Download PDF
Kinder- und Jugendmedizin 2022; 22(01): 41-46
DOI: 10.1055/a-1677-7487
Schwerpunkt

Die seltenen Histiozytosen (Non-Langerhans-Zell-Histiozytosen)

The rare histiocytoses (Non-Langerhans cell histiocytoses)
Carl Friedrich Classen
1   Universitätsmedizin Rostock, Sektion Pädiatrische Onkologie und Palliativmedizin, Univ.-Kinder- und Jugendklinik Rostock
› Author Affiliations
› Further Information
    Permissions and Reprints

ZUSAMMENFASSUNG

Seltene Histiozytosen, auch Non-Langerhans-Zell-Histiozytosen, sind alle proliferativen Erkrankungen der Histiozyten außer den Langerhans-Zell-Histiozytosen (LCH) und Hämophagozytischen Lymphohistiozytosen (HLH): benigne oder maligne, lokalisierte oder systemische, adulte oder pädiatrische Erkrankungen. Eine 2016 publizierte Klassifikation definiert Subgruppen als L-Gruppe (LCH-ähnlich, hierzu die Erdheim-Chester-Erkrankung, ECD), C-Gruppe (kutan/mukokutan, u. a. die Xanthogranulomatosen, XG), M-Gruppe (maligne Histiozytosen, MH), R-Gruppe (Rosai-Dorfman-Erkrankung, RDD, ähnlich) und H-Gruppe (HLH-ähnlich). In der Pädiatrie am häufigsten sind XG und RDD. Manche haben eine exzellente Prognose (v. a. C-Gruppe und RDD, mit Ausnahmen), andere sind rasch fatal (v. a. MH). So reicht die Therapie von wait-and-see bis zu intensiver Chemotherapie. Zunehmend werden klonale Veränderungen, v. a. im RAS/RAF- bzw. MAPK-Signalweg, identifiziert, mit der Möglichkeit von „targeted“ Therapien. Erfahrungen sind aber noch kaum publiziert, was die systematische Erfassung – in Deutschland im Register Seltene Histiozytosen als Teil des International Rare Histocytic Disorder Registry (Toronto) – nötig macht.

ABSTRACT

Rare histiocytoses are all histiocytic diseases that are not classified as Langerhanscell histiocytoses (LCH) or hemophagocytic lymphohistiocytosis (HLH): benign or malignant, localized or systemic, adult or pediatric diseases. A classification published in 2016 defined subgroups as L group (LCH-like, including the Erdheim-Chester disease, ECD), C group (cutanous/mucocutanous, e. g. the xanthogranulomatoses, XG), M group (malignant histiocytoses, MH), R group (Rosai-Dorfman disease, RDD, like), and H group (HLH like). Most common in pediatrics are XG and RDD. Some disorders have an excellent prognosis (especially C group and RDD, with exceptions), others are rapidly fatal (especially MH). So, therapy may include wait-and-see as well as intensive chemotherapy. Increasingly, clonal alterations are identified, especially in the RAS/RAF, or MAPK signalling pathway, opening opportunities of ”targeted“ therapies. Still, very limited experience is published, indicating that systematic registration is urgent – in Germany by the ”Register Seltene Histiozytosen“, part of the International Rare Histocytic Disorder Registry (Toronto).

Schlüsselwörter

Non-Langerhans-Zell-Histiozytosen - Erdheim-Chester-Erkrankung - juvenile Xanthogranulomatose - maligne Histiozytosen - Rosai-Dorfman-Erkrankung

Keywords

Non-Langerhanscell histiocytosis - Erdheim-Chester disease - juvenile xanthogranulomatoses - malignant histiocytoses - Rosai-Dorfman disease


Publication History

Received: 23 October 2020

Accepted: 11 November 2020

Article published online:
25 February 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG,
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • Literatur

