Fulminant Multiorgan Failure Due to Varicella Zoster Virus and HHV6 in an Immunocompetent Adult Patient, and Anhepatia

doi:10.1016/j.transproceed.2011.01.142

Transplantation Proceedings

Volume 43, Issue 4, May 2011, Pages 1184-1186

https://doi.org/10.1016/j.transproceed.2011.01.142 Get rights and content

Abstract

Varicella is a well-known contagious disease of childhood that can also affect both immunodepressed and immunocompetent adults. The present observations concern a previously healthy adult patient who presented with a fulminant hepatitis evolving in multiorgan failure (MOF), associated with an atypical papulo-ethemateous cutaneous rash without fever. An hepatic biopsy showed massive necrosis. Because of the persistent MOF and severe hemodynamic instability, total hepatectomy was performed as a bridge to urgent liver transplantation (OLT). Despite temporary improvement, the patients condition progressively deteriorated and he died 11 hours after the hepatectomy, i.e. 7 days after admission to the intensive care unit. High viral loads of varicella zoster virus (VZV) and human herpes virus 6 (HHV6) were demonstrated in the blood and in DNA at post mortem examination of the liver, kidneys, lung, and heart.

We hypothesize that VZV infection may occasionally occur in immunocompetent patients due to extremely virulent strains that can be rapidly fatal. The clinical influence of simultaneous infection with HHV6 is not clear. Moreover, the role of a previous steroid treatment as a trigger for a temporary immunodepressed state must be considered.

The diagnosis of liver disease from VZV should always be clinically suspected in the presence of concurrent atypical skin lesions and a temporarily immunocompromised state. Therapy with acyclovir was ineffective in our patient.

Based on the wide spectrum of VZV infections, fulminant MOF in immunocompetent adults must raise the possibility of VZV with simultaneous HHV6 infection with early listing of the patient for a urgent OLT, possibly with a total hepatectomy as a bridge, due to the therapeutic uncertainty of medical treatments.

Section snippets

Case Report

A previously healthy, 49-year-old man of 185 cm tall and 95 kg in weight was admitted to the emergency department of our hospital after the onset of acute retrosternal thoracic pain. Fifteen days prior he had suffered pharyngotonsillitis, which was treated with an antibiotic (ceftriaxon 1 g/d for 6 days) and corticosteroids (prednisone 5 mg tablet bid for 3 days, then 1 tablet/d for 3 days). Urgent laboratory tests showed an increased C-reactive protein of 2.8 mg/L (normal, <0.5) and marginally

Discussion

Varicella is generally diagnosed on the basis of clinical signs. However, because of a recent decline in the incidence of typical varicella, a prompt diagnosis of VZV infection can be problematic.⁷ In the present patient, despite a family history of varicella, there were several misleading factors. There was no history of immunodepression; the skin lesions appeared late and were not typical for varicella; the skin rash was not itchy and almost without heat; IgG and IgM for VZV were negative,

