Langerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte Society

Satter, Elizabeth K.; High, Whitney A.

doi:10.1111/j.1525-1470.2008.00669.x

Cited by 236 publications

(212 citation statements)

References 39 publications

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“…LCH in children over the age of 5 years tends to appear as an isolated lesion and most of such cases are benign with spontaneous regression. On the other hand, LCH patients who present at a younger age and those with widely disseminated disease and organ dysfunction have the highest mortality (Satter and High 2008). In one large study of 101 children with LCH, the overall survival rate was 71% at 5 years (Alston et al 2007).…”

Section: Discussionmentioning

confidence: 99%

Isolated Juvenile Xanthogranuloma in the Larynx of a Three-Year-Old Child

Kawamoto

Katori

Honkura

et al. 2013

Tohoku J. Exp. Med.

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Juvenile xanthogranuloma (JXG) is a benign manifestation of non-Langerhans cell histiocytosis characterized by yellowish cutaneous nodules. Its occurrence in the larynx is very rare, but laryngeal JXG may cause severe respiratory distress. We report a patient with isolated laryngeal JXG treated by laryngomicrosurgery, and this is the first report of JXG extending to vocal fold. A 3-year-old girl presented with hoarseness and inspiration stridor. A bulky tumor was found in right glottic to subglottic region. Subtotal resection of the tumor was carried out by laryngomicrosurgery, and airway distress was diminished after the operation. In pathological examination, the resected specimen showed proliferation of histiocytic cells and spindle cells with Touton giant cells that are characterized by polynuclei or wreath nuclei and are known to appear in JXG but not in LCH. Immunohistochemistry of histiocytic cell markers demonstrated positivity for CD68, lysozyme, alpha1-anti-chymotrypsin, factor XIIIa and vimentin, and negativity for CD1a and S-100, leading to diagnosis of JXG, but not LCH. The patient was thus expected with benign prognosis, and additional resection of the tumor including vocal fold was not indicated in the initial treatment. Six weeks later, the JXG recurred and a second procedure using CO 2 laser was needed. The tumor did not re-grow thereafter, and there was no residual voice handicap. Because of its favorable prognosis and tendency for spontaneous regression, JXG in the larynx needs to be considered carefully with regard to whether reduction surgery and/or tracheotomy are necessary, and thus precise diagnosis is required.

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Section: Discussionmentioning

confidence: 99%

Isolated Juvenile Xanthogranuloma in the Larynx of a Three-Year-Old Child

Kawamoto

Katori

Honkura

et al. 2013

Tohoku J. Exp. Med.

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“…Long-term complications have been reported in at least 71% of multisystem disease survivors despite adequate treatment (Satter & High 2008) with 66% mortality for young Children with multisystem involvement who do not respond to therapy (Swerdlow et al 2008). Diabetes insipidus is the most common complication (40%) and other endocrinopathies; as well as parenchymal brain disease (Minkov et al 2009).…”

Section: Discussionmentioning

confidence: 99%

“…Diabetes insipidus is the most common complication (40%) and other endocrinopathies; as well as parenchymal brain disease (Minkov et al 2009). Second malignancies, particularly solid tumors and haematological malignancies are also seen (Satter & High 2008).…”

Section: Discussionmentioning

confidence: 99%

“…Patient's response to the induction therapy is considered the single best prognostic indicator, with a survival rate of up to 91% in those who respond (Satter & High 2008). Other prognostic factors include age at presentation, where children less than two years old, multisystem involvement and end organ dysfunction have a worse outcome (Coleman et al 2013).…”

Section: Discussionmentioning

confidence: 99%

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Importance of Clinical and Morphological Correlations in Diagnosing Langerhans Cell Histiocytosis

Hussin¹

2018

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ABSTRAK Histiositosis sel Langerhans (LCH) adalah gangguan histiositik klonal. Pelbagai manifestasi klinikal, dari lesi tulang terisolasi kepada penyakit multisistem ABSTRACTLangerhans cell histiocytosis (LCH) is a clonal histiocytic disorder. The variable clinical manifestations from isolated bone lesion to multisystem disease can cause difficulties and delay in diagnosis. We report a 2 years and 8 months-old girl who presented with a 2 weeks history of persistent fever and weight loss associated with progressive abdominal distension. Physical examination revealed pallor, bilateral proptosis, seaborrheic dermatitis over the scalp and hepatosplenomegaly. Skull X-ray demonstrated multiple lytic lesions at the base and the skull vault. Bone marrow morphology showed numerous abnormal Langerhans cells (LCs) and foamy macrophages. The trephine immunohistochemistry (IHC) stains for CD1a, S-100 and CD68 were inconclusive. The diagnosis of multisystem Langerhans cell histiocytosis (MS-LCH) in this patient was based on the clinical presentation, radiological and morphological analysis. She subsequently received chemotherapy and currently she is on maintenance therapy with a good clinical response. LCH is a rare disease and although the IHC was inconclusive, the correlation of clinical, radiological and morphological data are essential for the diagnosis.

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“…It can be local asymptomatic form with indolent course or symptomatic form involving multiple organs. They commonly show positive S100/CD1a on immunohistochemistry [1]. The annual incidence rate reported to be 0.5-5.4 million children per year [2].…”

Section: Introductionmentioning

confidence: 99%

Congenital self-healing reticulohistiocytosis: a case report

Shetty¹,

Monappa²,

Pai³

et al. 2014

Our Dermatol Online

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Congenital self-healing reticulohistiocytosis (CSHRH) is a benign type of Langerhans cell histiocytosis (LCH) also known as HashimotoPritzker disease. Clinically it presents with skin lesions at birth or in neonatal period, usually without any systemic involvement. Lesions often heal spontaneously in period of weeks to months. We report a case of CSHRH presenting with skin lesions at birth, describing need to make an early diagnosis and to have a multidisciplinary approach with regular follow-up, in managing this rare type of LCH.

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Langerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte Society

Abstract: Langerhans cell histiocytosis is a rare proliferative disorder

Cited by 236 publications

References 39 publications

Isolated Juvenile Xanthogranuloma in the Larynx of a Three-Year-Old Child

Isolated Juvenile Xanthogranuloma in the Larynx of a Three-Year-Old Child

Importance of Clinical and Morphological Correlations in Diagnosing Langerhans Cell Histiocytosis

Congenital self-healing reticulohistiocytosis: a case report

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