For patients with recurrent and/or refractory disease, alternative treatment has not been standardized. Patients with recurrent disease, that is, disease that reappears
Table 22-10. Risk Groups According to the Histiocyte Society LCH-III Trial
Group 1—Multisystem "Risk" Patients
Multisystem patients with involvement of one or more "risk" organs (i.e., hematopoietic system, liver, spleen, or lungs).
Group 2—Multisystem "Low-Risk" Patients
Multisystem patients with multiple organs involved but without involvement of "risk" organs.
Group 3—Single System "Multifocal Bone Disease" or Localized "Special Site" Involvement
Patients with multifocal bone disease, i.e., lesions in two or more different bones or patients with localized special site involvement, such as lesions with intracranial soft-tissue extension or vertebral lesions with intraspinal soft-tissue extension.
Table 22-11. Systemic Therapy for LCH"
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