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Concurrent Thoracic Endovascular Aortic Repair and Liver Transplant: Multidisciplinary Management of Multiple Posttraumatic Lesions
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Only after the TEVAR aortic repair and the increase of BP, the patient developed the unresponsive abdominal bleeding because of irreversible liver injury that required the urgent listing for LT. Considering the shortage of available organs, the recipient selection in the setting of emergency transplantation is of paramount importance. Indeed, patients too sick for LT can be futile listed and transplanted.28,29 The ISS is primarily used to standardize severity of traumatic injuries.
Association of thoracic and abdominal injuries in patients with major trauma is common. Under emergency conditions, it is often difficult to promptly perform a certain diagnosis and identify treatment priorities of life-threatening lesions. We present the case of a young man with combined thoracic and abdominal injuries after a motorcycle accident. Primary evaluation through echography and X-ray showed fluid within the hepatorenal recess and an enlarged mediastinum. Volume load, blood transfusions, and vasoactive agents were initiated to sustain circulation. Despite hemodynamic instability, we decided to perform computed tomographic angiography (CTA) scan that revealed a high-grade traumatic aortic pseudoaneurysm, multiple and severe areas of liver contusion, and a small amount of hemoperitoneum, without active bleeding spots.
The patient was successfully submitted to thoracic endovascular aortic repair (TEVAR). Immediately after the end of the successful TEVAR, signs of massive abdominal bleeding revealed. Immediate explorative laparotomy was performed showing massive hepatic hemorrhage. After liver packing and Pringle's maneuver, control of bleeding was lastly obtained with hemostatic devices and selective cross-clamping of the right hepatic artery. The patient was then transferred to intensive care unit where, despite absence of further hemorrhage, hemodynamic instability, anuria, severe lactic acidosis together with liver necrosis indices appeared. A new CTA demonstrated massive parenchymal disruption within the right lobe of the liver and multiple hematomas in the left lobe. Considering the high-grade lesions of the hepatic vascular tree and liver failure, patient was listed for emergency liver transplantation (LT). LT occurred few hours later, and patient's clinical conditions rapidly improved even if the subsequent clinical course was characterized by a severe fungal infection because of immunosuppression.
Evaluation of life-threatening lesions and treatment priorities, availability of different excellence skills, and multidisciplinary collaboration have a key role to achieve clinical success in such severe cases.
Rare presentation of primary varicella zoster as fatal fulminant hepatitis in adult on low-dose,short-term steroid: Case report
2019, Annals of Medicine and Surgery
Citation Excerpt :
In Italy, a patient on low dose steroid (prednisolone 5 mg BID for 3 days then 5 mg daily for another 3 days) for tonsillitis developed VZV FAHF [6]. The only difference between our case and the case in Italy was that their patient had positive HHSV6 in concomitance with his VZV infection, and it is not clear whether such coexistent infection could have added to the burden of the VZV infection driving it into a systemic disease involvement [6]. In terms of presentation, our patient initially presented with excruciating right upper quadrant pain and tenderness, without the typical vesicular rash of primary varicella infection which appeared later on day 1.
Varicella zoster virus presents clinically as primary (chickenpox) or secondary (herpes zoster) infection. Cutaneous and extracutaneous dissemination may occur, usually in immunocompromised patients. VZV hepatitis that progresses to fulminant hepatic failure is very rare and fatal. To the best of our knowledge, 9 cases have been reported to date, of which 7 were in immunocompromised adults, and only one patient was on short duration steroid therapy.
We present a 26-year old man who was admitted initially with acute abdomen as query persistent biliary colic. Later, he showed clinical and laboratory findings of VZV hepatitis that progressed rapidly despite maximal medical ICU support and he expired on day 3 of admission.
Acute VZV infection may present as fulminant hepatitis. The presentation may initially be challenging for the diagnosis and should be considered if the patient has been in contact with a sick case. Low dose corticosteroid could carry a risk for fatal VZV fulminant hepatitis and should be used very cautiously especially with VZV patients’ contacts. Further causative relationships remain to be established.
Varicella-Zoster Virus-Induced Hepatitis in a Liver Transplant Recipient: A Case Report
2019, Transplantation Proceedings
Citation Excerpt :
VZV-associated acute hepatic insufficiency was observed in only 9 patients in these cases, 7 of which proved to be fatal. In the case of acute liver failure, LT is the most important treatment option [3,12–19]. Hepatic involvement of herpes zoster infection is rare, but it may be fatal in immunocompromised individuals.
