LANGERHANS CELL HISTIOCYTOSIS: Historical Perspectives

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THE LANGERHANS CELL

Paul Langerhans16 was born July 25, 1847 in Berlin. His father, Dr Paul Langerhans Sr, was a well-known physician and politician (member of the Reichstag in 1881) who became an honorary citizen of Berlin in 1900. His father lived for 89 years, whereas his mother Anna, née Keibel, of Huguenot descent, died young, at age 29, of tuberculosis. His cousin was Dr jur. Georg Langerhans, who became immortal in literature because he was the factual “Bürgermeister of Köpenick” who was arrested in 1906 by

DESCRIPTION OF THE DISEASE

In 1893 a classical case report15 already had been given by Alfred Hand, Jr (Resident Physician, Children's Hospital, Philadelphia):

“On the first day of December, 1893, a boy, three years old, was admitted to the Children's Hospital with a history of great thirst and polyuria, which had begun suddenly eight weeks before…The examination showed the boy to be undersized and puny.… The eyes were in a condition of exophthalmos, giving him a frog-like appearance.… His belly was distended and

MANAGEMENT

Although it is not yet known what causes the “non-neoplastic” proliferation of Langerhans cells, useful drugs have been available for more than 40 years—essentially the same drugs as today—which induce remissions and help to overcome the critical periods of risk. Prednisone2, 13 and vinblastine,5, 27 more recently supplemented by etoposide,7, 9 still are the cornerstones of cytotoxic chemotherapy, including desmopressinacetate (DDAVP), a synthetic analogue of vasopressin,25 for the often

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  • Cited by (34)

    • A Very Rare Case of Right Insular Lobe Langerhans Cell Histiocytosis (CD1a<sup>+</sup>) Mimicking Glioblastoma Multiforme in a Young Adult

      2019, World Neurosurgery
      Citation Excerpt :

      Historical terms that are no longer in use include Hand-Schüller-Christian disease to describe a child with exophthalmos, skin lesions, lytic bone lesions, and diabetes insipidus (DI) and Letterer-Siwe disease to describe infants with disseminated inflammatory lesions, including in the liver, spleen, and bone marrow. At present, both of these diseases would be classified as multisystemic, risk organ–positive LCH.6 Involvement of the CNS, excluding the pituitary gland, occurs in <4% of cases.

    • Comparison between low-dose chemotherapy and surgery for the treatment of extremity-associated solitary bone lesions in children with Langerhans cell histiocytosis in South China: A case-control study

      2018, Journal of Bone Oncology
      Citation Excerpt :

      Children and adolescents are susceptible to LCH, with an estimated annual incidence of 4–8 cases per million [2]. LCH describes a broad spectrum of clinical presentations ranging from an isolated lytic bone lesion with a self-limiting tendency to disseminated multisystem life-threatening harm [3]. Unifocal bone lesions are the most common presentation of LCH [4], and extremities are among the common sites in skeletal LCH [5].

    • How i treat Langerhans cell histiocytosis

      2015, Blood
      Citation Excerpt :

      LCH first appeared in the medical literature around 1900 with reports of children with skin lesions, lytic bone lesions, and diabetes insipidus (DI), classified as Hand-Schüller-Christian disease. Reports also emerged of infants with disseminated inflammatory lesions including liver, spleen, and bone marrow, classified as Letterer-Siwe disease.5,6 In the 1950s, Lichtenstein noted the common histologic appearance of lesions from “eosinophilic granulomas,” Hand-Schüller-Christian disease, and Letterer-Siwe disease, hypothesized a common cell of origin, and proposed the name “Histiocytosis X,” with “X” indicating the uncertain origin.7

    • Langerhans' cell histiocytosis

      2003, Lancet
      Citation Excerpt :

      Later that year he served in the army during the war between France and Prussia. After the end of the war he continued an active and productive career as an assistant professor of pathology at the University of Freiburg.13,14 Soon after being appointed professor in 1874, he developed tuberculosis.

    View all citing articles on Scopus

    Address reprint requests to Fritz Lampert, MD, Universitäts-Kinderpoliklinik, Feulgenstraβe 12, D-35385 Giessen, Germany

    *

    From the Department of General Pediatrics, Hematology and Oncology, Children's University Hospital, Justus-Liebig-University, Giessen, Germany

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