  • 1 Emile JF, Abla O, Fraitag S. et al Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016; 127 (22) 2672-2681
    CrossrefPubMedGoogle Scholar
  • 2 Classen CF, Minkov M, Lehrnbecher T. The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches. Klin Padiatr 2016; 228 6–7 294-306
    Article in Thieme ConnectPubMedGoogle Scholar
  • 3 Mallick S, Ghosh R, Iyer VK. et al Cytomorphological and morphometric analysis of 22 cases of Rosai-Dorfman disease: a large series from a tertiary care centre. Acta Cytol 2013; 57: 625-632
    CrossrefPubMedGoogle Scholar
  • 4 Strehl JD, Stachel KD, Hartmann A. et al Juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis: case report and literature review. Int J Clin Exp Pathol 2012; 05: 720-725
    Google Scholar
  • 5 Goyal G, Heaney ML, Collin M. et al Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era. Blood 2020; 135 (22) 1929-1945
    CrossrefPubMedGoogle Scholar
  • 6 Goyal G, Young JR, Koster MJ. et al Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease. Mayo Clinic Histiocytosis Working Group. Mayo Clin Proc 2019; 94 (10) 2054-2071
    CrossrefPubMedGoogle Scholar
  • 7 Durham BH. Molecular characterization of the histiocytoses: Neoplasia of dendritic cells and macrophages. Semin Cell Dev Biol 2019; 86: 62-76
    CrossrefPubMedGoogle Scholar
  • 8 Weitzman S, Jaffe R. The non-Langerhans cell histiocytoses. Pediatr Blood Cancer 2005; 45: 256-264
    CrossrefPubMedGoogle Scholar
  • 9 Diamond EL, Dagna L, Hyman DM. et al Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood 2014; 124: 483-492
    CrossrefPubMedGoogle Scholar
  • 10 Sheu SY, Wenzel RR, Kersting C. et al Erdheim-Chester disease: case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literature. J Clin Pathol 2004; 57: 1225-1228
    CrossrefPubMedGoogle Scholar
  • 11 Cavalli G, Guglielmi B, Berti A. et al The multifaceted clinical presentations and manifestations of Erdheim-Chester disease: comprehensive review of the literature and of 10 new cases. Ann Rheum Dis 2013; 72: 1691-1695
    CrossrefPubMedGoogle Scholar
  • 12 Mazor RD, Manevich-Mazor M, Shoenfeld Y. Erdheim-Chester Disease: a comprehensive review of the literature. Orphanet J Rare Dis 2013; 08: 137
    CrossrefPubMedGoogle Scholar
  • 13 Kim MS, Kim CH, Choi SJ. et al Erdheim-Chester Disease. Ann Dermatol 2010; 22: 439-443
    CrossrefPubMedGoogle Scholar
  • 14 Braiteh F, Boxrud C, Esmaeli B. et al Successful treatment of ErdheimChester disease, a non-Langerhans-cell histiocytosis, with interferonalpha. Blood 2005; 106: 2992-2994
    CrossrefPubMedGoogle Scholar
  • 15 Haroche J, Arnaud L, Cohen-Aubart F. et al Erdheim-Chester disease. Curr Rheumatol Rep 2014; 16: 412
    CrossrefPubMedGoogle Scholar
  • 16 Aggarwal K, Gupta S, Jain VK. et al Generalized eruptive histiocytoma. Indian Dermatol Online J 2010; 01: 27-29
    CrossrefPubMedGoogle Scholar
  • 17 Hilker O, Kovneristy A, Varga R. et al Progressive nodular histiocytosis. J Dtsch Dermatol Ges 2013; 11: 301-307
    CrossrefPubMedGoogle Scholar
  • 18 Chisolm SS, Schulman JM, Fox LP. Adult Xanthogranuloma, Reticulohistiocytosis, and Rosai-Dorfman Disease. Dermatol Clin 2015; 33: 465-472 discussion 473
    CrossrefPubMedGoogle Scholar
  • 19 Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol 2003; 27: 579-593
    CrossrefPubMedGoogle Scholar
  • 20 So N, Liu R, Hogeling M.. Juvenile xanthogranulomas: Examining single, multiple, and extracutaneous presentations. Pediatr Dermatol 2020; 37 (04) 637-644
    CrossrefPubMedGoogle Scholar