Infections after solid organ transplantation are a major cause of mortality and morbidity. Varicella-zoster virus (VZV) infection after solid organ transplantation is rare. Here we present a case presenting with acute hepatitis and shingles after a liver transplantation (LT). A 36-year-old male patient underwent a liver transplantation; 7 months later his liver function tests increased. An examination and test results revealed that he had VZV-induced hepatitis. After VZV treatment, his test results returned to normal levels. Hepatic involvement of VZV infection is rare, but it may be fatal in immunocompromised individuals. Early diagnosis and early initiation of antiviral therapy is important in the control of hepatitis and rare hepatotropic viruses in immunocompromised individuals.
Fatal varicella in immigrants from tropical countries: Case reports and forensic perspectives
2018, Legal Medicine
The primary Varicella Zoster Virus (VZV) infection results in varicella, a generally benign, self-limiting disease in immunocompetent children. Despite the usual course a possible fatal evolution of the primary infection is observed predominantly in immunocompromised subjects and in adults, especially emigrating from tropical regions. Two cases of fatal varicella have been investigated and discussed. Death occurred in two patients over 40 years of age, coming from South Asia and receiving chronic immunosuppressive therapy. The forensic expert must be cautious and consider all clinical records in managing fatal varicella cases, bearing in mind risk factors and pre-existing conditions such as age, geographical provenance and pathological comorbidity, which may lead to a bad prognosis irrespective of therapies. Based on the severe and fatal course observed in the reported cases, an extension of the immunization program appears advisable for immigrants from tropical countries, especially before scheduled immunotherapy.
American Gastroenterological Association Institute Technical Review on Initial Testing and Management of Acute Liver Disease
2017, Gastroenterology
Citation Excerpt :
There also were no data on the diagnostic accuracy of serology in adult ALF and no data on the impact of therapy on mortality. We identified 1043–52 single case reports of VZV ALF in adults. All but 2 cases45,49 were associated with immunosuppression and all but 1 case47 also was associated with a rash and/or disseminated VZV clinical picture.
Fulminant hepatic failure attributed to infection with human herpesvirus 6 (HHV-6) in an immunocompetent woman: A case report and review of the literature
2016, Journal of Clinical Virology
Citation Excerpt :
HHV-6 has been associated with autoimmune conditions [12,24], roseola infantum [2,18], the Stevens Johnson syndrome (SJS) [42], DIHS [30,49], encephalopathy [45], pulmonary failure [36], myocarditis [14,21,32,46,52], and ALF [8,19,20,28,39,50]. In both children and adults, upper respiratory tract symptoms and a rash may precede organ failure by days to weeks [7–9,13,14,33,36,43]. After a fatal FH case ascribed to primary HHV-6 infection was reported in an infant [8], several cases have been reported, primarily in very young children [26–28,35].
Mild disease due to human herpesvirus-6 (HHV-6) has been reported in healthy children. Severe disease due to this virus can occur in immunocompromised patients but is rarely reported in previously healthy adults. We report the case of a previously healthy woman who presented with a skin rash, mild upper respiratory symptoms, and abdominal pain and succumbed to fulminant hepatic failure attributed to infection with HHV-6B.
HHV-6 may be more commonly associated with fulminant hepatitis in immunocompetent patients than previously thought and should be considered in the differential diagnosis of patients presenting with skin rash, upper respiratory symptoms, and unexplained hepatitis.

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Case reportLiverFulminant Multiorgan Failure Due to Varicella Zoster Virus and HHV6 in an Immunocompetent Adult Patient, and Anhepatia

Abstract

Section snippets

Case Report

Discussion

The epidemiology of varicella zoster virus infection in Italy

BMC Public Health

Varicella vaccination in Europe—taking the practical approach

BMC Medicine

Disseminated varicella with multiorgan failure in an immunocompetent adult

J Med Virol

Chicken pox after pediatric liver transplantation

Liver Transpl

Fulminant varicella infection complicated with acute respiratory distress syndrome, and disseminated intravascular coagulation in an immunocompetent young adult

Intern Med

Biogeographical origin and varicella risk in the adult immigration population in catalonia, spain (2004–2006)

Euro Surveill

Early detection of varicella-zoster virus (VZV)-specific T-cells before seroconversion in primary varicella infection: case report

Virol J

Case report
Liver
Fulminant Multiorgan Failure Due to Varicella Zoster Virus and HHV6 in an Immunocompetent Adult Patient, and Anhepatia