Letterer-Siwe disease

Letterer-Siwe disease

DiseaseDisorder infobox
Name = PAGENAME
ICD10 = ICD10|C|96|0|c|81
ICD9 = ICD9|202.5
ICDO =


Caption =
OMIM = 246400
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
DiseasesDB = 5906
MeshID = D006646 |

Letterer-Siwe disease is a type of histiocytosiscite journal |author=Zhou HM, Zeng X, Chen QM |title= [Clinical and pathologic features of Letterer-Siwe disease: a case report and review] |language=Chinese |journal=Hua Xi Kou Qiang Yi Xue Za Zhi |volume=25 |issue=5 |pages=517–9 |year=2007 |month=October |pmid=18072575 |doi= |url=] (a condition where histiocytes proliferate in the body.) It is sometimes classified as a form of Langerhans cell histiocytosis,cite journal |author=Kobayashi M, Yamamoto O, Suenaga Y, Asahi M |title=Electron microscopic study of Langerhans cell histiocytosis |journal=J. Dermatol. |volume=27 |issue=7 |pages=453–7 |year=2000 |month=July |pmid=10935343 |doi= |url=] or as a form of histiocytosis X.cite journal |author=Novice FM, Collison DW, Kleinsmith DM, Osband ME, Burdakin JH, Coskey RJ |title=Letterer-Siwe disease in adults |journal=Cancer |volume=63 |issue=1 |pages=166–74 |year=1989 |month=January |pmid=2642729 |doi= |url=] It is most commonly seen in children less than two years old.

It is named for Erich Letterer and Sture Siwe. [WhoNamedIt|synd|1648] [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.]

Presentation

Symptoms include lymphadenopathy, hepatosplenomegaly, and seborrhea-like lesions on the skin.

Prognosis

Untreated, the disease is fatal. The five-year survival rate with treatment is fifty percent.

References

External links

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* [http://www.5mcc.com/Assets/SUMMARY/TP0529.html 5mcc]
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Look at other dictionaries:

  • Letterer-Siwe disease — Let·ter·er Si·we disease let ər ər sē və n an acute disease of children characterized by fever, hemorrhages, and other evidences of a disturbance in the reticuloendothelial system and by severe bone lesions esp. of the skull Letterer Erich (1895… …   Medical dictionary

  • letterer-siwe disease — ˈled.ərə(r)ˈzēvə noun Usage: usually capitalized L&S Etymology: after Erich Letterer b1895 German pathologist and Sture A. Siwe b1897 pediatrican and professor in Sweden, who first described it : an acute disease of children characterized by… …   Useful english dictionary

  • Letterer-Siwe disease — see Langerhans cell histiocytosis [Letterer (20th century) and S. A. Siwe (1897–1966), German physicians] …   The new mediacal dictionary

  • enfermedad de Letterer-Siwe — Eng. Letterer Siwe disease Variedad de histiocitosis X de aparición en el primer año de vida caracterizada por afectación multisistémica y mal pronóstico, apreciándose hepatoesplenomegalia, lesiones eczematosas, púrpura, linfadenopatías,… …   Diccionario de oftalmología

  • Болезнь Леттерера-Сиве (Letterer-Siwe Disease) — см. Ретикулоэндотеяиоз. Источник: Медицинский словарь …   Медицинские термины

  • Disease — Illness or sickness often characterized by typical patient problems (symptoms) and physical findings (signs). Disruption sequence: The events that occur when a fetus that is developing normally is subjected to a destructive agent such as the… …   Medical dictionary

  • Letterer — Erich, German pathologist, *1895. See L. Siwe disease …   Medical dictionary

  • Siwe — Sture A., Swedish pediatrician, 1897–1966. See Letterer S. disease …   Medical dictionary

  • Niemann–Pick disease — Niemann Pick disease Classification and external resources ICD 10 E75.2 (ILDS E75.230) ICD 9 272.7 …   Wikipedia

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