  • 21 Collie JS, Harper CD, Fillman EP.. Juvenile Xanthogranuloma (Nevoxanthoendothelioma, JXG). 2020 Jul 2. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing 2020
    Google Scholar
  • 22 Black J, Coffin CM, Dehner LP. Fibrohistiocytic tumors and related neoplasms in children and adolescents. Pediatr Dev Pathol 2012; 15 (Suppl. 01) 181-210
    CrossrefPubMedGoogle Scholar
  • 23 Voruz S, Cairoli A, Naveiras O. et al Response to MEK inhibition with trametinib and tyrosine kinase inhibition with imatinib in multifocal histiocytic sarcoma. Haematologica 2018; 103 (01) e39-e41
    CrossrefPubMedGoogle Scholar
  • 24 Shanmugam V, Griffin GK, Jacobsen ED. et al Identification of diverse activating mutations of the RAS-MAPK pathway in histiocytic sarcoma. Mod Pathol 2019; 32 (06) 830-843
    CrossrefPubMedGoogle Scholar
  • 25 Xerri L, Adélaïde J, Popovici C. et al CDKN2A/B Deletion and Double-hit Mutations of the MAPK Pathway Underlie the Aggressive Behavior of Langerhans Cell Tumors. Am J Surg Pathol 2018; 42 (02) 150-159
    CrossrefPubMedGoogle Scholar
  • 26 Snuderl M, Dolgalev I, Heguy A. et al Histone H3K36I mutation in a metastatic histiocytic tumor of the skull and response to sarcoma chemotherapy. Cold Spring Harb Mol Case Stud 2019; 05 (05) a004606
    CrossrefPubMedGoogle Scholar
  • 27 Hung YP, Qian X. Histiocytic Sarcoma. Arch Pathol Lab Med 2020; 144 (05) 650-654
    CrossrefPubMedGoogle Scholar
  • 28 Facchetti F, Pileri SA, Lorenzi L. et al Histiocytic and dendritic cell neoplasms: what have we learnt by studying 67 cases. Virchows Arch 2017; 471 (04) 467-489
    CrossrefPubMedGoogle Scholar
  • 29 Bleeke M, Johann P, Gröbner S. et al Genome-wide analysis of acute leukemia and clonally related histiocytic sarcoma in a series of three pediatric patients. Pediatr Blood Cancer 2020; 67 (02) e28074
    CrossrefPubMedGoogle Scholar
  • 30 Takada M, Hix JML, Corner S. et al Targeting MEK in a Translational Model of Histiocytic Sarcoma. Mol Cancer Ther 2018; 17 (11) 2439-2450
    CrossrefPubMedGoogle Scholar
  • 31 Llamas-Velasco M, Cannata J, Dominguez I. et al Coexistence of Langerhans cell histiocytosis, Rosai-Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver. J Cutan Pathol 2012; 39: 1125-1130
    CrossrefPubMedGoogle Scholar
  • 32 Kumar R Khan SP, Joshi DD. et al Pediatric histiocytic sarcoma clonally related to precursor B-cell acute lymphoblastic leukemia with homozygous deletion of CDKN2A encoding p16INK4A. Pediatr Blood Cancer 2011; 56: 307-310
    CrossrefPubMedGoogle Scholar
  • 33 Takahashi E, Nakamura S. Histiocytic sarcoma: an updated literature review based on the 2008 WHO classification. J Clin Exp Hematop 2013; 53: 1-8
    CrossrefPubMedGoogle Scholar
  • 34 Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: A pseudolymphomatous benign disorder. Analysis of 34 cases. Cancer 1972; 30: 1174-1188
    CrossrefPubMedGoogle Scholar
  • 35 Abla O, Jacobsen E, Picarsic J. et al Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood. 2018; 131 (26) 2877-2890
    CrossrefPubMedGoogle Scholar
  • 36 Wimmer DB, Ro JY, Lewis A. et al Extranodal rosai-dorfman disease associated with increased numbers of immunoglobulin g4 plasma cells involving the colon: case report with literature review. Arch Pathol Lab Med 2013; 137: 999-1004
    CrossrefPubMedGoogle Scholar
  • 37 Rossbach HC, Dalence C, Wynn T. et al Faisalabad Histiocytosis Mimics Rosai-Dorfman Disease: Brothers With Lymphadenopathy, Intrauterine Fractures, Short Stature and Sensorineural Deafness. Pediatr Blood Cancer 2006; 47: 629-632
    CrossrefPubMedGoogle Scholar