Histiocytosis -X of the Thymus

Development of Myelomonocytic Leukemia 5 Years after Thymectomy
  • Wiesław T. Dura
  • Małgorzata J. Dura

Abstract

Histiocytosis — X [H-X, “Langerhans’ cell histiocytosis”] is a nonneoplastic but often biologically aggressive proliferation of Langerhans’ cells [LC] which primary function is an uptake, processing and presentation of an antigen in squamous epithelia.5,7,8,27 Despite observations of impaired immunity in H-X, the cause of H-X remain largely unknown and its neoplastic potential is practically indeterminable upon histopathology alone.8 The following main non-histopathologic criteria for predicting a poor prognosis have been found: [i] organ involvement which has to be assessed on the basis of a score system; [ii] dysfunction of organs that are involved in the diseases; [iii] rapid progression and poor response to therapy and finally [iv] very young age, especially when related to pediatric patients.3,12,15,21,25,27 Thymic primary presentation of H-X is rare. No LC are found in normal thymus, instead corresponding function is ascribed to interdigitating cells (IDC) which constitute a cellular substrate for intrathymic negative selection process.26,29,30,31 Both LC and IDC are believed to develop from common ancestral cells based within the bone marrow monocytic precursor compartment.

Keywords

Mediastinal Mass Eosinophilic Granuloma Thymic Tissue Thymic Tumor Thymic Epithelium 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1997

Authors and Affiliations

  • Wiesław T. Dura
    • 1
  • Małgorzata J. Dura
    • 1
  1. 1.Department of PathologyChildren’s Memorial Health Institute (CMHI)WarsawPoland

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