis of chickenpox (varicella) was established. Primary VZV infection was then confirmed: cutaneous and plasma VZV polymerase chain reactions were positives, VZV serology was negative for IgG. Interventions: Treatment with aciclovir was started intravenously after the onset of the rash, for a total of 10 days. A 48-h surveillance in intensive care was carried out. Outcomes: Acute pancreatitis and biological abnormalities evolved favorably under aciclovir. Platelet count was normalized 6 days after admission to hospital. Lessons: A favorable outcome of primary VZV infection associated with severe acute pancreatitis and probable HLH in an immunocompromised patient is possible with aciclovir alone.

Impaired STING activation due to a variant in the E3 ubiqitin ligase AMFR in a patient with severe VZV infection and hemophagocytic lymphohistiocytosis

“…Interestingly, both familial and sporadic HLH might present with an infectious trigger, and herpesviruses are the most common causes of virus-induced HLH, with Epstein-Barr-virus (EBV) and cytomegalovirus (CMV) accounting for more than 50% of virus-associated HLH [27]. Similarly, VZV can trigger HLH upon primary infection or reactivation, although this is mostly reported in patients with underlying immunosuppression or fHLH [23,[29][30][31].…”

Bioinformatic analyses suggest augmented interleukin-17 signaling as the mechanism of COVID-19-associated herpes zoster

“…Herpes zoster (commonly known as shingles), common in middle age groups, is caused by the reactivation varicella zoster virus (VZV) and manifests as the painful vesicular rash along a dermatomal distribution (Nagel and Gilden 2013 ). VZV is also the causative agent of varicella (commonly known as chickenpox) that predominantly affects children under the age of 12 (Bérar et al 2021 , Shiraki et al 2021 ). Following the initial infection, this virus remains latent in the ganglion cells of dorsal root or cranial nerve (Fukuyasu et al 2021 , Osman 2020 , Zhu et al 2020 ).…”