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Letterer-Siwe disease

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Syndrome characterised by proliferation of nonlipid histiocytes in the viscera and bones. Clinical features include a variety of skin lesions, single or multiple eosinophilic granuloma of bone, enlarged lymph nodes, enlarged liver with or without jaundice, splenomegaly, pulmonary infiltration, spiking fever, anaemia, thrombocytopenia, mandibular hyperplasia, gingival inflammation and necrosis with loosening and loss of teeth. Infants and young children are most frequently affected, but an adult form may occur. Etiology unknown.

The chronic disseminated form of this disturbance is entered as Hand-Schüller-Christian disease, under Alfred Hand jr., American paediatrician, 1868-1949.


  • E. Letterer:
    Aleukämische Retikulose. (Ein Beitrag zu den proliferativen Erkrankungen des Retikuloendothelialen Apparates).
    Frankfurter Zeitschrift für Pathologie, 1924, 30: 377-394.
  • S. A. Siwe:
    Die Retikuloendotheliose, ein neues Krankheitsbild unter den Hepatosplenomegalien.
    Zeitschrift für Kinderheilkunde, Berlin; 1933, 55: 212-247.
  • A. F. Abt, E. J. Denenholz:
    Letterer-Siwe’s disease. Splenohepatomegaly associated with widespread hyperplasia of nonlipoid-storing macrophages; discussion of the so-called reticulo-endithelioses.
    American Journal of Diseases of Children, Chicago, 1936, 51: 499-522.

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