| 词条 | Letterer–Siwe disease |
| 释义 |
| name = Letterer–Siwe disease | synonyms = Acute and disseminated Langerhans cell histiocytosis | image = Autosomal recessive - en.svg | caption = This condition is inherited in an autosomal recessive manner | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} Letterer–Siwe disease is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH). It causes approximately 10% of LCH disease and is the most severe form.[1] Prevalence is estimated at 1:500,000 and the disease almost exclusively occurs in children less than three years old.[2] The name is derived from the names of Erich Letterer and Sture Siwe. PresentationLetterer-Siwe is characterized by skin lesions, ear drainage, lymphadenopathy, osteolytic lesions, and hepatosplenomegaly. The skin lesions are scaly and may involve the scalp, ear canals, and abdomen.[3] CauseOncogenic mutation of BRAF 50-70% cases {{citation needed|date=February 2018}} Diagnosis{{Empty section|date=July 2017}}PrognosisThe disease is often rapidly fatal, with a five year survival rate of 50%. The development of thrombocytopenia is a poor prognostic sign.[1] References1. ^1 {{Cite news|url=https://www.merckmanuals.com/professional/hematology-and-oncology/histiocytic-syndromes/langerhans-cell-histiocytosis|title=Langerhans Cell Histiocytosis - Hematology and Oncology - Merck Manuals Professional Edition|work=Merck Manuals Professional Edition|access-date=2017-05-19|language=en-US}} 2. ^{{Cite web|url=http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=99870&lng=EN|title=Orphanet: Letterer Siwe disease|last=RESERVED|first=INSERM US14 -- ALL RIGHTS|website=www.orpha.net|language=en|access-date=2017-05-19}} 3. ^{{Cite web|url=http://www.dermnetnz.org/topics/langerhans-cell-histiocytosis/|title=Langerhans cell histiocytosis {{!}} DermNet New Zealand|website=www.dermnetnz.org|language=en|access-date=2017-05-19}} External links{{Medical resources| ICD10 = {{ICD10|C|96|0|c|81}} | ICD9 = {{ICD9|202.5}} | ICDO = 9722/3 | OMIM = 246400 | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | DiseasesDB = 5906 | MeshID = C538636 | Orphanet = 99870 }}{{Histiocytosis}}{{DEFAULTSORT:Letterer-Siwe Disease}}{{dermatology-stub}} 3 : Rare diseases|Autosomal recessive disorders|Monocyte- and macrophage-related cutaneous conditions |
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