This volume provides a comprehensive and world-class review of the field of histiocytic
neoplasms and hemophagocytic lymphohistiocytosis (HLH). It reviews all the advances in the
field of histiocytoses during the last ten years particularly with regards to the genomic
findings in LCH and other histiocytic neoplasms and the new suggested classification of the
histiocytic disorders. Additionally it features a state-of-the art update on the most recent
treatment strategies for LCH including the results of the last LCH-III international trial
salvage therapies such as reduced-intensity conditioning (RIC) stem cell transplant (SCT) and
targeted therapies with BRAF and MEK inhibitors as well as the challenging cases of
CNS-neurodegenerative LCH and its therapeutic perspectives. For primary and secondary HLH the
book updates the most recent genetic and pathophysiological findings including
macrophage-activation syndrome (MAS) and includes a special chapter on HLH in adults.
Treatment chapters encompass therapy for newly diagnosed HLH and refractory disease as well as
stem-cell transplantation and novel therapies. The text also highlights the most recent
advances in the treatment of the uncommon histiocytic disorders such as Erdheim-Chester
disease (ECD) Juvenile xanthogranuloma (JXG) and JXG-like conditions Rosai-Dorfman disease
(RDD) and the very rare malignant histiocytoses.Written by international experts in the field
Histiocytic Disorders is a valuable resource for clinicians researchers fellows and residents
who are interested in or manage histiocytic disorders in children and adults.
