Which histiocytosis classifications are no longer used?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

Langerhans cell histiocytosis formerly was divided into 3 disease categories: eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe (or Abt-Letterer-Siwe) disease, depending on the severity and extent of involvement. This classification and its related risk groups no longer are used. Systems based on these categories were meant to reflect the extent of involvement and its relationship to the patient's prognosis. [1, 63]

Some classifications, such as that of the 1987 Histiocyte Society classification schema, simply divide histiocytic disorders into class I Langerhans cell disease, class II non-Langerhans cell histiocytic disease without features of malignant disorders, and class III malignant histiocytic disorders.

A clinical-grouping system for Langerhans cell histiocytosis based on age, extent of the disease, and organ dysfunction, as once constructed, [64] can provide a means to compare patient data and prognoses. Various categories, such as limited and extensive multiorgan involvement, have also been proposed.


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