Langerhans’ cell histiocytosis

(redirected from Letterer-Siwe)

Langerhans’ cell histiocytosis

A group of conditions characterised by proliferation of Langerhans cells, which are lymphoreticular cells. Langerhans cell aggregates are nodular, especially in the lungs, and are variably accompanied by eosinophils, foamy cells, neutrophils, and fibrosis.

Prognosis
Good if limited to a single system. Multi-system involvement carries a 10–20% mortality rate due to organ failure; 50–60% have chronic disease; 30–40% have long-term clinical remission.

Langerhans cell histiocytosis types 
Pulmonary LCH
A lesion virtually exclusive to cigarette smokers, which is a form of smoking-related interstitial lung disease. Smoking cessation may lead to reversal of changes or evolution to pulmonary fibrosis and pulmonary hypertension.
 
Unifocal LCH
Eosinophilic granuloma, solitary bone involvement
A lesion affecting younger patients; may affect any bone, most commonly the cranial vault, jaw, humerus, rib and femur (often spares the hands and feet).

Imaging
Mimics Ewing sarcoma.
 
Treatment
Curettage.
 
Prognosis
Excellent.
 
Multifocal unisystem
LCH Hand-Schüller-Christian disease, multiple bone involvement.

Polyostotic eosinophilic granuloma 
A lesion that may affect the skin, accompanied by proptosis, diabetes insipidus, or chronic otitis media or combination thereof, marked by a chronic course with waxing and waning symptoms.

Prognosis
Relatively good.
 
Multifocal multisystem LCH
Letterer-Siwe disease, multiple organ involvement
A lesion that affects bone, lung and skin; while histologically indistinct, it is more aggressive than the other forms.

Prognosis
Poor if < 18 months at time of diagnosis; haemorrhagic skin lesions; hepatomegaly, anemia; thrombocytopenia; bone marrow involvement.
References in periodicals archive ?
1) At that time, histiocytosis X was subdivided into three types: (1) eosinophilic granuloma, an isolated osteolytic lesion: (2) Hand-Schuller-Christian disease, a multifocal version of eosinophilic granuloma that is often accompanied by systemic manifestations; and (3) Letterer-Siwe disease, a systemic, rapidly progressive form of histiocytosis X.
edu LETTERER-SIWE DISEASE See: Histiocytosis LEUKEMIA See also: Bone Marrow Transplant; Cancer Leukemia Research Fund of Canada Attn: Maureen E.
Langerhans' cell histiocytosis can manifest as three distinct clinicopathologic entities: acute disseminated Langerhans' histiocytosis (previously known as Letterer-Siwe disease), unifocal Langerhans' cell histiocytosis (previously known as eosinophilic granuloma).
Both terms lump together various diseases previously called Hand-Schuller-Christian disease, Letterer-Siwe disease, and eosinophilic granuloma of the bone.
In Letterer-Siwe disease, there is multisystem involvement.
LHH, Letterer-Siwe hastaligi, Hand-Schuller-Christian hastaligi, eozinofilik granulom ve konjenital kendiliginden iyilesen retikulohistiyositoz adli dort belirgin formdan olussa da bazen ortusme sendromlari seklinde de ortaya cikabilmektedir.
It should be distinguished from histiocytosis X, a systemic condition characterized by a neoplastic, clonal proliferation of Langerhans cells, with variable manifestations, including Letterer-Siwe and Hans-Schuller-Christian diseases.
The disease is manifested in three different clinical conditions: eosinophilic granuloma, Hand-Schtiller-Christian disease, and Letterer-Siwe disease.
Discussion: Letterer-Siwe syndrome was described in infants as an acutely disseminated, rapidly progressive disease affecting primarily the soft tissues with minimal or no bone involvement.
Pauline, 32, had a rare condition called Letterer-Siwe disease which stunted her growth so she is only 4ft 10in.
LCH encompasses a spectrum of clinical manifestations ranging from a solitary chronic lesion, known as an eosinophilic granuloma, to a fulminant multisystem process called Letterer-Siwe disease.