Differentiating Skin-Limited and Multisystem Langerhans Cell Histiocytosis - 22/10/14
, Benjamin Garmezy, BA 1, ∗, Harshal Abhyankar, MS 1, Philip J. Lupo, PhD 1, Rikhia Chakraborty, PhD 1, Karen Phaik Har Lim, MS 1, Albert Shih, BS 1, M. John Hicks, MD, PhD, DDS 2, Teresa S. Wright, MD 3, Moise L. Levy, MD 3, 4, 5, Kenneth L. McClain, MD, PhD 1, Carl E. Allen, MD, PhD 1Abstract |
Objective |
To identify features associated with multisystem involvement and therapeutic failure in patients with skin Langerhans cell histiocytosis (LCH).
Study design |
We reviewed medical records of 71 consecutive patients with LCH with skin involvement evaluated at Texas Children's Hospital and analyzed clinical features, laboratory results, and the presence of circulating cells with the BRAF-V600E mutation with respect to initial staging and clinical outcomes.
Results |
Skin disease in patients older than 18 months of age at diagnosis was associated with the presence of multisystem disease (OR, 9.65; 95% CI, 1.17-79.4). Forty percent of patients referred for presumed skin-limited LCH had underlying multisystem involvement, one-half of these with risk-organ involvement. Patients with skin-limited LCH had a 3-year progression-free survival of 89% after initial therapy, and none developed multisystem disease. Patients with skin/multisystem involvement had a 3-year progression-free survival of 44% with vinblastine/prednisone therapy, and risk-organ involvement did not correlate with failure to achieve nonactive disease. Circulating cells with BRAF-V600E were detected at higher frequency in patients with multisystem involvement (8 of 11 skin/multisystem vs 1 of 13 skin-limited; P = .002).
Conclusion |
Skin-limited LCH necessitates infrequent therapeutic intervention and has a lower risk of progression relative to skin plus multisystem LCH. The less-aggressive clinical course and lack of circulating cells with the BRAF-V600E mutation in skin-limited LCH suggest a different mechanism of disease origin compared with multisystem or risk-organ disease.
Le texte complet de cet article est disponible en PDF.Keyword : AD, LCH, NAD, OS, PFS
Plan
| 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. |
Vol 165 - N° 5
P. 990-996 - novembre 2014 Retour au numéro
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