Familial Lymphohistiocytosis

  • G. E. Janka
  • B. H. Belohradsky
  • S. Däumling
  • J. Müller-Höcker
  • P. Meister
  • R. J. Haas
Part of the Haematology and Blood Transfusion / Hämatologie und Bluttransfusion book series (HAEMATOLOGY, volume 27)


Familial lymphohistiocytosis is a genetically transmitted disease affecting infants and very young children with usually a fatal outcome. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Histologic examination shows infiltration of all organs with phagocytosing histiocytes and lymphocytes as well as atrophy of the normal lymphoid tissue. The distinction from other histiocytic disorders, i.e., Letterer-Siwe disease or malignant histiocytosis, may be difficult. However, the familial occurrence and characteristic findings in the coagulation system and lipid pattern make familial lymphohistiocytosis a sufficiently distinct clinical entity. This report reviews 79 cases from the literature and adds four of own observations.


Turner Syndrome Familial Occurrence Distinct Clinical Entity Miliary Tuberculosis Malignant Histiocytosis 
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-Verlag Berlin Heidelberg New York 1981

Authors and Affiliations

  • G. E. Janka
  • B. H. Belohradsky
  • S. Däumling
  • J. Müller-Höcker
  • P. Meister
  • R. J. Haas

There are no affiliations available

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