Journal of Clinical Oncology, Vol 13, 1697-1703, Copyright © 1995 by American Society of Clinical Oncology
Progressive varicella presenting with pain and minimal skin involvement in children with acute lymphoblastic leukemia
P Rowland, ER Wald, JR Mirro Jr, E Yunis, VC Albo, MR Wollman and J Blatt
Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, PA 15213, USA.
PURPOSE: Here we report the experience at the Children's Hospital of
Pittsburgh (CHP) with varicella zoster virus (VZV) in children with acute
lymphoblastic leukemia (ALL). This record review was prompted by a patient
with ALL who died suddenly of varicella hepatitis within 24 hours of
presentation with a single skin lesion. METHODS: We reviewed the medical
records of children diagnosed with ALL at the CHP from January 1984 through
December 1993, who subsequently developed VZV infection. RESULTS: Of 294
patients aged 0 to 15 years, 41 (14%) were identified as having had 42
episodes of VZV infection. Twenty patients (49%) had received prophylaxis
with varicella zoster immunoglobulin (VZIG), and all 39 patients in whom
the diagnosis was made premortem were treated with acyclovir. Twenty-nine
of the 42 cases (70%) had disease limited to the skin. Thirteen cases (30%)
had extracutaneous involvement, and five of these episodes (12% of all
cases) ended in death. Risk factors for progressive varicella included age
greater than 6 years and intensive immunosuppressive therapy at the time of
exposure. Six of eight patients with progressive varicella, including two
who died, had received VZIG. The clinical presentation in 10 of 13 patients
with progressive disease and in four of five patients who died was
dominated by severe abdominal and/or back pain. In seven cases, these
symptoms preceded the development of skin lesions by several days, and in
six patients were associated with extensive involvement of the spleen by
varicella, as demonstrated histopathologically by the presence of
Howell-Jolly bodies on peripheral-blood smear or radiographically. No
patient with uncomplicated varicella was reported to have had premonitory
pain. CONCLUSION: Recognition of these prodromes and suspicion of varicella
even in the absence of skin lesions and even in children with a history of
prior disease or VZIG administration should prompt early diagnostic and
therapeutic measures.