Concepts in staging and clinical studies for treatment of Langerhans' cell histiocytosis.

Abstract

I N 1993, the centennial of the publication of the first case of the disorder we now call “Langerhans’ cell histiocytosis” will be celebrated. Dr Alfred Hand, Jr, then a 25year-old pediatric resident at Children’s Hospital of Philadelphia, thought he was describing a case of polyuria associated with tuberculosis (Fig 1) when he exhibited specimens to the Philadelphia Pathological Society.’ Twenty-eight years later, he revised his speculation when he noted the similarity of his case to patients reported by radiologist Schtiller,’ internist Christian3 and pediatrician Kay.4 Hand then wondered whether there was an infectious etiology because of the prominent gum involvement in two of the cases: “The thought occurs that the infective agent may gain entrance through these structures and may be animal (ameboid) rather than vegetable in nature.“’ With the description of these several cases evolved the notion that what came to be known as “HandSchiiller-Christian” syndrome was a distinct disease. The concept of separate diseases rather than different clinical manifestations (or “stages”) of the same disease was strengthened by the work over the next decade of pathologist Letterer6 and pediatrician Siwe.’ In his paper, Siwe referred to the emerging Hand-Schiiller-Christian syndrome only to give pathologic grounds as to why he considered them separate entities. The term “Letterer-Siwe disease” was coined by Abt in 1939 who again made a pathological distinction of this “disease” from Hand-SchtillerChristian syndrome.8 Ironically, the simplest came last. It was not until 1940 that cases of solitary bone lesions were reported.‘,” It took pediatrician and pathologist Sidney Farber to note the overlap of cases and challenge the