Original ArticleDifferentiating Skin-Limited and Multisystem Langerhans Cell Histiocytosis
Section snippets
Methods
Medical records of 71 consecutive patients who presented with any LCH skin lesions, either as skin-limited disease or as multisystem disease, at the Texas Children's Cancer and Hematology Centers between March 2005 and October 2011 were reviewed. Patients who presented either with de novo disease or from referral after diagnosis were included in this study. The age, date of diagnosis, date of symptom onset, location of LCH involvement, type of therapy, response to therapy, and time to
Results
Seventy-one patients presented with LCH skin lesions between March 2005 and August 2012 (Table). Of these patients, 21 were determined to have skin-limited disease, and 43 patients had multisystem involvement. Seven patients with multisystem disease, referred late in the course of treatment, could not be categorized due to incomplete information at diagnosis, and these patients were not included in subsequent analyses.
The majority of patients with skin-limited and skin plus multisystem disease
Discussion
LCH occurs in 2-10 per million children and 1-2 per million adults per year, with extreme clinical heterogeneity.26, 27, 28 Involvement of risk organs (liver, spleen, and bone marrow) and failure to respond to the first 12 weeks of therapy predict increased mortality risk.5, 8 Accurate staging is essential to determine optimal therapy, because observation or curettage may be appropriate for single-system disease, whereas systemic chemotherapy is required for multisystem LCH. Survival of
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Supported by the HistioCure Foundation (Texas Children's Cancer Center Histiocytosis Program), National Institutes of Health (R01 CA154489 [to C.A. and K.M.], P50CA126752 [to C.A.], and NIH K12 CA090433 [to S.S.]), and Dan L. Duncan Cancer Center (P30CA125123). The authors declare no conflicts of interest.
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Contributed equally.