Langerhans’ cell histiocytosis

(redirected from multifocal eosinophilic granuloma)

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.

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).

Mimics Ewing sarcoma.
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.

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.

Poor if < 18 months at time of diagnosis; haemorrhagic skin lesions; hepatomegaly, anemia; thrombocytopenia; bone marrow involvement.
References in periodicals archive ?
20] Similarly, because parathyroid hormone levels have been shown to rise during clodronate-associated resolution of multifocal eosinophilic granuloma of bone,[21] it is unlikely that parathyroid hormone per se is responsible for the osteolytic lesions in LCH.
Experiences of clodronate treatment of multifocal eosinophilic granuloma of bone.
Histologically, the skin, oral mucosa, duodenum, pancreas, and mesenteric lymph nodes contained multifocal eosinophilic granulomas that were characterized by a central core of eosinophilic material surrounded by eosinophils, macrophages, multinucleated giant cells, occasional plasma cells, and fibrovascular connective tissue proliferation (Figure 1C).

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