Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages by Jean-François Emile, Oussama Abla, Sylvie Fraitag, Annacarin.

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Presentation transcript:

Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages by Jean-François Emile, Oussama Abla, Sylvie Fraitag, Annacarin Horne, Julien Haroche, Jean Donadieu, Luis Requena-Caballero, Michael B. Jordan, Omar Abdel-Wahab, Carl E. Allen, Frédéric Charlotte, Eli L. Diamond, R. Maarten Egeler, Alain Fischer, Juana Gil Herrera, Jan-Inge Henter, Filip Janku, Miriam Merad, Jennifer Picarsic, Carlos Rodriguez-Galindo, Barret J. Rollins, Abdellatif Tazi, Robert Vassallo, and Lawrence M. Weiss Blood Volume 127(22):2672-2681 June 2, 2016 ©2016 by American Society of Hematology

Histology and somatic mutations of histiocytoses of group L, C, R, M, and H. (A) L group: Histology of LCH (skin [i-ii] and bone [iii]) and of ECD (perirenal [iv-v]). Histology and somatic mutations of histiocytoses of group L, C, R, M, and H. (A) L group: Histology of LCH (skin [i-ii] and bone [iii]) and of ECD (perirenal [iv-v]). Pie chart of relative frequencies of activating kinase mutations in LCH (vi) and ECD (vii). (B) C group: Histology of JXG (i-ii). (C) R group: Histology of RDD (meningeal with high IgG4+ plasma cell infiltration [i-ii]). (D) M group: Histology of MH (i-ii). (E) H group: Histology of inherited HLH (liver [i-ii]). Staining with CD1a (Lii in red), IgG4 (Rii in brown), CD163 (Hii in brown), or hematoxylin and eosin (all others). NOS, not otherwise specified. Jean-François Emile et al. Blood 2016;127:2672-2681 ©2016 by American Society of Hematology

Examples of clinical involvement by histiocytoses. Examples of clinical involvement by histiocytoses. (A) Examples of cutaneous manifestations in (i) a child with multisystemic LCH, (ii) adult with intertrigo-like lesions, (ii) xanthelasma of ECD (ii), and (iii) skin manifestations of RDD. (B) Radiographic imaging and CT scans of (i) lytic skull bone lesions and (ii) pulmonary nodules and cysts in LCH, (iii) CT scan revealing typical “hairy kidney” lesions and (iv) micronodular ground-glass opacities and thickening of interlobular pulmonary septa in ECD. (C) 18F-labeled fluorodeoxyglucose (PET) imaging revealing (i) bilateral and symmetric signal in femurs, tibiae, and humeri in ECD, (ii) cutaneous multiple lesions in RDD, and (iii) signal over wrist, knees, and ankles of a patient with XD. Jean-François Emile et al. Blood 2016;127:2672-2681 ©2016 by American Society of Hematology