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Letters to the Editors

Fatal Intracerebral Hemorrhage Caused by Varicella-Induced Thrombocytopenia

Marcus, Karen A. MD; Halbertsma, Feico J. J. MD; Ten, Walther E. Tjon A. MD, PhD

Author Information
The Pediatric Infectious Disease Journal: November 2007 - Volume 26 - Issue 11 - p 1075
doi: 10.1097/INF.0b013e31815768f1
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To the Editors:

Primary infection with varicella-zoster virus usually is characterized by mild to moderate symptoms, although serious complications can occur, even in immunocompetent children. Rarely, symptomatic acute thrombocytopenia is observed. Death caused by isolated, acute thrombocytopenia resulting in a massive intracerebral hemorrhage (ICH) is extremely rare and has been reported to follow a varicella infection only once before.1 Here we present a patient who died of such a devastating complication.

A 2-year-old previously healthy boy developed chickenpox. Four days after the onset of the characteristic cutaneous lesions, he developed bruises and petechia. On examination, he appeared in good health. There were no indications of bleeding at other sites. The symptoms were interpreted as varicella-induced thrombocytopenia and in the absence of mucosal bleeding and other warning signs a watchful waiting policy was proposed. Two days later, the patient presented to the emergency department. That morning, the parents had noticed that he was suddenly unresponsive to stimuli. No trauma or mucosal bleeding had occurred. On arrival, he was in cardiorespiratory distress, which was accompanied by Cushing triad. The Glascow Coma Scale score was 4 (E1M2V1). Neurologic examination revealed a dilated right pupil unresponsive to light, with bilateral deviated eyes to the right. His skin showed chickenpox lesions and numerous ecchymoses. Laboratory studies, including coagulation studies, were normal with the exception of the platelet count, which was 4000/mL in repeated samples. A computed tomography of the brain revealed a massive hemorrhage within the right hemisphere. Despite maximal therapy, the boy died several hours after arrival. Blood cultures remained sterile, and there was no evidence of systemic varicella. Serologic tests confirmed a recent varicella infection. In our patient, isolated thrombocytopenia led to fatal intracranial hemorrhage.

Thrombocytopenia during a varicella infection is probably the result of molecular mimicry between the viral antigen and platelet surface glycoproteins, causing a cross-reaction between host antibodies and platelet’s membrane antigens with subsequent increased clearance of platelets. This phenomenon is better known as acute idiopathic thrombocytopenic purpura (ITP). Although platelet count can be very low in children presenting with acute ITP, serious spontaneous hemorrhage is unusual. A rare, but devastating complication of all ITP is ICH (incidence 0.1%–1% of acute ITP or less), which is associated with a mortality rate of 55%.2 Prevention of ICH is the principal motivation to treat ITP, but it is generally thought that the outcome of childhood ITP is not altered by medication.3,4 Consequently, management of ITP is controversial, especially in minimally or moderately symptomatic children.5 Lately, consensus is emerging to adopt a “watchful waiting” approach in cases of no or minimal bleeding and relatively less severe thrombocytopenia (platelet count >10.000/mL).

Varicella-induced ITP can be prevented by means of universal varicella vaccination. A sharp decline in ambulatory visits, severe (hemorrhagic) complications, and death caused by varicella has been reported after the implementation of universal childhood vaccination. Since death and serious complications represent only a small proportion of the total disease burden, cost-effectiveness ratio is only favorable when (indirect) health-care costs due to prevention of mild disease is taken into account. Several countries have already adopted universal childhood varicella immunization program. In the Netherlands, this is under consideration.

Karen A. Marcus, MD

Feico J. J. Halbertsma, MD

Walther E. Tjon A. Ten, MD, PhD

Department of Paediatrics

Màxima Medical Centre

Veldhoven, the Netherlands

REFERENCES

1. Tobin JD Jr, Ten Bensel RW. Varicella with thrombocytopenia causing fatal intracerebral hemorrhage. Am J Dis Child. 1972;124:577–578.
2. Butros LJ, Bussel JB. Intracranial hemorrhage in immune thrombocytopenic purpura: a retrospective analysis. J Pediatr Hematol Oncol. 2003;25:660–664.
3. Kuhne T, Imbach P, Bolton-Maggs PH, Berchtold W, Blanchette V, Buchanan GR. Newly diagnosed idiopathic thrombocytopenic purpura in childhood: an observational study. Lancet. 2001;358:2122–2125.
4. Blanchette VS, Luke B, Andrew M, et al. A prospective, randomized trial of high-dose intravenous immune globulin G therapy, oral prednisone therapy, and no therapy in childhood acute immune thrombocytopenic purpura. J Pediatr. 1993;123:989–995.
5. Chandra J, Ravi R, Singh V, Narayan S, Sharma S, Dutta AK. Bleeding manifestations in severely thrombocytopenic children with immune thrombocytopenic purpura. Hematology. 2006;11:131–133.
© 2007 Lippincott Williams & Wilkins, Inc.