Langerhans’ cell

A group of conditions characterised by proliferation of Langerhans cells, a lymphoreticular cell. Langerhans cell aggregates are nodular—especially in the lungs—and 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 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 of younger patients that may affect any bone (except in hands and feet), most commonly the cranial vault, jaw, humerus, rib and femur.

Imaging
Mimics Ewing sarcoma.

Treatment
Curettage.

Prognosis
Excellent.

Multifocal unisystem LCH
Hand-Schüller-Christian disease, polyostotic eosinophilic granuloma; multiple bone involvement—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, anaemia, thrombocytopenia, bone marrow involvement.

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References in periodicals archive

Diabetes Insipidus and Sclerosing Cholangitis in a child may be a clue to the diagnosis of Langerhans' Cell Histiocytosis: A Case Report.

Primary Langerhans Cell Histiocytosis of the Extrahepatic Bile Duct Occurring in an Adult Patient

Pulmonary Langerhans' cell granulomatosis (histiocytosis X): a clinical study of 48 cases.

Morphometric Study of Pulmonary Arterial Changes in Pulmonary Langerhans Cell Histiocytosis

Langerhans' cell histiocytosis with bilateral temporal bone involvement.

Langerhans Cell Histiocytosis of Bilateral Mastoid Cavity

Oral mucosal involvement in Langerhans' cell histiocytosis: long-term follow-up of a rare case.

Concurrent Mastoid Cellulitis and Langerhans Cells Histiocytosis: A Challenging Diagnosis

Labyrinthine involvement in Langerhans' cell histiocytosis.

Langerhans cell histiocytosis presenting as a scalp mass in a child

Podda, "Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement," Pediatric Radiology, vol.

Going Bone Deep: Osseous Rosai-Dorfman Disease in an Adult with Recurrent, Culture-Negative Osteomyelitis

Mixed response to thalidomide therapy in adults: two cases of multisystem Langerhans' cell histiocytosis.

Adult cutaneous Langerhans' cell histiocytosis: a rare presentation, successful treatment with thalidomide

Langerhans' cell histiocytosis in paediatric population: presentation and treatment of head and neck manifestations.

Langerhans cell histiocytosis involving bilateral temporal bones in infant patient: a case report

Eddie, who plays for Dewsbury Moor under-9s in West Yorkshire, was found to have the cancer-like condition Langerhans' Cell Histiocytosis.

TUMOUR LAD SAVED BY PAT ON THE HEAD; Brain op after rugby coach spots lump

A despaired Maria Karetnikova told XPRESS that her daughter Katerina was diagnosed with Multisystem Langerhans' Cell Histiocytosis (MS-LCH) which has affected her liver, spleen, skin and GI along with "secondary hemophagocytosis" of the bone marrow in January, following which it has been one long battle against the odds.

Russian mother fights a double whammy

[10.] Marioni G, De Filippis C, Stramare R, et al.: Langerhans' cell histiocytosis: Temporal bone involvement.

Hand-schuller-Christian disease in twins: a very rare case