Langerhans’ cell histiocytosis of the liver in adults

Abdallah, M.; Généreau, T; Donadieu, Jean; Émile, Jean-François; Chazouillères, Olivier; Gaujoux-Viala, C.; Cabané, J.

doi:10.1016/j.clinre.2011.03.012

Cited by 52 publications

(54 citation statements)

References 28 publications

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“…Disseminated disease, organ dysfunction and younger age at presentation are generally poor prognostic factors while liver dysfunction was defined as the worst prognostic factor (13,14). Our first patient did well with slowly disseminating skin involvement for years and mild hypothyroidism with polydipsia due to CNS involvement for a year.…”

Section: Discussionmentioning

confidence: 75%

ATYPICAL PRESENTATION IN LANGERHANS CELL HISTIOCYTOSIS: Hearing Loss, Diabetes Insipidus, - Extensive Cutaneous Lesions with Nail Distrophy

Hocazade¹,

Doğan²,

Yorulmaz³

et al. 2017

Acta Oncol Tur.

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ÖZETLangerhans hücreli histiositoz (LCH) CD-1a boyanması gösteren klonal proliferatif lenfohistiositik bir hastalıktır. Hastalık unifokal veya multifokal tek bir sitemi veya birçok sitemi tutacak şekilde geniş bir klinik seyir gösterebilir. Tutulan alana göre diabetes insipitus, işitme kaybı veya tırnak değişiklikleri oluşabilir. Burada atipik presentasyon gösteren iki vakayı litaretür derlemesi ile beraber sunduk. Anahtar Kelimeler: Langerhans hücreli histiositoz, diabetes insipitus, işitme kaybı, tırnak distrofisi ABSTRACT Langerhans cell histiocytosis (LCH) is a clonal proliferative lymphohistiocytic disorder with mainly CD-1a positive staining. It has a wide spectrum of unifocal or multifocal mono or multisystemic disease. Rare symptoms like diabetes insipidus, hearing loss or nail distrophy might also occur according to the involved sites. In here, two cases of LCH with atypical presentation are reported with review of the literature.

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Section: Discussionmentioning

confidence: 75%

ATYPICAL PRESENTATION IN LANGERHANS CELL HISTIOCYTOSIS: Hearing Loss, Diabetes Insipidus, - Extensive Cutaneous Lesions with Nail Distrophy

Hocazade¹,

Doğan²,

Yorulmaz³

et al. 2017

Acta Oncol Tur.

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“…1 The incidence of LCH in adults is roughly 1-2 cases per million. 2 The diagnosis of LCH is established by histopathologic examination. A definitive diagnosis requires CD1a positivity by immunohistochemistry in the lesional cells.…”

Section: Discussionmentioning

confidence: 99%

“…2 Late LCH liver involvement with sclerosing cholangitis, cirrhosis, or liver insufficiency is difficult to treat. In such cases, liver transplantation is the only treatment option.…”

Section: Discussionmentioning

confidence: 99%

Solitary Langerhans cell histiocytosis of liver with sclerosing cholangitis in an adult female

2017

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Liver is affected as a late complication of high risk cases of Langerhans cell histiocytosis. Sclerosing cholangitis is a rare pattern associated with Langerhans cell histiocytosis of liver, which is even rarer in the adult population and has high mortality. The treatment is difficult and may require liver transplantation. We report a unique case of a 40-year-old female who developed sclerosing cholangitis associated with Langerhans cell histiocytosis without any evidence of involvement of other systems. Our patient required only surgery, and had been followed up for two years without recurrence. We could not find any other case of solitary liver involvement of Langerhans cell histiocytosis in literature published so far.

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“…They can rarely reveal LCH-related abnormalities (such as cholestasis in liver LCH involvement) and are useful for distinguishing alternative diagnoses or for patient therapeutic management [32,45,46]. Morning urine osmolality is a screening test for suspected diabetes insipidus [7].…”

Section: Initial Evaluation and Follow-upmentioning

confidence: 99%

How I manage pulmonary Langerhans cell histiocytosis

Lorillon

Tazi

2017

Eur Respir Rev

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Pulmonary Langerhans cell histiocytosis (PLCH) is a rare sporadic cystic lung disease of unknown aetiology that is characterised by the infiltration and destruction of the wall of distal bronchioles by CD1a + Langerhans-like cells. In adults, PLCH is frequently isolated and affects young smokers of both sexes. Recent multicentre studies have led to the more standardised management of patients in clinical practice. Smoking cessation is essential and is occasionally the only suitable intervention. Serial lung function testing is important because a significant proportion of patients may experience an early decline in forced expiratory volume in 1 s and develop airflow obstruction. Cladribine was reported to dramatically improve progressive PLCH in some patients. Its efficacy and tolerance are currently being evaluated. Patients who complain of unexplained dyspnoea with decreased diffusing capacity of the lung for carbon monoxide should be screened for pulmonary hypertension by Doppler echocardiography, which must be confirmed by right heart catheterisation. Lung transplantation is a therapeutic option for patients with advanced PLCH.The identification of the BRAF V600E mutation in approximately half of Langerhans cell histiocytosis lesions, including PLCH, and other mutations of the mitogen-activated protein kinase (MAPK) pathway in a subset of lesions has led to targeted treatments (BRAF and MEK (MAPK kinase) inhibitors). These treatments need to be rigorously evaluated because of their potentially severe side-effects.

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Langerhans’ cell histiocytosis of the liver in adults

Cited by 52 publications

References 28 publications

ATYPICAL PRESENTATION IN LANGERHANS CELL HISTIOCYTOSIS: Hearing Loss, Diabetes Insipidus, - Extensive Cutaneous Lesions with Nail Distrophy

ATYPICAL PRESENTATION IN LANGERHANS CELL HISTIOCYTOSIS: Hearing Loss, Diabetes Insipidus, - Extensive Cutaneous Lesions with Nail Distrophy

Solitary Langerhans cell histiocytosis of liver with sclerosing cholangitis in an adult female

How I manage pulmonary Langerhans cell histiocytosis

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