Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998

Alston, Richard; Tatevossian, Ruth; McNally, Richard; Kelsey, Anna; Birch, Jillian M; Eden, Tim

doi:10.1002/pbc.20884

Cited by 123 publications

(120 citation statements)

References 18 publications

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“…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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“…Oko 65% oboljelih ima jednosistemski oblik bolesti i većina njih ima odličnu prognozu (2,3,6). Najčešće su zahvaćene kosti lobanje, femur, tibija, skapula, vilica, humerus, pršljenovi i rebra.…”

Section: Diskusijaunclassified

Histiocitoza Langerhansovih Ćelija - Od Jednosistemskog Do Multisistemskog Oblika

Malčić-Zanić¹,

Predojević-Samardžić²,

Đurđević-Banjac³

et al. 2018

SCEPED

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SažetakHistiocitoza Langerhansovih ćelija je rijetko oboljenje nastalo usljed proliferacije antigen prezentujućih Langerhansovih dendriditičnih ćelija iz koštane srži koje infiltrišu različite organe. Bolest je nepredvidivog toka, od moguće spontane remisije pa do teških diseminovanih oblika sa oštećenjem vitalnih organa i letalnim ishodom uprkos primjenjenoj terapiji.U radu je prikazan slučaj 22 mjeseca stare djevojčice kod koje su se od drugog mjeca života javile promjene po koži sa tendencijom pogoršanja tokom vremena. U dobi od 22 mjeseca djevojčici je verifikovana tumorska masa iza lijevog uha sa znacima diabetes insipidusa (žedjanje, poliurija, polidipsija). Dijagnoza LCH je postvaljena patohistološki na osnovu bioptata kože i tumorske mase lijeve temporalne kosti. Uprkos terapiji bolest se reaktivirala nakon godinu dana terapije zbog čega je primjenjena i sekundarna hemoterapija. Usljed razvijenog diabetes inspidusa uvedena je doživotna supstituciona terapija antiduretskim hormonom.Izolovane kožne lezije koje ne reaguju na lokalnu terapiju i koje imaju tendenciju pogoršanja bi trebale pobuditi sumnju na LCH. U ovim slučajevima dodatna ispitivanja i bipsija kože su mandatorni radi blagovremene potvrde dijagnoze i adekvatnog liječenja. Uprkos terapiji djeca sa razvijenim multisistemskim oblikom bolesti mogu imati neadekvatan odgovor na terapiju.UDK 616.289-07

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“…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]. Congenital self-healing reticulohistiocytosis (CSHRH) is a rare benign variant of LCH first described by Hashimoto and Pritzker [3].…”

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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Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998

Abstract: Incidence rates varied significantly by age at diagnosis, and have been stable over time. Survival has improved considerably over time, but varies strongly by age and systems affected at diagnosis.

Cited by 123 publications

References 18 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

Histiocitoza Langerhansovih Ćelija - Od Jednosistemskog Do Multisistemskog Oblika

Congenital self-healing reticulohistiocytosis: a case report

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